Ralph A. Nixon, Ph.D., M.D.
Professor
Departments of Psychiatry and Cell Biology
New York University School of Medicine
(845)398-5423
nixon@nki.rfmh.org
The Nixon Lab
NYU Profile

Dr. Nixon is Vice Chairman for Research in the Department of Psychiatry at NYU Medical School. He is also Director of Research and Director of the Center for Dementia Research at the Nathan S. Kline Institute for Psychiatric Research, an NYU-affiliated research institute.

Education

Postdoctoral Training

Awards and Honors

Research Interests

The Fate of Pathogenic Proteins in Neurodegenerative Diseases

In major neurodegenerative diseases, such as Alzheimer’s and Parkinson’s, the abnormal accumulation of one or more polypeptides within or around neurons is central to pathogenesis. Our research focuses on two aspects of neurobiology that govern the fate of normal and pathogenic proteins: the regulation of proteolytic processing and the control of protein export into axons and synapses. We have identified dysfunction of the endosomal-lysosomal system, involving altered endocytosis and mistrafficking of proteases to endosomes, as the earliest known pathological response of neurons in Alzheimer’s disease. Our cell modeling studies show early endosomes to be major generators of the toxic ß-amyloid peptide and implicate dysfunction of endosomes in the mechanism of ß-amyloid accumulation in "sporadic" Alzheimer’s, the most common form of the disease. Genetic manipulations of proteolytic systems in mice are being used, together with cell culture models, to determine the consequences of endosomal-lysosomal and calpain system dysfunction on processing of Alzheimer-related proteins, receptor-mediated signal transduction, and neuronal cell death pathways.

To maintain neural circuitry, neurons transport a large proportion of their newly synthesized proteins into axons. The perikaryal accumulation of specific cytoskeletal proteins - a pathological hallmark of Alzheimer’s, ALS, and other neurologic diseases - is believed to arise in part from impaired axonal transport. A second interest of our research is to identify the molecular determinants of cytoskeletal protein transport and assembly in neurons. For example, we are defining the minimum structural requirements for neurofilament translocation by studying axonal transport and axon ultrastructure in mice after targeted deletion or mutagenesis of each of the three neurofilament subunit genes. Neurofilament transport is also regulated by sequential protein phosphorylation, triggered in part by signals from oligodendroglial cells. We have been determining the signaling pathways, phosphorylation sites, and functional implications of these post-translational modifications. Disease relevance is also being explored in several behavioral and psychiatric settings.

Publications

Original Reports

Boyd D, Nixon R, Gillespie S, Gillespie D. Screening of Escherichia coli temperature sensitive mutants by pretreatment with glucose starvation. J Bacteriol 1968; 94:1040 50.

Patterson D, Weinstein M, Nixon R, Gillespie D. Interaction of ribosomes and the cell envelope of Escherichia coli mediated by lysozyme. J Bacteriol 1970; 101:585 91.

Nixon R, Kanfer JN. Comparison of cerebral sphingolipid metabolism in vivo in "Quaking" and normal mice. Life Sciences 1971; 10:71 79.

Nixon R. Volatile amines in mouse brain: A radioassay with picogram sensitivity. Analyt Biochem 1972; 48:460 70.

Nixon R. Neurotoxicity of a non metabolizable amino acid, 1 aminocyclopentane 1  carboxylic acid: (ACPC): ACPC transport mechanisms in tissues. Biochem Pharmacol 1976; 25:726 29.

Nixon RA. Neurotoxicity of a non metabolizable amino acid, 1 aminocyclopentane 1  carboxylic acid: Regional protein levels and lipid composition of nervous tissue. J Neurochem 1976; 27:237 44.

Nixon RA, Suva M, Wolf MK. Neurotoxicity of a non metabolizable amino acid, 1 aminocyclopentane 1 carboxylic acid: Antagonism by amino acids in cultures of cerebellum. J Neurochem 1976; 27:245 51.

Nixon RA, Karnovsky ML. Uptake and metabolism of intraventricularly administered piperidine and its effects on sleep and wakefulness in the rat. Brain Res 1977; 134:501 11.

Nixon RA. Quinine sulfate for pain in the Guillain Barre syndrome. Ann Neurol 1978; 4:386 7.

LaVail JH, Nixon RA, Sidman RL. Genetic control of retinal ganglion cell projections. J Comp Neurol 1978; 182:399 421.

Nixon RA. Protein degradation in the mouse visual system I. Degradation of axonally transported and retinal proteins. Brain Res 1980; 200:69 83.

Brown BA, Nixon RA, Strocchi P, Marotta CA. Characterization and comparison of neurofilament proteins from rat and mouse CNS. J Neurochem 1981; 36:143 53.

Pope A, Amelotte JA, Belfer H, Nixon RA. Protease activities in normal and schizophrenic human prefrontal cortex and white matter. Neurochem Res 1981; 6:1043 52.

Nixon RA. Increased axonal proteolysis in myelin deficient mutant mice. Science 1982; 215:999-1001.

Nixon RA, Brown BA, Marotta CA. Posttranslational modification of a neurofilament protein during axoplasmic transport: Implications for regional specialization of axons. J Cell Biol 1982; 94:150 8.

Brown BA, Nixon RA, Marotta CA. Posttranslational processing of ß-tubulin during axoplasmic transport in CNS axons. J Cell Biol 1982; 94:159 64.

Nixon RA, Rothman JS, Chin W. Demeclocycline in the prophylaxis of self induced water intoxication. Am J Psychiatry 1982; 139:828 30.

Nixon RA. Water intoxication and lithium. Am J Psychiatry 1982; 139:1525.

Nixon RA, Brown BA, Marotta CA. Limited proteolytic modification of a neurofilament protein involves a proteinase activated by endogenous levels of calcium. Brain Res 1983; 275:384 88.

Nixon RA, Chin WW. Water intoxication and antidiuretic hormone. Am J Psychiatry 1983;140:815.

Zubenko GS, Nixon RA. Mood elevating effect of captopril in depressed patients. Am J Psychiatry 1984; 141:110 11.

Nixon RA, Marotta CA. Degradation of neurofilament proteins by purified human brain cathepsin D. J Neurochem 1984; 43:507 16.

Zubenko GS, Volicer L, Direnfeld LK, Freeman M, Langlais PJ, Nixon RA. Cerebrospinal fluid levels of angiotensin converting enzyme in Alzheimer's disease, Parkinson's disease and progressive supranuclear palsy. Brain Res 1985; 328:215 21.

Zubenko GS, Nixon RA. Captopril and teprotide as discriminators of angiotensin converting enzyme activity in brain tissue. Neurosci Lett 1985; 60:33 7.

Nixon RA, Quackenbush R, Vitto A. Multiple calcium activated neutral proteinases (CANP) in mouse retinal ganglion cell neurons: Specificities for endogenous neuronal substrates and comparison to purified brain CANP. J Neurosci 1986; 6:1252 63.

Nixon RA. Fodrin degradation by calcium activated neutral proteinase (CANP) in retinal ganglion cell neurons and optic glia: Preferential localization of CANP activities in neurons. J Neurosci 1986; 6:1264 71.

Nixon RA, Logvinenko KB. Multiple fates of newly synthesized neurofilament proteins: Evidence for a stationary neurofilament network distributed nonuniformly along axons of retinal ganglion cell neurons. J Cell Biol 1986; 102:647 59.

Pope HG Jr, Hudson JI, Nixon RA, Herridge PL. The epidemiology of Ipecac abuse. New England J Med 1986; 314:245 6.

Fleming N, Nixon RA. A versatile transition metal salt reaction for a wide range of common biochemical reagents; An instantaneous and quantifiable color test. Analyt Biochem 1986; 154:691 701.

Vitto A, Nixon RA. Calcium activated neutral proteinase of human brain: Subunit structure and enzymatic properties of multiple molecular forms. J Neurochem 1986; 47:1039 51.

Zubenko GS, Marquis JK, Volicer L, Direnfeld LK, Langlais PJ, Nixon RA. Cerebrospinal fluid levels of angiotensin converting enzyme, acetylcholinesterase, and dopamine metabolites in dementia associated with Alzheimer's disease and Parkinson's disease: A correlative study. Biol Psychiatry 1986; 21:1365 81.

Nixon RA, Lewis SE. Differential turnover of phosphate groups on neurofilament subunits in mammalian neurons in vivo. J Biol Chem 1986; 261:16298 30l.

Nixon RA, Lewis SE, Marotta CA. Posttranslational modification of neurofilament proteins by phosphate during axoplasmic transport in retinal ganglion cell neurons. J Neurosci 1987; 7(4):1145 58.

Shea TB, Majocha RE, Marotta, CA, Nixon RA. Soluble, phosphorylated forms of the high molecular weight neurofilament protein in perikarya of cultured neuronal cells. Neurosci Lett 1988; 92:291 7.

Shea TB, Nixon RA. Differential distribution of vimentin and neurofilament protein immunoreactivity in NB2a/d1 neuroblastoma cells following neurite retraction distinguishes two separate intermediate filament systems. Devel Brain Res 1988;41:298 302.

Shea TB, Sihag RK, Nixon RA. Neurofilament triplet proteins of NB2a/d1 neuroblastoma: posttranslational modification and incorporation into the cytoskeleton during differentiation. Devel Brain Res 1988; 43:97 109.

Sihag RK, Jeng AY, Nixon RA. Phosphorylation of neurofilament proteins by protein kinase C. FEBS Lett 1988; 233:181 5.

Pope HG, Jr, McElroy SL, Nixon RA. Possible synergism between fluoxetine and lithium in refractory depression. Am J Psychiatry 1988; 145:1292 4.

Lewis SE, Nixon RA. Multiple phosphorylated variants of the high molecular mass subunit of neurofilaments in axons of retinal cell neurons: Characterization and evidence for their differential association with stationary and moving neurofilaments. J Cell Biol 1988; 107:2689 2701.

Nixon RA, Lewis SE, Dahl D, Marotta CA, Drager UC. Early posttranslational modifications of the three neurofilament subunits in mouse retinal ganglion cells: neuronal sites and time course in relation to subunit polymerization and axonal transport. Molec Brain Res 1989; 5:93 108.

Sihag RK, Nixon, RA. In vivo phosphorylation of distinct domains of the 70 kilodalton neurofilament subunit involves different protein kinases. J Biol Chem 1989; 264:457 64.

Shea TB, Clarke JF, Wheelock TR, Paskevich PA, Nixon RA. Aluminum salts induce the  accumulation of neurofilaments in perikarya of NB2a/d1 neuroblastoma. Brain Res 1989; 492:53-64.

Shea TB, Beermann ML, Nixon RA. Appearance and localization of phosphorylated variants of the high molecular weight neurofilament protein in NB2/d1 cytoskeletons during differentiation. Devel Brain Res 1989; 50:142 6.

Falk WE, Rosenbaum JT, Otto MW, Zusky PM, Weilburg JF, Nixon RA. Fluoxetine versus trazodone in depressed geriatric patients. J Geriatric Psychiatry Neurol 1989; 2:208 14.

Cataldo AM, Thayer CY, Bird ED, Wheelock TR, Nixon RA. Lysosomal proteinase antigens are prominently localized within senile plaques of Alzheimer's disease: Evidence for a neuronal origin. Brain Res 1990; 513:181 92.

Shea TB, Beermann ML, Nixon RA. Post translational modification of ß tubulin by acetylation and detyrosination in NB2a/d1 neuroblastoma cells. Devel Brain Res 1990; 51:195 204.

Nixon RA, Fischer I, Lewis SE. Synthesis, axonal transport and turnover of the high molecular weight microtubule associated protein MAP 1A in mouse retinal ganglion cells: Tubulin and MAP 1A display distinct transport kinetics. J Cell Biol. 1990; 110:437 48.

Sihag RK, Nixon RA. Phosphorylation of the amino terminal head domain of the middle molecular mass 145 kDa subunit of neurofilaments: Evidence for regulation by second messenger dependent protein kinases. J Biol Chem. 1990; 265:4166 71.

Canoso RT, de Oliveira RM, Nixon RA. Neuroleptic associated autoantibodies. A prevalence study. Biol Psychiatry 1990; 27:863 70.

Nixon RA, Clarke JF, Logvinenko KB, Tan MKH, Hoult M, Grynspan F. Aluminum inhibits calpain mediated proteolysis and induces human neurofilament proteins to form protease resistant high molecular weight complexes. J Neurochem 1990; 55:1950 9.

Shea TB, Sihag RK, Nixon RA. Dynamics of phosphorylation and assembly of the high molecular weight neurofilament subunit in NB2a/d1 neuroblastoma. J Neurochem 1990; 55:1784 92.

Cataldo AM, Nixon RA. Enzymatically active lysosomal proteases are associated with amyloid deposits in Alzheimer brain. Proc Nat Acad Sci USA 1990; 87:3861 5.

Cataldo AM, Paskevich PA, Kominami E, Nixon RA. Lysosomal hydrolases of different classes are abnormally distributed in brains of patients with Alzheimer disease. Proc Nat Acad Sci USA , 1991; 88:10998 11002.

Shea TB, Beermann ML, Nixon RA. Multiple proteases regulate neurite outgrowth in NB2a/d1 neuroblastoma cells. J Neurochem 1991; 56:842 51.

Sihag RK, Nixon RA. Identification of Ser 55 as a major protein kinase A phosphorylation site on the 70 kDa subunit of neurofilaments: Early turnover during axonal transport. J Biol Chem 1991; 266:18861 7.

Takeuchi KS , Saito K I, Nixon RA. Immunoassay and activity of calcium activated neutral proteinase (mCANP): Distribution in soluble and membrane associated fractions in human and mouse brain. J Neurochem. 1992; 58:1526 32.

Shea TB, Beermann ML, Nixon RA. Sequential effects of astroglial derived factors on neurite outgrowth: Initiation by protease inhibitors and potentiation by extracellular matrix components. J Neurosci Res 1992; 31:309 17.

Shea TB, Beermann ML, Nixon RA. Aluminum alters the electrophoretic properties of neurofilament proteins: Role of phosphorylation state. J Neurochem 1992;58:542 7.

Leli U, Cataldo A, Shea TB, Nixon RA, Hauser G. Distinct mechanisms of differentiation of SH SY5Y neuroblastoma cells by protein kinase C activators and inhibitors. J Neurochem 1992; 58:1191 8.

Shea TB, Beermann ML, Nixon RA. Complementary effects of laminin and protease inhibitors on neurite outgrowth and maintenance. Neurosci Res Commun 1992; 10:53 61.

Shea TB, Beermann ML, Nixon RA, Fischer I. Microtubule associated protein tau is required for axonal neurite elaboration by neuroblastoma cells. J Neurosci Res 1992; 32:363 74.

Shea TB, Beermann ML, Leli U, Nixon RA. Opposing influences of protein kinase activities on neurite outgrowth in human neuroblastoma cells: Initiation by kinase A and restriction by kinase C. J Neurosci Res 1992; 33:398 407.

Leli U, Shea TB, Cataldo A, Hauser G, Grynspan F, Beermann ML, Liepkalns VA, Nixon RA, Parker PJ. Differential expression and subcellular localization of protein kinase C alpha, beta, gamma, delta, and epsilon isoforms in SH SY5Y neuroblastoma cells: Modifications during differentiation. J Neurochem 1993; 60:289 98.

Saito K S, Nixon RA. Specificity of calcium activated neutral proteinase (CANP) inhibitors for human muCANP and mCANP. J Neurochem Res 1993; 18(2):231 3.

Albert M, Jenike M, Nixon R, Nobel K. Thyrotropin response to thyrotropin  releasing hormone in patients with dementia of the Alzheimer type. Biol Psychiatry 1993; 33:267 71.

Shea TB, Beermann ML, Honda T, Nixon RA. Secretion of amyloid precursor protein and laminin by cultured astrocytes is influenced by culture conditions. J Neurosci Res 1994; 37:197 207.

Cataldo AM, Hamilton DJ, Nixon RA. Lysosomal abnormalities in degenerating neurons link neuronal compromise to senile plaque development in Alzheimer disease. Brain Res 1994; 640:68 80.

Nixon RA, Paskevich PA, Sihag RK, Thayer C. Phosphorylation on carboxyl terminus domains of neurofilament proteins in retinal ganglion cell neurons in vivo: Influences on regional neurofilament accumulation, interneurofilament spacing, and axon caliber. J Cell Biol 1994; 126:1031 46.

Nixon RA, Lewis SE, Mercken M, Sihag RK. [32P]Orthophosphate and [35S]methionine label separate pools of neurofilaments with markedly different axonal transport kinetics in mouse retinal ganglion cells in vivo. Neurochem Res 1994; 19:1445 53 (Festschrift issue).

Sihag RK, Wang L, Cataldo AM, Hamlin M, Cohen C, Nixon RA. Evidence for the association of protein 4.1 immunoreactive forms with neurofibrillary tangles in Alzheimer's disease brains. Brain Res 1994; 656:14 26.

Burke WJ, Galvin NJ, Hyung DC, Stoff SA, Gillespie KN, Cataldo AM, Nixon RA. Degenerative changes in epinephrine tonic vasomotor neurons in Alzheimer's disease. Brain Res 1994; 661:35 42.

Cataldo AM, Barnett JL, Berman SA, Li J, Quarless S, Bursztajn S, Lippa C, Nixon RA. Gene expression and cellular content of cathepsin D in Alzheimer's disease brain: Evidence for early up regulation of the endosomal lysosomal system. Neuron 1995; 14:671-80.

Schwagerl AL, Mohan PS, Cataldo AM, Vonsattel JP, Kowall NW, Nixon RA. Elevated levels of the endosomal lysosomal proteinase cathepsin D in cerebrospinal fluid in Alzheimer disease. J Neurochem 1995; 64:443 6.

Mohan PS, Nixon RA. Purification and properties of high molecular weight calpastatin from bovine brain. J Neurochem 1995; 64: 859-66.

Compaine A, Schein JD, Tabb JS, Mohan PS, Nixon RA. Limited proteolytic processing of the mature form of cathepsin D in human and mouse brain: Postmortem stability of enzyme structure and activity. Neurochem Intl 1995; 27:385-96.

Shea TB, Beermann ML, Nixon RA. Aluminum treatment of intact neuroblastoma cells alters neurofilament subunit phosphorylation, solubility and proteolysis. Molec Chem Neuropathol 1995; 26:1-14.

Cressman CM, Mohan PS, Nixon RA, Shea TB. Proteolysis of protein kinase C: mM and mu M calcium-requiring calpains have different abilities to generate, and degrade the free catalytic subunit, protein kinase M. FEBS Lett 1995; 367:223-7.

Shea TB, Cressman CM, Spencer MJ, Beermann ML, Nixon RA. Enhancement of neurite outgrowth following calpain inhibition is mediated by protein kinase C. J Neurochem 1995; 65:517-27.

Mercken M, Grynspan F, Nixon RA. Differential sensitivity to proteolysis by brain calpain of adult human tau, fetal human tau and PHF tau. FEBS Lett 1995; 368:10-14.

Mercken M, Fischer I, Kosik KS , Nixon RA. Three distinct axonal transport rates for tau, tubulin and other microtubule-associated proteins: Evidence for dynamic interactions of tau with microtubules in vivo. J Neurosci 1995; 15:8259-67.

Li J, Grynspan F, Berman S, Nixon R, Bursztajn S. Regional differences in gene expression for the calcium activated neutral proteases (calpains) and their endogenous inhibitor (calpastatin) in the mouse brain and spinal cord. J Neurobiol 1996; 30(2):177-191.

Cataldo AM, Hamilton DJ, Barnett JL, Paskevich PA, Nixon RA. Properties of the endosomal-lysosomal system in the human central nervous system: Disturbances mark most neurons in populations at risk to degenerate in Alzheimer's disease. J Neurosci 1996; 16:186-99.

Didier M, Bursztajn S, Adamec E, Passani L, Nixon RA, Coyle JT, Wei JY, Berman SA. DNA strand breaks induced by sustained glutamate excitotoxicity in primary neuronal cultures. J Neurosci 1996; 16:2238-2250.

Shea TB, Spencer MJ, Beermann ML, Cressman CM, Nixon RA. Calcium influx into human neuroblastoma cells induces ALZ-50 immunoreactivity: Involvement of calpain-mediated hydrolysis of protein kinase C. J Neurochem 1996; 66:1539-49.

Cataldo AM, Barnett JL, Mann DA, Nixon RA. Colocalization of lysosomal hydrolase and ß-amyloid in diffuse plaques of Alzheimer's disease and Down's syndrome brains. J Neuropathol Exp Neurol 1996; 55(6):704-715.

Shiurba, Ishiguro K, Takahashi M, Sato K, Spooner ET, Mercken M, Yoshida R, Wheelock TR, Yanagawa H, Imahori K, Nixon, RA. Immunocytochemistry of tau phosphoserine 413 and tau protein kinase I in Alzheimer pathology. Brain Res 1996; 737:119-132.

Sanchez I, Hassinger L, Paskevich P, Shine HD, Nixon RA. Oligodendroglia regulate the regional expansion of axon caliber and local accumulation of neurofilaments during development independently of myeline formation. J Neurosci 1996; 16:5095-5105.

Kampfl A, Posmantur R, Nixon R, Grynspan F, Zhao X, Liu SJ, Newcomb JK, Clifton GL, Hayes RL. Calpain I activation and calpain I mediated cytoskeletal proteolysis following traumatic brain injury. J Neurochem 1996; 67(4):1575-1583.

Cataldo AM, Hamilton DJ, Barnett JL, Paskevich PA, Nixon RA. Abnormalities of the endosomal-lysosomal system in Alzheimer’s Disease: relationship to disease pathogenesis. Adv Exp Med Biol 1996; 389:271-80.

Grynspan F, Griffin WB, Mohan PS, Shea TB, Nixon RA. Calpains and calpastatin in SH-Sy5Y neuroblastoma cells during retinoic acid induced differentiation and neurite outgrowth: Comparison to the human brain calpain system. J Neurosci Res 1997; 48:181-191.

Grynspan F, Griffin WR, Cataldo A, Katayama S, Nixon RA. Active site-directed antibodies identify calpain II as an early-appearing and pervasive component of neurofibrillary pathology in Alzheimer’s disease. Brain Res 1997; 763:145-158.

Shea TB, Dahl DC , Nixon RA, Fischer I. Triton-soluble phosphovariants of the heavy neurofilament subunit in developing and mature mouse central nervous system. J Neurosci Res 1997; 48:515-523.

Adamec E, Mercken M, Beerman ML, Didier M, Nixon RA. Acute rise in the concentration of free cytoplasmic calcium leads to dephosphorylation of the microtubule-associated protein tau. Brain Res 1997; 757:93-101.

Cataldo AM, Barnett JL, Pieroni C, Nixon RA. Increased neuronal endocytosis and protease delivery to early endosomes in sporadic Alzheimer’s disease: neuropathologic evidence for a mechanism of increased ß-amyloidogenesis. J Neurosci 1997; 17:6142-6151.

Capell A, Saffrich R, Olivo J-C, Meyn L, Walter J, Grunberg J, Mathews P, Nixon RA, Dotti C, Haass C. Cellular expression and protelytic processing of presenilin proteins is developmentally regulated during neuronal differentiation. J Neurochem 1997; 68:2432-2440.

Shiurba RA, Spooner ET, Ishiguro K, Takahashi M, Yoshida R, Wheelock TR, Imahori K, Cataldo AM, Nixon RA. Immunocytochemistry of formalin-fixed human brain tissues: microwave irradiation of free-floating sections. Brain Res Protocols 1998; 2:109-119.

Li J, Nixon RA, Messer A, Berman S, Bursztajn S. Altered gene expression for calpain/calpastatin in motorneuron degeneration (mnd) mutant mouse brain and spinal cord. Molec Brain Res 1998; 53:174-186.

Capell A, Grunberg J, Pesold B, Diehlmann A, Citron M, Nixon R, Beyreuther K, Selkoe DJ, Haass C. The proteolytic fragments of the Alzheimer’s disease-associated presenilin-1 form heterodimers and occur as a 100-150-kDa molecular mass complex. J Biol Chem 1998; 273:3205 - 3211.

Adamec E, Beerman ML, Nixon RA. Calpain I activation in rat hippocampal neurons in culture is NMDA receptor selective and not essential for excitotoxic cell death. Molec Brain Res 1998; 54:35-48.

Troncoso J, Cataldo AM, Nixon RA, Barnett JL, Lee MK, Checler F, Fowler DR, Smialek JE, Crain B, Martin LJ, Kawas CH. Neuropathology of preclinical and clinical late-onset Alzheimer’s disease. Annals of Neurology 1998; 43:673-676.

Adamec E, Didier M, Nixon RA. Developmental regulation of the recovery process following glutamate-induced calcium rise in rodent primary neuronal cultures. Devel Brain Res 1998; 108:101-110.

Wang, KKW, Posmantur R, Nadimpalli R, Nath R, Mohan P, Nixon RA, Talanian RV, Keegan M, Herzog L, Allen H. Caspase-mediated fragmentation of calpain inhibitor protein calpastatin during apoptosis. Arch Biochem Biophys 1998; 356:187-196.

Buchner K, Adamec E, Beermann ML, Nixon RA. Isoform-specific translocation of protein kinase C following glutamate administration in primary hippocampal neurons. Molec Brain Res 1999; 64:222-235.

Sihag RK, Jaffe H, Nixon RA, Rong X. Serine-23 is a major protein kinase a phosphorylation site on the amino-terminal head domain of the middle molecular mass subunit of neurofilament proteins. J Neurochem 1999; 72:491-499.

Lai F, Kammann E, Rebeck GW, Anderson A, Chen Y, Nixon RA. APOE genotype and gender effects on Alzheimer’s disease in 100 adults with Down syndrome. Neurology 1999; 53:331-336.

Levesque L, Annaert W, Craessaerts K, Mathews PM, Seeger M, Nixon RA, Van Leuven F, Gandy S, Westaway D, St. George-Hyslop P, De Strooper B, Fraser PE. Developmental expression of wild-type and mutant presenilin-1 in hippocampal neurons from transgenic mice: evidence for novel species-specific properties of human presenilin-1. Molec Medicine 1999; 5:542- 554.

Adamec E, Vonsattel JP, Nixon RA. DNA strand breaks in Alzheimer’s disease. Brain Res 1999; 849:67-77.

Anderson AJ, Stoltzner S, Lai F, Su J, Nixon RA. Morphological and biochemical assessment of DNA damage and apoptosis in Down syndrome and Alzheimer disease, and effect of postmortem tissue archival on TUNEL. Neurobiology of Aging 2000; 21:511-524.

Cataldo AM, Peterhoff CM, Troncoso JC, Gomez-Isla T, Hyman BT, & Nixon RA. Endocytic pathway abnormalities precede amyloid-ß deposition in sporadic Alzheimer’s disease and Down syndrome. Am J Path 2000; 157:277-286.

Sanchez I, Hassinger L, Sihag RK, Cleveland DW, Mohan P, Nixon RA. Local control of neurofilament accumulation during radial growth of myelinating axons In Vivo: Selective role of site- specific phosphorylation. J Cell Biol  2000; 151:1013-1024.

Mathews PM, Cataldo AM, Kao BH, Rudnicki AG, Qin X, Yang JL, Jiang Y, Picciano M, Hulette C, Lippa CF, Bird TD, Nochlin D, Walter J, Haass C, Levesque L, Fraser PE, Andreadis A, Nixon RA. Brain expression of presenilins in sporadic and early-onset, familial Alzheimer’s disease. Molec Medicine 2000; 6:878-891.

Adamec E, Mohan PS, Cataldo AM, Vonsattel JP, Nixon RA. Up-regulation of the lysosomal system in experimental models of neuronal injury: implications for Alzheimer’s disease. Neuroscience 2000; 100:663-675

Janus C, Pearson J, McLaurin J, Mathews PM, Jiang Y, Schmidt SD, Chishti MA, Horne P, Heslin D, French J, Mount HTJ, Nixon RA, Mercken M, Bergeron C, Fraser PE, St. George- Hyslop P, Westaway D. Aß peptide immunization reduces behavioural impairment and plaques in a model of Alzheimer’s disease. Nature 2000; 408:979-982.

Adamec E, Yang F, Cole GM, Nixon RA. Multiple-label immunocytochemistry for the evaluation of nature of cell death in experimental models of neurodegeneration. Brain Res Protocols 2001; 7:193-202

Nixon RA, Mathews PM, Cataldo AM. The neuronal endosomal-lysosomal system in Alzheimer’s disease. Journal of Alzheimer’s Disease 2001; 3:97-107.

Cataldo AM, Rebeck GW, Ghetti B, Hulette C, Lippa C, Van Broeckhoven C, van Duijn C, Cras P, Bogdanovic N, Bird T, Peterhoff CM, Nixon RA. Endocytic disturbances distinguish among subtypes of Alzheimer’s disease and related disorders. Ann Neurol 2001; 50:661-665.

Mathews PM, Guerra CB, Jiang Y, Grbovic OM, Kao BH, Schmidt SD, Dinakar R, Mercken M, Hille-Rehfeld A, Rohrer J, Mehta P, Cataldo AM, Nixon RA. Alzheimer’s disease-related overexpression of the cation-dependent mannose 6-phosphate receptor increases Aß secretion: Role for altered lysosomal hydrolase distribution in ß-amyloidogenesis. J Biol Chem 2002; 277:5299-5307.

Yang D-S, Tandon A, Chen F, Yu G, Yu H, Arawaka S, Hasegawa H, Duthie M, Schmidt, SD, Triprayer V, Ramabhadran TV, Nixon RA, Mathews PM, Gandy SE, Mount HTJ, St. George-Hyslop P, Fraser PE. Mature Glycosylation and Trafficking of Nicastrin Modulate Its Binding to Presenilins. J Biol Chem 2002; 277:28135-28142.

Mathews PM, Jiang Y, Schmidt SD, Grbovic OM , Mercken M, Nixon RA. Calpain activity regulates the cell surface distribution of amyloid precursor protein: inhibition of calpains enhances endosomal generation of ß-cleaved C-terminal APP fragments. J Biol Chem 2002; 277:36415-36424.

Di Rosa G, Odrijin T, Nixon RA, Arancio O. Calpain inhibitors: a treatment for Alzheimer's disease. J Molec Neurosci. 2002 Aug-Oct;19(1-2):135-41..

Rozmahel R, Mount HT, Chen F, Nguyen V, Huang J, Erdebil S, Liauw J, Yu G, Hasegawa H, Gu Y, Song YQ, Schmidt SD, Nixon RA, Mathews PM, Bergeron C, Fraser P, Westaway D, St. George-Hyslop P. Alleles at the nicastrin locus modify presenilin 1-deficiency phenotype. Proc Nat Acad Sci USA 2002; 99:14452-14457.

Adamec E, Mohan PS, Vonsattel JP, Nixon RA. Calpain activation in neurodegenerative diseases: confocal immunofluorescence study with antibodies specifically recognizing the active form of calpain 2. Acta Neuropathologica 2002; 104:92-104.

Adamec E, Murrell JR, Takao M, Hobbs W, Nixon RA, Ghetti B, Vonsattel JP. P301L tauopathy: confocal immunofluorescence study of perinuclear aggregation of the mutated protein. J Neurol Sci 2002; 200:85-93.

Rao, MV, Engle LJ, Mohan PS, Yuan A, Qiu D, Cataldo A, Hassinger L, Jacobsen S, Lee VM-Y, Andreadis A, Julien J-P, Bridgman PC, Nixon RA. Myosin Va binding to neurofilaments is essential for correct myosin Va distribution and transport and neurofilament density. J Cell Biol 2002; 159:279-289.

Rao MV, Garcia ML, Miyazaki Y, Gotow T, Yuan A, Mattina S, Ward CM, Calcutt NA, Uchiyama Y, Nixon RA, Cleveland DW. Gene replacement in mice reveals that the heavily phosphorylated tail of neurofilament heavy subunit does not affect axonal caliber or the transit of cargoes in slow axonal transport. J Cell Biol 2002; 158:681-693.

Noble W, Olm V, Takata K, Casey E, O M, Meyerson J, Gaynor K, LaFrancois J, Wang L, Kondo T, Davies P, Burns M, Veeranna , Nixon R, Dickson D, Matsuoka Y, Ahlijanian M, Lau L-F, Duff K.  Cdk5 is a key factor in tau aggregation and tangle formation. Neuron 2003; 38:555-565.

Battaglia F, Trinchese F, Liu S, Walter S, Nixon RA, Arancio O. Calpain inhibitors, a treatment for Alzheimer's disease: position paper. J Mol Neurosci. 2003 Jun;20(3):357-62.

Cataldo AM, Petanceska S, Peterhoff CM, Terio NB, Epstein CJ, Villar A, Carlson EJ, Staufenbiel M, and Nixon RA. Endosomal Abnormalities of Alzheimer’s Disease in a Trisomy 16 Mouse Model of Down Syndrome: Modulation by App Gene Dosage. J Neurosci. 2003; 23(17):6788-6792.

Grbovic OM, Mathews PM, Jiang Y, Schmidt SD, Dinaker R, Summers-Terio NB, Ceresa BP, Nixon RA, Cataldo AM. Rab5-stimulated up-regulation of the endocytic pathway increases intracellular ßCTF levels and A production. J Biol Chem. 2003; 278(33):31261-31268.

Yuan A, Rao MV, Kumar A, Julien J-P, Nixon RA. Neurofilament transport in vivo requires hetero-oligomer formation. J Neurosci. 2003; 23(28):9452-9458.

Phinney AL, Drisaldi B, Lugowski S, Schmidt SD, Coronado V, Liang Y, Horne P, Yang J, Sekoulidis J, Coomaraswamy J, Mathews PM, Nixon RA, Carlson GA, St George-Hyslop P, Westaway D. In vivo reduction of Aß by a mutant CuATPase7b transporter.  Proc. Natl. Acad. Sci. USA. 2003; 100:14193-14198.

Rao MV, Campbell J, Yuan A, Kumar A, Gotow T, Uchiyama Y, Nixon RA. The neurofilament middle molecular weight subunit carboxyl terminal tail domains are essential for the radial growth and cytoskeletal architecture of axons but not for regulating neurofilament transport rate. J Cell Biol 2003; 163:1021-1031.

Pawlik M, Sastre M, Calero M, Mathews PM, Schmidt SD, Nixon RA, Levy E.  Overexpression of human cystatin C in transgenic mice does not affect levels of endogenous brain amyloid ß peptide.  J Molecular Neuroscience, 2004; 22 (1-2): 13-8. 

Helpern JA, Lee S-P, Falangola MF, Dyakin VV, Bogart A, Estok K, Ardekani B, Duff K, Branch C, Wisniewski T, deLeon MJ, Wolf O, O’Shea J, Wegiel J, Nixon RA. Magnetic resonance imaging assessment of neuropathology in a transgenic mouse model of Alzheimer’s disease. Magn Reson Medicine, 2004; 51:794-798.

Laudon H, Mathews PM, Karlström H, Berman A, Farmery MR, Nixon RA, Winblad B, Gandy SE, Lendahl U, Lundkvist J, & Näslund J. Co-expressed presenilin 1 NTF and CTF form functional ß-secretase complexes in cells devoid of full-length protein. J Neurochem, 2004; 89:44-53.

Sadowski M, Pankiewicz J, Scholtzova H, Yong J, Quartermain D, Jensen CH, Duff K, Nixon RA, Gruen RJ, and Wisniewski T.  Amyloid-ß deposition is associated with decreased hippocampal glucose metabolism and spatial memory impairment in APP/PS1 mice.  JNEN, 2004; 63:418-428.

Yao J, Petanceska SS, Montine TJ, Holtzman DM, Schmidt SD, Parker SD, Callahan MJ, Lipinski WJ, Bisgaier CL, Turner BA, Nixon RA, Martins RN, Ouimet C, Smith JD, Ehrlich ME, Walker LC, Mathews PM, Gandy S.  Aging, gender, and apoE isotype modulate parallel trends in levels of Alzheimer’s amyloid ß peptides and F2-isoprostane biomarkers. J. Neurochemistry 2004;90:1011-1018.

Cataldo AM, Petanceska S, Terio NB, Peterhoff CM, Durham R, Mercken M, Mehta PD, Buxbaum J, Haroutunian V, Nixon RA. Aß localization in abnormal endosomes: association with earliest Aß elevation in AD and Down syndrome.  Neurobiol Aging 2004; 25:1263-1272.

Sastre M, Calero M, Pawlik M, Mathews PM, Kumar A, Danilov V, Schmidt SD, Nixon RA, Frangione B, and Levy E. Binding of cystatin C to Alzheimer’s amyloid-ß inhibits in vitro amyloid fibril formation.  Neurobiol Aging 2004 25:1033-1043.

Yu WH, Kumar A, Peterhoff C, Shapiro Kulkane L, Uchiyama Y, Lamb BT, Cuervo AM, Nixon RA. Autophagic vacuoles are enriched in APP-secretase activities: Implications for Aß peptide over-production and localization in Alzheimer’s disease. Int J Biochem Cell Biol 2004; 36:2531-2540.

Lee S-P, Falangola MF, Nixon RA, Duff K, Helpern JA. Visualization of ß-Amyloid Plaques in a Transgenic Mouse Model of Alzheimer's disease using MR microscopy without contrast reagents. Magn Reson Med  2004; 52:538-544.

Cataldo AM, Peterhoff CM, Schmidt SD , Terio N, Duff K, Beard M, Mathews PM, Nixon RA. Presenilin mutations in familial Alzheimer’s disease and transgenic mouse models accelerate neuronal lysosomal pathology. JNEN 2004; 63:821-830.

Veeranna , Kaji T, Boland B, Odrljin T, Mohan P, Basavarajappa B, Peterhoff C, Cataldo A, Rudnicki A, Li B-S, Pant HC, Hungund L, Arancio O, Nixon RA. Calpain mediates calcium-induced activation of the Erk1,2 MAPK pathway and cytoskeletal phosphorylation in neurons: relevance to Alzheimer’s disease. AM J Path 2004; 165:795-805.

Carter TL, Verdile Giuseppe, Groth D, Bogush A, Thomas S, Shen P, Fraser PE, Mathews P, Nixon RA, Ehrlich ME, Kwok JB, St. George-Hyslop P, Schofield P, Li Y, Yang A, Martins RN, Gandy S. Alzheimer amyloid precursor aspartyl proteinase activity in CHAPSO homogenates of Spodoptera frugiperda cells. Alzheimer Dis Assoc Disord 2004; 18:261-263.

Nixon RA, Wegiel J, Kumar A, Yu WH, Peterhoff C, Cataldo A, Cuervo AM. Extensive involvement of autophagy in Alzheimer disease: an immuno-electron microscopy study.  J Neuropathol Exp Neurol 2005; 64:113-122.

Falangola MF, Lee S-P, Nixon RA, Duff K, Helpern JA.  Histological co-localization of iron in Aß plaques of PS/APP Transgenic Mice. Neurochem Res 2005; 30:201-205.

Mastrangelo P, Mathews PM, Chishti MA, Schmidt SD, Gu Y, Yang J, Mazella MJ, Coomaraswamy J, Horne P, Strome B, Pelly H, Levesque G, Eberling C, Jiang Y, Nixon RA, Rozmahel R, Fraser PE, St George-Hyslop P, Carlson GA, Westaway D. Dissociated phenotypes in presenilin transgenic mice define functionally distinct gamma-secretases. Proc Nat Acad Sci USA 2005; 102:8972-8977.

de Grey AD, Alvarez PJ, Brady RO, Cuervo AM, Jerome WG, McCarty PL, Nixon RA, Rittmann BE, Sparrow JR.  Medical bioremediation: prospects for the application of microbial catabolic diversity to aging and several major age-related diseases.  Ageing Res Rev 2005 4:315-338.

Yu WH, Cuervo AM, Kumar A, Peterhoff CM, Schmidt SD, Lee JH, Mohan PS, Mercken M, Farmery MR, Tjernberg LO, Jiang Y, Duff K, Uchiyama Y, Naslund J, Mathews PM, Cataldo AM, Nixon RA. Macroautophagy– a novel amyloid-ß (Aß) peptide-generating pathway activated in Alzheimer’s disease. J Cell Biology 2005; 171:87-98.

Yuan A, Nixon RA, Rao MV.  Deleting the phosphorylated tail domain of the neurofilament heavy subunit does not alter neurofilament transport rate in vivo. Neurosci Lett 2006; 393:264-268.

Yuan A, Rao MV, Chen Y, Kumar A, Veeranna , Peterson A, Julien J-P, Nixon RA. a-internexin is structurally and functionally associated with the neurofilament triplet proteins in the mature CNS. JNeurosci (submitted).

Invited Reviews and Chapters

Nixon RA. Proteolysis of neurofilaments. In: Marotta CA , ed. Neurofilaments. Minneapolis : University of Minnesota Press, 1983; 17 54. 

Pope A, Nixon RA. Proteases of human brain. In: Neurochem Res 1984; 9:291 323.

Nixon RA. Axonal transport of cytoskeletal proteins. In: Burgoyne RD , ed. The Neuronal Cytoskeleton. New York : Wiley Liss, Inc., 1991; 283 307.

Nixon RA, Sihag RK. Neurofilament phosphorylation: A new look at regulation and function. Trends Neurosci 1991; 14:501 6.

Nixon RA. Slow axonal transport. In: Current Opinion in Cell Biology 1992; 4:8 14.

Nixon RA, Shea TB. Dynamics of neuronal intermediate filaments: A developmental perspective. In Cell Motil Cytoskel 1992; 22:81-91.

Nixon RA. The regulation of neurofilament protein dynamics by phosphorylation: Clues to neurofibrillary pathobiology. Brain Pathol 1993; 3:29 38.

Nixon RA, Cataldo AM. Free radicals, proteolysis and the degeneration of neurons in Alzheimer disease: How essential is the ß-amyloid link? Neurobiol Aging 1994; 15:463 69.

Nixon RA, Saito K I, Grynspan F, Griffin WR, Katayama S, Honda T, Mohan PS, Shea TB, Beermann ML. Calcium activated neutral proteinase (calpain) system in aging and Alzheimer disease. Ann N Y Acad Sci 1994; 747:77 91.

Nixon RA. Neuronal degenerative mechanisms as clues to pathogenesis and treatment of Alzheimer's disease. Neurobiol Aging 1994; 15:S61 S65. 

Nixon RA, Cataldo AM. The endosomal lysosomal system in Alzheimer disease. Intl Comm on Proteolysis Newsletter, Feb. 1995.

NixonRA, Cataldo AM. The endosomal lysosomal system of neurons: New roles. Trends Neurosci 1995; 18:489-96.

Nixon RA. The dynamic behavior and organization of cytoskeletal proteins in neurons: Reconciling old and new findings. Bioessays 1998; 20:798-807.

Nixon RA. Slow axonal transport of cytoskeletal proteins. Current Opinion in Cell Biol 1998; 10:87-92. 

Nixon RA. The slow axonal transport debate. Trends in Cell Biology 1998; 8:100.

Nixon RA, Albert MS. Disorders of cognition. In: The Harvard Guide to Modern Psychiatry. Nicoli A (ed.) Harvard University Press, Cambridge , 1998; 328-361.

Nixon RA. “Neurofilaments” In: Encyclopedia of Molecular Biology. Creighton TE (ed.) Wiley, New York , 1999; 1589-1595.

Nixon RA, Mohan PS. “Calpains in the pathogenesis of Alzheimer’s disease” In: Calpain: Pharmacology and Toxicology of Calcium-Dependent Protease. Taylor & Francis, Ann Arbor   1999, 267-291.

Nixon RA, Cataldo AM, Mathews PM. The endosomal-lysosomal system of neurons in Alzheimer’s disease pathogenesis: a review. Neurochem Res 2000; 25:1161-1172.

Nixon RA. A ‘protease activation cascade’ in the pathogenesis of Alzheimer’s disease. In: Alzheimer’s Disease: A Compendium of Current Theories. Khachaturian ZS and Mesulam MM (eds.) Ann NY Acad Sci 2000; 924:117-131.

Nixon RA and Rao MV. Neurofilaments. In: Encyclopedia of Molecular Medicine. Creighton TE (ed.). John Wiley & Sons, Inc., New York 2001; 1589-1595. 

Nixon RA. Neural circuitry and signaling in dementia and Alzheimer’s disease. In: Brain Circuitry and Signaling in Psychiatry: Basic Science and Clinical Implications. Kaplan GB and Hammer RP, Jr. (eds.) American Psychiatric Publishing, Inc., WDC, 2002; 201-226.

Nixon RA. Cell and molecular neuropathology of Alzheimer’s disease. In: Neuropsycho- pharmacology: The Fifth Generation of Progress; Davis KL, Charney D, Coyle JT, and Nemeroff C (eds.) American College of Neuropsychopharmacology 2002; Lippincott Williams & Wilkins, Philadelphia, PA.

Tatton W, Chen D, Chalmers-Redman R, Wheeler L, Nixon R, Tatton N. Nixon RA. Hypothesis for a common basis for neuroprotection in glaucoma and Alzheimer’s disease: anti-apoptosis by alpha-2-adrenergic receptor activation. Survey of Ophthalmology 2003; 48: Supplement 1 p:S25-S37.

Rao MV and Nixon RA. Defective neurofilament transport in mouse models of amyotrophic lateral sclerosis: A review. Neurochem Res 2003; 28:1041-1047.

Matthews PM, Nixon RA. Setback for an Alzheimer’s disease vaccine: Lessons learned. Neurology 2003; 61(1):7/8. 

Nixon RA. The calpains in aging and aging-related diseases. Ageing  Research Reviews 2003; 2:407-418.

Nixon RA.  Niemann-Pick Type C disease and Alzheimer’s disease: The APP-endosome connection fattens up. American Journal of Pathology 2004; 164(3): 757-61.

Nixon RA. Endosome function and dysfunction in Alzheimer’s disease and other neurodegenerative diseases. Neurobiology of Aging 2005; 26:373-382.

Schmidt SD , Nixon RA, Mathews PM. ELISA method for measurement of amyloid-b levels. Methods in Molecular Biology: Amyloid Proteins, Methods and Protocols (Sigurdsson EM, editor), The Humana Press, Inc., Totowa, NJ 2005; 299:279-297. 

Schmidt SD , Jiang Y, Nixon RA, Mathews PM. Tissue processing prior to protein analysis and amyloid-b quantitation.  Methods in Molecular Biology: Amyloid Proteins, Methods and Protocols  (Sigurdsson EM, editor), The Humana Press, Inc., Totowa , NJ , 2005; 299:267-278.

Nixon RA and Cataldo AM. Lysosomal system pathways: Genes to neurodegeneration in Alzheimer’s disease. J Alzheimer’s Dis 2006; 7:1-13.

Nixon RA.  Autophagy in Neurodegenerative Disease:  Friend, Foe, or Turncoat? Trends in Neurosci 2006 (in press). 

Cataldo AM and Nixon RA. Neuronal trafficking in Alzheimer disease Niemann- Pick Type C disease. Neuro Biol Aging 2006; (in press).

Proceedings of Meetings

Nixon RA, Logvinenko KB, Fisher WH. Evidence for a stationary nonuniform network of   neurofilaments along retinal ganglion cell axons. Ann N Y Acad Sci 1985; 455:797 800.

Nixon RA. The axonal transport of cytoskeletal proteins: A reappraisal. In: Bisby MA, Smith RS, eds. Axonal Transport. New York : Alan R. Liss, Inc., 1987;175 200.

Nixon RA, Lewis SE. Phosphorylation and dephosphorylation of neurofilament proteins in retinal ganglion cell neurons in vivo. In: Ehrlich YH, Lenox RH, Kornecki E, Berry WO, eds. Adv Exp Med Biol: Molecular Mechanisms of Neuronal Responsiveness. New York : Plenum Press, 1987;167 86.

Nixon RA. Calcium activated neutral proteinases as regulators of cellular function: Implications for Alzheimer's disease pathogenesis. Ann N Y Acad Sci 1989; 568:198 206.

Sihag RK, Nixon RA. Evidence for site  and domain specific phosphorylation of the 145 kDa neurofilament subunit in vivo. In: Heilmeyer, Jr, LMG, Cellular Regulation by Protein Phosphorylation. Heidelberg : Springer Verlag NATO ASI Series 1991;H56:465 9.

Nixon RA, Cataldo AM. Lysosomal proteolysis in Alzheimer brain: Possible roles in neuronal cell death and amyloid formation. In: Ishii T, Allsop D, Selkoe DJ, eds. Frontiers of Alzheimer Research. Amsterdam : Elsevier Press, 1991:133 46.

Nixon RA, Cataldo AM, Paskevich PA, Hamilton DJ, Wheelock TR, Kanaley Andrews L. The lysosomal system in neurons: Involvement at multiple stages of Alzheimer's disease pathogenesis. Ann N Y Acad Sci 1992;674:65 88.

Saito K I, Elce JS, Hamos JE, Nixon RA. Widespread activation of calcium activated neutral proteinase (calpain) in the brain in Alzheimer disease: A potential molecular basis for neuronal degeneration. Proc Nat Acad Sci USA 1993; 90:2628 32.

Nixon RA, Cataldo AM. The lysosomal system in neuronal cell death: A review. Ann N Y Acad Sci. 1993;679:87 109.

Nixon RA, Cataldo A, Mann DMA, Paskevich PA, Hamilton DJ, Wheelock TR. Abnormalities of lysosomal proteolysis in neurons in Alzheimer's disease and Down's syndrome: Possible relationship to ß amyloid deposition. In: Corain B, Iqbal K, Nicolini M, Winblad B, Wisniewski H, Zatta, P, eds. Alzheimer's Disease: Advances in Clinical and Basic Research. New York : John Wiley & Sons Ltd., 1993; 441 50.

Cataldo AM, Hamilton DJ, Barnett JL,Paskevich PA, Nixon RA. Abnormalities of the endosomal-lysosomal system in Alzheimer's disease: Relationship to disease pathogenesis. In: Intracellular Protein Catabolism. Bond JS, Suzuki K, (eds.) Adv Exp Med Biol 1996 389:271- 280.

Nixon RA, Mathews PM, Cataldo AM, Mohan PS, Schmidt SD, Duff K, Berg M, Marks N, Peterhoff C, Sershen H. In vivo perturbation of lysosomal function promotes neurodegeneration in the PS1M146V/APPK670N,M671L mouse model of Alzheimer’s disease pathology. In: Alzheimer’s Disease: Advances in Etiology, Pathogenesis and Therapeutics. Iqbal K, Sisodia SS, and Winblad B (eds.) John Wiley & Sons, Ltd., New York , 2001; 687-696.

Mathews PM, Guerra CB, Jiang Y, Kao BH, Dinakar R, Mehta P, Cataldo AM, Nixon RA. Accelerated Aß generation in a cell model of Alzheimer’s disease-related endosomal-lysosomal system upregulation. In: Alzheimer’s Disease: Advances in Etiology, Pathogenesis and Therapeutics. Iqbal K, Sisodia SS, and Winblad B (eds.) John Wiley & Sons, Ltd., New York , 2001; 461-467.

Books and Monographs

Nixon RA. Studies on the neurotoxicity of a non metabolized amino acid, 1 amino 1. Banner CDB, Nixon RA, Eds. Proteases and protease inhibitors in Alzheimer's disease pathogenesis. Ann N Y Acad Sci 1992; Vol. 674, 259 pages.

Nixon RA, Yu WH, Cuervo AM, Kumar A, Yang D-S, Peterhoff C.  Macroautophagy in Alzheimer’s disease: failure of a neuroprotective mechanism.  In: Alzheimer’s Disease and Related Disorders: Clinical and Basic Research Advances.  Iqbal K and Winblad B (eds.) In Press.