Natural animal models of neurodegenerative protein misfolding diseases

Current Pharmaceutical Design

Volumn 18, Issue 8, 2012, Pages 1148-1158

  Moreno Gonzalez, Ines ^a   Soto, Claudio ^a  

^a UNIVERSITY OF TEXAS MEDICAL SCHOOL   (United States)

Author keywords

Alzheimer's disease; Amyloid; Natural model; Non transgenic; Prion; Protein misfolding

Indexed keywords

AGING; ALZHEIMER DISEASE; AMYOTROPHIC LATERAL SCLEROSIS; ARTICLE; BEAR; BOVINE SPONGIFORM ENCEPHALOPATHY; BRAIN DISEASE; BRAIN SPONGIOSIS; CAT; CHEETAH; CHICKEN; CHRONIC WASTING DISEASE; DEGENERATIVE DISEASE; DOG; EXOTIC UNGULATE ENCEPHALOPATHY; EXPERIMENTAL ANIMAL; FELINE SPONGIFORM ENCEPHALOPATHY; GOAT; GUINEA PIG; HUMAN; HUNTINGTON CHOREA; NONHUMAN; PARKINSON DISEASE; PRION DISEASE; PRIORITY JOURNAL; PROTEIN FOLDING; SCRAPIE; SHEEP; TRANSGENIC ANIMAL; TRANSGENIC MOUSE; TRANSGENIC RAT; TRANSMISSIBLE MINK ENCEPHALOPATHY; WOLVERINE;

AGING; ALZHEIMER DISEASE; ANIMALS; BRAIN; DISEASE MODELS, ANIMAL; HUMANS; MICE; MICE, TRANSGENIC; NEURODEGENERATIVE DISEASES; PRION DISEASES; PROTEIN FOLDING; SPECIES SPECIFICITY;

EID: 84857131037     PISSN: 13816128     EISSN: 18734286     Source Type: Journal    
DOI: 10.2174/138161212799315768     Document Type: Article

References (186)

1. Soto C. Unfolding the role of protein misfolding in neurodegenerative diseases. Nat Rev Neurosci 2003; 4(1): 49-60.
2. Moreno-Gonzalez I, Soto C. Misfolded protein aggregates: Mechanisms, structures and potential for disease transmission. Semin Cell Dev Biol 2011; 22(5): 482-487.
3. Stromer T, Serpell LC. Structure and morphology of the Alzheimer's amyloid fibril. Microsc Res Tech 2005; 67(3-4): 210-7.
4. Cheon M, Chang I, Mohanty S, et al. Structural reorganisation and potential toxicity of oligomeric species formed during the assembly of amyloid fibrils. PLoS Comput Biol 2007; 3(9): 1727-1738.
5. Caughey B, Lansbury PT. Protofibrils, pores, fibrils, and neurodegeneration: separating the responsible protein aggregates from the innocent bystanders. Annu Rev Neurosci 2003; 26: 267-98.
6. Walsh DM, Selkoe DJ. A beta oligomers -a decade of discovery. J Neurochem 2007; 101(5): 1172-84.
7. Jarrett JT, Lansbury PT, Jr. Seeding "one-dimensional crystallization" of amyloid: a pathogenic mechanism in Alzheimer's disease and scrapie? Cell 1993; 73(6): 1055-8.
8. Soto C, Estrada L, Castilla J. Amyloids, prions and the inherent infectious nature of misfolded protein aggregates. Trends Biochem Sci 2006; 31(3): 150-5.
9. Wyss-Coray T, Mucke L. Inflammation in Neurodegenerative Disease -A double-edged sword. Neuron 2002; 35: 419-32.
10. McGeer PL, McGeer EG. Glial cell reactions in neurodegenerative diseases: pathophysiology and therapeutic interventions. Alzheimer Dis Assoc Disord 1998; 12 Suppl 2: S1-S6.
11. Haass C, Selkoe DJ. Soluble protein oligomers in neurodegeneration: lessons from the Alzheimer's amyloid beta-peptide. Nat Rev Mol Cell Biol 2007; 8(2): 101-12.
12. Jucker M. The benefits and limitations of animal models for translational research in neurodegenerative diseases. Nat Med 2010; 16(11): 1210-1214.
13. Telling GC. Transgenic mouse models of prion diseases. Methods Mol Biol 2008; 459: 249-63.
14. Van Leuven F. Single and multiple transgenic mice as models for Alzheimer's disease. Prog Neurobiol 2000; 61(3): 305-12.
15. Hsiao K, Chapman P, Nilsen S, et al. Correlative memory deficits, Abeta elevation, and amyloid plaques in transgenic mice. Science 1996; 274(5284): 99-102.
16. Selkoe DJ. Alzheimer's disease: genotypes, phenotypes, and treatments. Science 1997; 275(5300): 630-1.
17. Mangiarini L, Sathasivam K, Seller M, et al. Exon 1 of the HD gene with an expanded CAG repeat is sufficient to cause a progressive neurological phenotype in transgenic mice. Cell 1996; 87(3): 493-506.
18. Masliah E, Rockenstein E, Veinbergs I, et al. Dopaminergic loss and inclusion body formation in alpha-synuclein mice: implications for neurodegenerative disorders. Science 2000; 287(5456): 1265-9.
19. Prusiner SB. Prions. Proc Natl Acad Sci USA 1998; 95(23): 13363-83.
20. Williams ES, Miller MW. Transmissible spongiform encephalopathies in non-domestic animals: origin, transmission and risk factors. Rev Sci Tech 2003; 22(1): 145-56.
21. Foster JD, Parnham D, Chong A, Goldmann W, Hunter N. Clinical signs, histopathology and genetics of experimental transmission of BSE and natural scrapie to sheep and goats. Vet Rec 2001; 148(6): 165-71.
22. Williams ES. Scrapie and chronic wasting disease. Clin Lab Med 2003; 23(1): 139-159.
23. Baylis M, Goldmann W. The genetics of scrapie in sheep and goats. Curr Mol Med 2004; 4(4): 385-96.
24. Clouscard C, Beaudry P, Elsen JM, et al. Different allelic effects of the codons 136 and 171 of the prion protein gene in sheep with natural scrapie. J Gen Virol 1995; 76 (Pt 8): 2097-101.
25. Cullie J, Chelle PL. Experimental transmission of trembling to the goat. Comptes Rendus des Seances de l'Academie des Sciences 1939; 208: 1058-160.
26. Hoinville LJ. A review of the epidemiology of scrapie in sheep. Rev Sci Tech 1996; 15(3): 827-52.
27. Wells GA, Scott AC, Johnson CT, et al. A novel progressive spongiform encephalopathy in cattle. Vet Rec 1987; 121(18): 419-20.
28. Simmons MM, Harris P, Jeffrey M, Meek SC, Blamire IW, Wells GA. BSE in Great Britain: consistency of the neurohistopathological findings in two random annual samples of clinically suspect cases. Vet Rec 1996; 138(8): 175-7.
29. Jeffrey M, Gonzalez L. Pathology and pathogenesis of bovine spongiform encephalopathy and scrapie. Curr Top Microbiol Immunol 2004; 284: 65-97.
30. Fraser H, Bruce ME, Chree A, McConnell I, Wells GA. Transmission of bovine spongiform encephalopathy and scrapie to mice. J Gen Virol 1992; 73 (Pt 8): 1891-7.
31. Kao RR, Gravenor MB, Baylis M, et al. The potential size and duration of an epidemic of bovine spongiform encephalopathy in British sheep. Science 2002; 295(5553): 332-5.
32. Bradley R. Bovine spongiform encephalopathy. Update. Acta Neurobiol Exp (Wars) 2002; 62(3): 183-95.
33. Lasmezas CI, Deslys JP, Demaimay R, et al. BSE transmission to macaques. Nature 1996; 381(6585): 743-4.
34. Collinge J, Sidle KC, Meads J, Ironside J, Hill AF. Molecular analysis of prion strain variation and the aetiology of 'new variant' CJD. Nature 1996; 383(6602): 685-90.
35. Bruce ME, Will RG, Ironside JW, et al. Transmissions to mice indicate that 'new variant' CJD is caused by the BSE agent. Nature 1997; 389(6650): 498-501.
36. Scott MR, Will R, Ironside J, et al. Compelling transgenetic evidence for transmission of bovine spongiform encephalopathy prions to humans. Proc Natl Acad Sci USA 1999; 96(26): 15137-42.
37. Williams ES, Young S. Chronic wasting disease of captive mule deer: a spongiform encephalopathy. J Wildl Dis 1980; 16(1): 89-98.
38. Sigurdson CJ, Aguzzi A. Chronic wasting disease. Biochim Biophys Acta 2007; 1772(6): 610-8.
39. Race RE, Raines A, Baron TG, Miller MW, Jenny A, Williams ES. Comparison of abnormal prion protein glycoform patterns from transmissible spongiform encephalopathy agent-infected deer, elk, sheep, and cattle. J Virol 2002; 76(23): 12365-8.
40. Belay ED, Gambetti P, Schonberger LB, et al. Creutzfeldt-Jakob disease in unusually young patients who consumed venison. Arch Neurol 2001; 58(10): 1673-8.
41. Xie Z, O'Rourke KI, Dong Z, et al. Chronic wasting disease of elk and deer and Creutzfeldt-Jakob disease: Comparative analysis of the scrapie prion protein. J Biol Chem 2005; 281: 4199-205.
42. Marsh RF, Kincaid AE, Bessen RA, Bartz JC. Interspecies Transmission of Chronic Wasting Disease Prions to Squirrel Monkeys (Saimiri sciureus). J Virol 2005; 79(21): 13794-6.
43. Race B, Meade-White KD, Miller MW, et al. Susceptibilities of nonhuman primates to chronic wasting disease. Emerg Infect Dis 2009; 15(9): 1366-76.
44. Kong Q, Huang S, Zou W, et al. Chronic wasting disease of elk: transmissibility to humans examined by transgenic mouse models. J Neurosci 2005; 25(35): 7944-9.
45. Tamguney G, Giles K, Bouzamondo-Bernstein E, et al. Transmission of elk and deer prions to transgenic mice. J Virol 2006; 80(18): 9104-14.
46. Sandberg MK, Al-Doujaily H, Sigurdson CJ, et al. Chronic wasting disease prions are not transmissible to transgenic mice overexpressing human prion protein. J Gen Virol 2010; 91(Pt 10): 2651-7.
47. Barria MA, Telling GC, Gambetti P, Mastrianni JA, Soto C. Generation of a New Form of Human PrPSc in vitro by Interspecies Transmission from Cervid Prions. J Biol Chem 2011; 286(9): 7490-5.
48. Hartsough GR, Burger D. Encephalopathy of mink. I. Epizootiologic and clinical observations. J Infect Dis 1965; 115(4): 387-92.
49. Barlow RM. Transmissible mink encephalopathy: pathogenesis and nature of the aetiological agent. J Clin Pathol Suppl (R Coll Pathol) 1972; 6: 102-9.
50. Marsh RF, Bessen RA, Lehmann S, Hartsough GR. Epidemiological and experimental studies on a new incident of transmissible mink encephalopathy. J Gen Virol 1991; 72 (Pt 3): 589-94.
51. Sigurdson CJ, Miller MW. Other animal prion diseases. Br Med Bull 2003; 66: 199-212.
52. Bessen RA, Marsh RF. Identification of two biologically distinct strains of transmissible mink encephalopathy in hamsters. J Gen Virol 1992; 73 (Pt 2): 329-34.
53. Pearson GR, Gruffydd-Jones TJ, Wyatt JM, et al. Feline spongiform encephalopathy. Vet Rec 1991; 128(22): 532.
54. Eiden M, Hoffmann C, Balkema-Buschmann A, Muller M, Baumgartner K, Groschup MH. Biochemical and immunohistochemical characterization of feline spongiform encephalopathy in a German captive cheetah. J Gen Virol 2010; 91(Pt 11): 2874-83.
55. Kirkwood JK, Wells GA, Wilesmith JW, Cunningham AA, Jackson SI. Spongiform encephalopathy in an arabian oryx (Oryx leucoryx) and a greater kudu (Tragelaphus strepsiceros). Vet Rec 1990; 127(17): 418-20.
56. Cunningham AA, Wells GA, Scott AC, Kirkwood JK, Barnett JE. Transmissible spongiform encephalopathy in greater kudu (Tragelaphus strepsiceros). Vet Rec 1993; 132(3): 68.
57. Kirkwood JK, Cunningham AA, Austin AR, Wells GA, Sainsbury AW. Spongiform encephalopathy in a greater kudu (Tragelaphus strepsiceros) introduced into an affected group. Vet Rec 1994; 134(7): 167-8.
58. Bons N, Mestre-Frances N, Charnay Y, Tagliavini F. Spontaneous spongiform encephalopathy in a young adult rhesus monkey. Lancet 1996; 348(9019): 55.
59. Gajdusek DC, Gibbs CJ, Alpers M. Experimental transmission of a Kuru-like syndrome to chimpanzees. Nature 1966; 209(25): 794-6.
60. Zlotnik I, Grant DP, Dayan AD, Earl CJ. Transmission of Creutzfeldt-Jakob disease from man to squirrel monkey. Lancet 1974; 2(7878): 435-8.
61. Goedert M, Spillantini MG. A century of Alzheimer's disease. Science 2006; 314(5800): 777-81.
62. Hiltunen M, van GT, Jolkkonen J. Functional roles of amyloid-beta protein precursor and amyloid-beta peptides: evidence from experimental studies. J Alzheimers Dis 2009; 18(2): 401-12.
63. Selkoe DJ. Cell biology of the beta-amyloid precursor protein and the genetics of Alzheimer's disease. Cold Spring Harb Symp Quant Biol 1996; 61: 587-96.
64. Brandt R, Hundelt M, Shahani N. Tau alteration and neuronal degeneration in tauopathies: mechanisms and models. Biochim Biophys Acta 2005; 1739(2-3): 331-54.
65. Medeiros R, Baglietto-Vargas D, LaFerla FM. The Role of Tau in Alzheimer's Disease and Related Disorders. CNS Neurosci Ther 2010.
66. Duff K. Transgenic mouse models of Alzheimer's disease: phenotype and mechanisms of pathogenesis. Biochem Soc Symp 2001; (67): 195-202.
67. Tomasello M, Kaminski J. Behavior. Like infant, like dog. Science 2009; 325(5945): 1213-4.
68. Head E. Brain aging in dogs: parallels with human brain aging and Alzheimer's disease. Vet Ther 2001; 2(3): 247-60.
69. Head E. Neurobiology of the aging dog. Age (Dordr) 2010.
70. Cotman CW, Head E. The canine (dog) model of human aging and disease: dietary, environmental and immunotherapy approaches. J Alzheimers Dis 2008; 15(4): 685-707.
71. Bosch MN, Pugliese M, Gimeno-Bayon J, Rodriguez MJ, Mahy N. Dogs with Cognitive Dysfunction Syndrome: A Natural Model of Alzheimer's Disease. Curr Alzheimer Res 2011.
72. Selkoe DJ, Bell DS, Podlisny MB, Price DL, Cork LC. Conservation of brain amyloid proteins in aged mammals and humans with Alzheimer's disease. Science 1987; 235(4791): 873-7.
73. Johnstone EM, Chaney MO, Norris FH, Pascual R, Little SP. Conservation of the sequence of the Alzheimer's disease amyloid peptide in dog, polar bear and five other mammals by cross-species polymerase chain reaction analysis. Brain Res Mol Brain Res 1991; 10(4): 299-305.
74. Okuda R, Uchida K, Tateyama S, Yamaguchi R, Nakayama H, Goto N. The distribution of amyloid beta precursor protein in canine brain. Acta Neuropathol (Berl) 1994; 87(2): 161-7.
75. Takeuchi Y, Uetsuka K, Murayama M, et al. Complementary distributions of amyloid-beta and neprilysin in the brains of dogs and cats. Vet Pathol 2008; 45(4): 455-66.
76. Sarasa M, Pesini P. Natural non-trasgenic animal models for research in Alzheimer's disease. Curr Alzheimer Res 2009; 6(2): 171-8.
77. Sarasa L, Gallego C, Monleon I, et al. Cloning, sequencing and expression in the dog of the main amyloid precursor protein isoforms and some of the enzymes related with their processing. Neuroscience 2010; 171(4): 1091-101.
78. Wisniewski H, Johnson AB, Raine CS, Kay WJ, Terry RD. Senile plaques and cerebral amyloidosis in aged dogs. A histochemical and ultrastructural study. Lab Invest 1970; 23(3): 287-96.
79. Cummings BJ, Su JH, Cotman CW, White R, Russell MJ. Betaamyloid accumulation in aged canine brain: a model of early plaque formation in Alzheimer's disease. Neurobiol Aging 1993; 14(6): 547-60.
80. Cummings BJ, Head E, Ruehl W, Milgram NW, Cotman CW. The canine as an animal model of human aging and dementia. Neurobiol Aging 1996; 17(2): 259-68.
81. Czasch S, Paul S, Baumgartner W. A comparison of immunohistochemical and silver staining methods for the detection of diffuse plaques in the aged canine brain. Neurobiol Aging 2006; 27(2): 293-305.
82. Tekirian TL, Cole GM, Russell MJ, et al. Carboxy terminal of betaamyloid deposits in aged human, canine, and polar bear brains. Neurobiol Aging 1996; 17(2): 249-57.
83. Pop V, Head E, Berchtold NC, et al. Abeta aggregation profiles and shifts in APP processing favor amyloidogenesis in canines. Neurobiol Aging 2010.
84. Head E, Pop V, Sarsoza F, et al. Amyloid-beta peptide and oligomers in the brain and cerebrospinal fluid of aged canines. J Alzheimers Dis 2010; 20(2): 637-46.
85. Gonzalez-Martinez A, Rosado B, Pesini P, et al. Plasma betaamyloid peptides in canine aging and cognitive dysfunction as a model of Alzheimer's disease. Exp Gerontol 2011; 46(7): 590-6.
86. Cummings BJ, Satou T, Head E, et al. Diffuse plaques contain Cterminal A beta 42 and not A beta 40: evidence from cats and dogs. Neurobiol Aging 1996; 17(4): 653-9.
87. Colle M-A, Hauw J-J, Crespeau F, et al. Vascular and parenchymal Abeta deposition in the aging dog: correlation with behavior. Neurobiol Aging 2000; 21(5): 695-704.
88. Braak H, Braak E, Strothjohann M. Abnormally phosphorylated tau protein related to the formation of neurofibrillary tangles and neuropil threads in the cerebral cortex of sheep and goat. Neurosci Lett 1994; 171(1-2): 1-4.
89. Yu CH, Song GS, Yhee JY, et al. Histopathological and immunohistochemical comparison of the brain of human patients with Alzheimer's disease and the brain of aged dogs with cognitive dysfunction. J Comp Pathol 2011; 145(1): 45-58.
90. Wegiel J, Wisniewski HM, Soltysiak Z. Region-and cell-typespecific pattern of tau phosphorylation in dog brain. Brain Res 1998; 802(1-2): 259-66.
91. Giaccone G, Verga L, Finazzi M, et al. Cerebral preamyloid deposits and congophilic angiopathy in aged dogs. Neurosci Lett 1990; 114(2): 178-83.
92. Shimada A, Kuwamura M, Awakura T, et al. Topographic relationship between senile plaques and cerebrovascular amyloidosis in the brain of aged dogs. J Vet Med Sci 1992; 54(1): 137-44.
93. Shimada A, Kuwamura M, Awakura T, Umemura T, Itakura C. An immunohistochemical and ultrastructural study on age-related astrocytic gliosis in the central nervous system of dogs. J Vet Med Sci 1992; 54(1): 29-36.
94. Morys J, Narkiewicz O, Maciejewska B, Wegiel J, Wisniewski HM. Amyloid deposits and loss of neurones in the claustrum of the aged dog. Neuroreport 1994; 5(14): 1825-8.
95. Tapp PD, Siwak CT, Gao FQ, et al. Frontal lobe volume, function, and beta-amyloid pathology in a canine model of aging. J Neurosci 2004; 24(38): 8205-13.
96. Bernedo V, Insua D, Suarez ML, Santamarina G, Sarasa M, Pesini P. Beta-amyloid cortical deposits are accompanied by the loss of serotonergic neurons in the dog. J Comp Neurol 2009; 513(4): 417-29.
97. Insua D, Suarez ML, Santamarina G, Sarasa M, Pesini P. Dogs with canine counterpart of Alzheimer's disease lose noradrenergic neurons. Neurobiol Aging 2010; 31(4): 625-35.
98. Pugliese M, Gangitano C, Ceccariglia S, et al. Canine cognitive dysfunction and the cerebellum: acetylcholinesterase reduction, neuronal and glial changes. Brain Res 2007; 1139: 85-94.
99. Head E, Mehta R, Hartley J, et al. Spatial learning and memory as a function of age in the dog. Behav Neurosci 1995; 109(5): 851-8.
100. Adams B, Chan A, Callahan H, et al. Use of a delayed nonmatching to position task to model age-dependent cognitive decline in the dog. Behav Brain Res 2000; 108(1): 47-56.
101. Studzinski CM, Araujo JA, Milgram NW. The canine model of human cognitive aging and dementia: pharmacological validity of the model for assessment of human cognitive-enhancing drugs. Prog Neuropsychopharmacol Biol Psychiatry 2005; 29(3): 489-98.
102. Cummings BJ, Pike CJ, Shankle R, Cotman CW. Beta-amyloid deposition and other measures of neuropathology predict cognitive status in Alzheimer's disease. Neurobiol Aging 1996; 17(6): 921-33.
103. Cotman CW, Head E, Muggenburg BA, Zicker S, Milgram NW. Brain aging in the canine: a diet enriched in antioxidants reduces cognitive dysfunction. Neurobiol Aging 2002; 23(5): 809-18.
104. Head E, Pop V, Vasilevko V, et al. A two-year study with fibrillar beta-amyloid (Abeta) immunization in aged canines: effects on cognitive function and brain Abeta. J Neurosci 2008; 28(14): 3555-66.
105. Gunn-Moore D, Moffat K, Christie LA, Head E. Cognitive dysfunction and the neurobiology of ageing in cats. J Small Anim Pract 2007; 48(10): 546-53.
106. Nakamura S, Nakayama H, Kiatipattanasakul W, Uetsuka K, Uchida K, Goto N. Senile plaques in very aged cats. Acta Neuropathol (Berl) 1996; 91(4): 437-9.
107. Brellou G, Vlemmas I, Lekkas S, Papaioannou N. Immunohistochemical investigation of amyloid beta-protein (Abeta) in the brain of aged cats. Histol Histopathol 2005; 20(3): 725-31.
108. Head E, Moffat K, Das P, et al. Beta-amyloid deposition and tau phosphorylation in clinically characterized aged cats. Neurobiol Aging 2005; 26(5): 749-63.
109. Gunn-Moore DA, McVee J, Bradshaw JM, Pearson GR, Head E, Gunn-Moore FJ. Ageing changes in cat brains demonstrated by beta-amyloid and AT8-immunoreactive phosphorylated tau deposits. J Feline Med Surg 2006; 8(4): 234-42.
110. Nakamura S, Nakayama H, Kiatipattanasakul W, Uetsuka K, Uchida K, Goto N. Senile plaques in very aged cats. Acta Neuropathol 1996; 91(4): 437-9.
111. Papendick RE, Munson L, O'Brien TD, Johnson KH. Systemic AA amyloidosis in captive cheetahs (Acinonyx jubatus). Vet Pathol 1997; 34(6): 549-56.
112. Serizawa S, Chambers JK, Une Y. Beta Amyloid Deposition and Neurofibrillary Tangles Spontaneously Occur in the Brains of Captive Cheetahs (Acinonyx jubatus). Vet Pathol 2011.
113. Cork LC, Powers RE, Selkoe DJ, Davies P, Geyer JJ, Price DL. Neurofibrillary tangles and senile plaques in aged bears. J Neuropathol Exp Neurol 1988; 47(6): 629-41.
114. Uchida K, Yoshino T, Yamaguchi R, et al. Senile plaques and other senile changes in the brain of an aged American black bear. Vet Pathol 1995; 32(4): 412-4.
115. Roertgen KE, Parisi JE, Clark HB, Barnes DL, O'Brien TD, Johnson KH. A beta-associated cerebral angiopathy and senile plaques with neurofibrillary tangles and cerebral hemorrhage in an aged wolverine (Gulo gulo). Neurobiol Aging 1996; 17(2): 243-247.
116. Nelson PT, Greenberg SG, Saper CB. Neurofibrillary tangles in the cerebral cortex of sheep. Neurosci Lett 1994; 170(1): 187-90.
117. Nelson PT, Saper CB. Ultrastructure of neurofibrillary tangles in the cerebral cortex of sheep. Neurobiol Aging 1995; 16(3): 315-23.
118. Wang GP, Grundke-Iqbal I, Kascsak RJ, Iqbal K, Wisniewski HM. Alzheimer neurofibrillary tangles: monoclonal antibodies to inherent antigen(s). Acta Neuropathol (Berl) 1984; 62(4): 268-75.
119. Nelson PT, Saper CB. Injections of okadaic acid, but not betaamyloid peptide, induce Alz-50 immunoreactive dystrophic neurites in the cerebral cortex of sheep. Neurosci Lett 1996; 208(2): 77-80.
120. Woodruff-Pak DS, Papka M, Romano S, Li YT. Eyeblink classical conditioning in Alzheimer's disease and cerebrovascular dementia. Neurobiol Aging 1996; 17(4): 505-12.
121. Sparks DL, Scheff SW, Hunsaker JC, III, Liu H, Landers T, Gross DR. Induction of Alzheimer-like beta-amyloid immunoreactivity in the brains of rabbits with dietary cholesterol. Exp Neurol 1994; 126(1): 88-94.
122. Woodruff-Pak DS. Animal models of Alzheimer's disease: therapeutic implications. J Alzheimers Dis 2008; 15(4): 507-21.
123. Sparks DL, Schreurs BG. Trace amounts of copper in water induce beta-amyloid plaques and learning deficits in a rabbit model of Alzheimer's disease. Proc Natl Acad Sci USA 2003; 100(19): 11065-9.
124. Woodruff-Pak DS, Agelan A, Del VL. A rabbit model of Alzheimer's disease: valid at neuropathological, cognitive, and therapeutic levels. J Alzheimers Dis 2007; 11(3): 371-83.
125. Sparks DL. Intraneuronal beta-amyloid immunoreactivity in the CNS. Neurobiol Aging 1996; 17(2): 291-9.
126. Sparks DL, Kuo YM, Roher A, Martin T, Lukas RJ. Alterations of Alzheimer's disease in the cholesterol-fed rabbit, including vascular inflammation. Preliminary observations. Ann N Y Acad Sci 2000; 903: 335-44.
127. Sparks DL. The early and ongoing experience with the cholesterolfed rabbit as a model of Alzheimer's disease: the old, the new and the pilot. J Alzheimers Dis 2008; 15(4): 641-56.
128. Sparks DL, Ziolkowski C, Lawmaster T, Martin T. Influence of water quality on cholesterol-induced tau pathology: preliminary data. Int J Alzheimers Dis 2011; 2011: 987023.
129. Wisniewski HM, Sturman JA, Shek JW. Aluminum chloride induced neurofibrillary changes in the developing rabbit a chronic animal model. Ann Neurol 1980; 8(5): 479-90.
130. Wisniewski HM, Shek JW, Gruca S, Sturman JA. Aluminuminduced neurofibrillary changes in axons and dendrites. Acta Neuropathol 1984; 63(3): 190-7.
131. Beal MF, Mazurek MF, Ellison DW, Kowall NW, Solomon PR, Pendlebury WW. Neurochemical characteristics of aluminuminduced neurofibrillary degeneration in rabbits. Neuroscience 1989; 29(2): 339-46.
132. Kowall NW, Pendlebury WW, Kessler JB, Perl DP, Beal MF. Aluminum-induced neurofibrillary degeneration affects a subset of neurons in rabbit cerebral cortex, basal forebrain and upper brainstem. Neuroscience 1989; 29(2): 329-37.
133. Beck M, Muller D, Bigl V. Amyloid precursor protein in guinea pigs--complete cDNA sequence and alternative splicing. Biochim Biophys Acta 1997; 1351(1-2): 17-21.
134. Beck M, Bruckner MK, Holzer M, et al. Guinea-pig primary cell cultures provide a model to study expression and amyloidogenic processing of endogenous amyloid precursor protein. Neuroscience 2000; 95(1): 243-54.
135. Beck M, Bigl V, Rossner S. Guinea pigs as a nontransgenic model for APP processing in vitro and in vivo. Neurochem Res 2003; 28(3-4): 637-44.
136. Carrodeguas JA, Rodolosse A, Garza MV, et al. The chick embryo appears as a natural model for research in beta-amyloid precursor protein processing. Neuroscience 2005; 134(4): 1285-300.
137. Mileusnic R, Rose S. The chick as a model for the study of the cellular mechanisms and potential therapies for Alzheimer's disease. Int J Alzheimers Dis 2010; 2010.
138. Small DH, Nurcombe V, Moir R, et al. Association and release of the amyloid protein precursor of Alzheimer's disease from chick brain extracellular matrix. J Neurosci 1992; 12(11): 4143-50.
139. Solomonia RO, Morgan K, Kotorashvili A, McCabe BJ, Jackson AP, Horn G. Analysis of differential gene expression supports a role for amyloid precursor protein and a protein kinase C substrate (MARCKS) in long-term memory. Eur J Neurosci 2003; 17(5): 1073-81.
140. Small DH, Nurcombe V, Reed G, et al. A heparin-binding domain in the amyloid protein precursor of Alzheimer's disease is involved in the regulation of neurite outgrowth. J Neurosci 1994; 14(4): 2117-27.
141. Rose SP. God's organism? The chick as a model system for memory studies. Learn Mem 2000; 7(1): 1-17.
142. Vonsattel JP, Myers RH, Stevens TJ, Ferrante RJ, Bird ED, Richardson EP, Jr. Neuropathological classification of Huntington's disease. J Neuropathol Exp Neurol 1985; 44(6): 559-77.
143. A novel gene containing a trinucleotide repeat that is expanded and unstable on Huntington's disease chromosomes. The Huntington's Disease Collaborative Research Group. Cell 1993; 72(6): 971-83.
144. Nance MA, Mathias-Hagen V, Breningstall G, Wick MJ, McGlennen RC. Analysis of a very large trinucleotide repeat in a patient with juvenile Huntington's disease. Neurol 1999; 52(2): 392-4.
145. Brinkman RR, Mezei MM, Theilmann J, Almqvist E, Hayden MR. The likelihood of being affected with Huntington disease by a particular age, for a specific CAG size. Am J Hum Genet 1997; 60(5): 1202-10.
146. Hatters DM. Protein misfolding inside cells: the case of huntingtin and Huntington's disease. IUBMB Life 2008; 60(11): 724-8.
147. Menalled LB, Chesselet MF. Mouse models of Huntington's disease. Trends Pharmacol Sci 2002; 23(1): 32-9.
148. Kumar P, Kalonia H, Kumar A. Huntington's disease: pathogenesis to animal models. Pharmacol Rep 2010; 62(1): 1-14.
149. Yang SH, Cheng PH, Banta H, et al. Towards a transgenic model of Huntington's disease in a non-human primate. Nature 2008; 453(7197): 921-4.
150. Jacobsen JC, Bawden CS, Rudiger SR, et al. An ovine transgenic Huntington's disease model. Hum Mol Genet 2010; 19(10): 1873-82.
151. Shibuya H, Liu PC, O'Brien DP, Chen YW, Johnson GS. A BbvI mismatch PCR/RFLP marker for the canine huntingtin gene. Anim Genet 1998; 29(3): 239-40.
152. Spillantini MG, Schmidt ML, Lee VM, Trojanowski JQ, Jakes R, Goedert M. Alpha-synuclein in Lewy bodies. Nature 1997; 388(6645): 839-40.
153. Samii A, Nutt JG, Ransom BR. Parkinson's disease. Lancet 2004; 363(9423): 1783-93.
154. Venda LL, Cragg SJ, Buchman VL, Wade-Martins R. alpha-Synuclein and dopamine at the crossroads of Parkinson's disease. Trends Neurosci 2010; 33(12): 559-68.
155. Clayton DF, George JM. The synucleins: a family of proteins involved in synaptic function, plasticity, neurodegeneration and disease. Trends Neurosci 1998; 21(6): 249-54.
156. Jellinger KA. Neuropathological spectrum of synucleinopathies. Mov Disord 2003; 18 Suppl 6: S2-12.
157. Goedert M, Jakes R, Crowther RA, Hasegawa M, Smith MJ, Spillantini MG. Intraneuronal filamentous tau protein and alphasynuclein deposits in neurodegenerative diseases. Biochem Soc Trans 1998; 26(3): 463-71.
158. Trojanowski JQ, Lee VMY. Parkinson's disease and related alphasynucleinopathies are brain amyloidoses. Ann NY Acad Sci 2003; 991: 107-10.
159. Gandhi S, Wood NW. Molecular pathogenesis of Parkinson's disease. Human Molecular Genetics 2005; 14(18): 2749-55.
160. Bertoli-Avella AM, Oostra BA, Heutink P. Chasing genes in Alzheimer's and Parkinson's disease. Human Genetics 2004; 114(5): 413-38.
161. Sherman MY, Goldberg AL. Cellular defenses against unfolded proteins: a cell biologist thinks about neurodegenerative diseases. Neuron 2001; 29(1): 15-32.
162. Wilkinson KD. Ubiquitination and deubiquitination: targeting of proteins for degradation by the proteasome. Semin Cell Dev Biol 2000; 11(3): 141-8.
163. McNaught KS, Olanow CW, Halliwell B, Isacson O, Jenner P. Failure of the ubiquitin-proteasome system in Parkinson's disease. Nat Rev Neurosci 2001; 2(8): 589-94.
164. Hurley PJ, Elsworth JD, Whittaker MC, Roth RH, Redmond DE, Jr. Aged monkeys as a partial model for Parkinson's disease. Pharmacol Biochem Behav 2011; 99(3): 324-32.
165. Hurley PJ, Elsworth JD, Whittaker MC, Roth RH, Redmond DE, Jr. Aged monkeys as a partial model for Parkinson's disease. Pharmacol Biochem Behav 2011; 99(3): 324-32.
166. McFarlane D. Advantages and limitations of the equine disease, pituitary pars intermedia dysfunction as a model of spontaneous dopaminergic neurodegenerative disease. Ageing Res Rev 2007; 6(1): 54-63.
167. McFarlane D, Dybdal N, Donaldson MT, Miller L, Cribb AE. Nitration and increased alpha-synuclein expression associated with dopaminergic neurodegeneration in equine pituitary pars intermedia dysfunction. J Neuroendocrinol 2005; 17(2): 73-80.
168. Nakajima T, Takauchi S, Ohara K, et al. Alpha-synuclein-positive structures induced in leupeptin-infused rats. Brain Res 2005; 1040(1-2): 73-80.
169. Julien JP. Amyotrophic lateral sclerosis. unfolding the toxicity of the misfolded. Cell 2001; 104(4): 581-91.
170. Bruijn LI, Houseweart MK, Kato S, et al. Aggregation and motor neuron toxicity of an ALS-linked SOD1 mutant independent from wild-type SOD1. Science 1998; 281: 1851-4.
171. Rosen DR. Mutations in Cu/Zn superoxide dismutase gene are associated with familial amyotrophic lateral sclerosis. Nature 1993; 364(6435): 362.
172. Wong PC, Borchelt DR. Motor neuron disease caused by mutations in superoxide dismutase 1. Curr Opin Neurol 1995; 8(4): 294-301.
173. Kato S. Amyotrophic lateral sclerosis models and human neuropathology: similarities and differences. Acta Neuropathol 2008; 115(1): 97-114.
174. Messer A, Flaherty L. Autosomal dominance in a late-onset motor neuron disease in the mouse. J Neurogenet 1986; 3(6): 345-55.
175. Duchen LW, Strich SJ. An hereditary motor neurone disease with progressive denervation of muscle in the mouse: the mutant 'wobbler'. J Neurol Neurosurg Psychiatry 1968; 31(6): 535-42.
176. Shultz LD, Sweet HO, Davisson MT, Coman DR. 'Wasted', a new mutant of the mouse with abnormalities characteristic to ataxia telangiectasia. Nature 1982; 297(5865): 402-4.
177. Schmalbruch H, Jensen HJ, Bjaerg M, Kamieniecka Z, Kurland L. A new mouse mutant with progressive motor neuronopathy. J Neuropathol Exp Neurol 1991; 50(3): 192-204.
178. Sillevis Smitt PA, de Jong JM. Animal models of amyotrophic lateral sclerosis and the spinal muscular atrophies. J Neurol Sci 1989; 91(3): 231-58.
179. Pioro EP, Mitsumoto H. Animal models of ALS. Clin Neurosci 1995; 3(6): 375-85.
180. Doble A, Kennel P. Animal models of amyotrophic lateral sclerosis. Amyotroph Lateral Scler Other Motor Neuron Disord 2000; 1(5): 301-12.
181. Sack GH, Jr., Cork LC, Morris JM, Griffin JW, Price DL. Autosomal dominant inheritance of hereditary canine spinal muscular atrophy. Ann Neurol 1984; 15(4): 369-73.
182. Cork LC, Griffin JW, Munnell JF, Lorenz MD, Adams RJ. Hereditary canine spinal muscular atrophy. J Neuropathol Exp Neurol 1979; 38(3): 209-21.
183. Lorenz MD, Cork LC, Griffin JW, Adams RJ, Price DL. Hereditary spinal muscular atrophy in Brittany Spaniels: clinical manifestations. J Am Vet Med Assoc 1979; 175(8): 833-9.
184. Vandevelde M, Greene CE, Hoff EJ. Lower motor neuron disease with accumulation of neurofilaments in a cat. Vet Pathol 1976; 13(6): 428-35.
185. Cummings JF, de LA, George C, et al. Equine motor neuron disease; a preliminary report. Cornell Vet 1990; 80(4): 357-9.
186. Step DL, Cummings JF, de LA, et al. Motor neuron degeneration in a horse. J Am Vet Med Assoc 1993; 202(1): 86-8.