메뉴 건너뛰기




Volumn 286, Issue 1, 2011, Pages 1-20

Animal models of human genetic diseases: Do they need to be faithful to be useful?

Author keywords

Animal models of human genetic diseases; Mouse genetics; Rat genetics

Indexed keywords

ALLELE; DISEASE MODEL; EPISTASIS; EXPERIMENTAL MOUSE; EXPERIMENTAL RAT; GENE IDENTIFICATION; GENE INTERACTION; GENE MUTATION; GENETIC DISORDER; GENETIC MODEL; GENOME; HUMAN; HUMAN VERSUS ANIMAL COMPARISON; MAMMALIAN GENETICS; MULTIFACTORIAL INHERITANCE; NONHUMAN; PHENOTYPE; PRIORITY JOURNAL; REVIEW; TRANSGENIC ANIMAL; TRANSGENICS;

EID: 79955496674     PISSN: 16174615     EISSN: 16174623     Source Type: Journal    
DOI: 10.1007/s00438-011-0627-y     Document Type: Review
Times cited : (13)

References (105)
  • 11
    • 0034988226 scopus 로고    scopus 로고
    • The frissonnant mutant mouse, a model of dopamino-sensitive, inherited motor syndrome
    • DOI 10.1006/nbdi.2001.0393
    • Callizot N, Guénet JL, Baillet C, Warter JM, Poindron P (2001) The frissonnant mutant mouse, a model of dopamino-sensitive, inherited motor syndrome. Neurobiol Dis 8:447-458 (Pubitemid 32566135)
    • (2001) Neurobiology of Disease , vol.8 , Issue.3 , pp. 447-458
    • Callizot, N.1    Guenet, J.-L.2    Baillet, C.3    Warter, J.-M.4    Poindron, P.5
  • 14
    • 78650431705 scopus 로고    scopus 로고
    • Duchenne muscular dystrophy models show their age
    • Chamberlain JS (2010) Duchenne muscular dystrophy models show their age. Cell 143:1040-1042
    • (2010) Cell , vol.143 , pp. 1040-1042
    • Chamberlain, J.S.1
  • 15
    • 77955406842 scopus 로고    scopus 로고
    • Angelman syndrome, a genomic imprinting disorder of the brain
    • Chamberlain SJ, Lalande M (2010) Angelman syndrome, a genomic imprinting disorder of the brain. J Neurosci 30:9958-9963
    • (2010) J Neurosci , vol.30 , pp. 9958-9963
    • Chamberlain, S.J.1    Lalande, M.2
  • 16
  • 21
    • 0017962931 scopus 로고
    • Diabetes and obesity: Thrifty mutants?
    • Coleman DL (1978) Diabetes and obesity: thrifty mutants? Nutr Rev 36:129-132
    • (1978) Nutr Rev , vol.36 , pp. 129-132
    • Coleman, D.L.1
  • 22
    • 0015864927 scopus 로고
    • The influence of genetic background on the expression of the obese (Ob) gene in the mouse
    • Coleman DL, Hummel KP (1973) The influence of genetic background on the expression of the obese (Ob) gene in the mouse. Diabetologia 9:287-293
    • (1973) Diabetologia , vol.9 , pp. 287-293
    • Coleman, D.L.1    Hummel, K.P.2
  • 23
    • 0034660686 scopus 로고    scopus 로고
    • AKR resistance allele
    • Cormier RT, Dove WF (2000) Dnmt1N/+ reduces the net growth rate and multiplicity of intestinal adenomas in C57BL/6-multiple intestinal neoplasia (Min/+) mice independently of p53 but demonstrates strong synergy with the modifier of Min 1(AKR) resistance allele. Cancer Res 60:3965-3970 (Pubitemid 32204593)
    • (2000) Cancer Research , vol.60 , Issue.14 , pp. 3965-3970
    • Cormier, R.T.1    Dove, W.F.2
  • 26
    • 0000638596 scopus 로고
    • Les races pures et leurs combinaisons chez les souris
    • Cuénot L (1905) Les races pures et leurs combinaisons chez les souris. Arch Zool Exp Gén Ser 4 3:123-127
    • (1905) Arch Zool Exp Gén ser , vol.4 , Issue.3 , pp. 123-127
    • Cuénot, L.1
  • 27
    • 0031736120 scopus 로고    scopus 로고
    • Mouse alleles: If you've seen one, you haven't seen them all
    • DOI 10.1016/S0168-9525(98)01579-0
    • Davis AP, Justice M (1998) Mouse alleles: if you've seen one, you haven't seen them all. Trends Genet 14:438-441 (Pubitemid 28508917)
    • (1998) Trends in Genetics , vol.14 , Issue.11 , pp. 438-441
    • Davis, A.P.1    Justice, M.J.2
  • 28
    • 15944400297 scopus 로고    scopus 로고
    • Ultrafine mapping of SNPs from mouse strains C57BL/6J, DBA/ 2J, and C57BLKS/J for loci contributing to diabetes and atherosclerosis susceptibility
    • Davis RC, Schadt EE, Cervino ACL, Péterfy M, Lusis AJ (2005) Ultrafine mapping of SNPs from mouse strains C57BL/6J, DBA/ 2J, and C57BLKS/J for loci contributing to diabetes and atherosclerosis susceptibility. Diabetes 54:119-1199
    • (2005) Diabetes , vol.54 , pp. 119-1199
    • Davis, R.C.1    Schadt, E.E.2    Cervino, A.C.L.3    Péterfy, M.4    Lusis, A.J.5
  • 29
    • 77953666105 scopus 로고    scopus 로고
    • Genetic animal models of Parkinson's disease
    • Dawson TM, Ko HS, Dawson VL (2010) Genetic animal models of Parkinson's disease. Neuron 66:646-661
    • (2010) Neuron , vol.66 , pp. 646-661
    • Dawson, T.M.1    Ko, H.S.2    Dawson, V.L.3
  • 31
    • 0027363063 scopus 로고
    • Genetic identification of Mom-1, a major modifier locus affecting Min- induced intestinal neoplasia in the mouse
    • DOI 10.1016/0092-8674(93)90484-8
    • Dietrich WF, Lander ES, Smith JS, Moser AR, Gould KA, Luongo C, Borenstein N, Dove W (1993) Genetic identification of Mom-1, a major modifier locus affecting Min-induced intestinal neoplasia in the mouse. Cell 75:631-639 (Pubitemid 23346362)
    • (1993) Cell , vol.75 , Issue.4 , pp. 631-639
    • Dietrich, W.F.1    Lander, E.S.2    Smith, J.S.3    Moser, A.R.4    Gould, K.A.5    Luongo, C.6    Borenstein, N.7    Dove, W.8
  • 32
    • 0033757718 scopus 로고    scopus 로고
    • Inactivation of hdh in the brain and testis results in progressive neurodegeneration and sterility in mice
    • Dragatsis I, Levine MS, Zeitlin S (2000) Inactivation of hdh in the brain and testis results in progressive neurodegeneration and sterility in mice. Nat Genet 26:300-306
    • (2000) Nat Genet , vol.26 , pp. 300-306
    • Dragatsis, I.1    Levine, M.S.2    Zeitlin, S.3
  • 33
    • 0036366669 scopus 로고    scopus 로고
    • The mousetrap: What we can learn when the mouse model does not mimic the human disease
    • Elsea SH, Lucas RE (2002) The mousetrap: what we can learn when the mouse model does not mimic the human disease. ILAR J 43:66-79 (Pubitemid 135743763)
    • (2002) ILAR Journal , vol.43 , Issue.2 , pp. 66-79
    • Elsea, S.H.1    Lucas, R.E.2
  • 34
    • 0024551796 scopus 로고
    • Why isn't a mouse more like a man?
    • Erickson RP (1989) Why isn't a mouse more like a man? Trends Genet 5:1-3 (Pubitemid 19045710)
    • (1989) Trends in Genetics , vol.5 , Issue.1 , pp. 1-3
    • Erickson, R.P.1
  • 35
    • 65749318026 scopus 로고
    • Two new mutants, "trembler" and "reeler", with neurological actions in the house mouse
    • Falconer DS (1951) Two new mutants, "trembler" and "reeler", with neurological actions in the house mouse. J Genet 50:192-201
    • (1951) J Genet , vol.50 , pp. 192-201
    • Falconer, D.S.1
  • 36
    • 77957218441 scopus 로고
    • Adipose, a new inherited obesity of the mouse
    • Falconer DS, Isaacson JH (1959) Adipose, a new inherited obesity of the mouse. J Hered 50:290-292
    • (1959) J Hered , vol.50 , pp. 290-292
    • Falconer, D.S.1    Isaacson, J.H.2
  • 37
    • 63449102727 scopus 로고    scopus 로고
    • FOXP2 as a molecular window into speech and language
    • Fisher SE, Scharff C (2009) FOXP2 as a molecular window into speech and language. Trends Genet 25:166-177
    • (2009) Trends Genet , vol.25 , pp. 166-177
    • Fisher, S.E.1    Scharff, C.2
  • 38
  • 39
    • 80052576716 scopus 로고    scopus 로고
    • Garrod A.
    • Garrod A. http://www.esp.org/books/garrod/inborn-errors/facsimile/
  • 40
    • 45749151056 scopus 로고    scopus 로고
    • Animal models of Alzheimer's disease and frontotemporal dementia
    • DOI 10.1038/nrn2420, PII NRN2420
    • Götz J, Ittner LM (2008) Animal models of Alzheimer's disease and frontotemporal dementia. Nat Rev Neurosci 9:532-544 (Pubitemid 351873467)
    • (2008) Nature Reviews Neuroscience , vol.9 , Issue.7 , pp. 532-544
    • Gotz, J.1    Ittner, L.M.2
  • 43
    • 0021947289 scopus 로고
    • Do non-linked genes really reassort at random?
    • Guénet JL (1985) Do non-linked genes really reassort at random? Ann Inst Pasteur Immunol C 136:85-90
    • (1985) Ann Inst Pasteur Immunol C , vol.136 , pp. 85-90
    • Guénet, J.L.1
  • 44
    • 0037213794 scopus 로고    scopus 로고
    • Wild mice: An ever-increasing contribution to a popular mammalian model
    • DOI 10.1016/S0168-9525(02)00007-0, PII S0168952502000070
    • Guénet JL, Bonhomme F (2003) Wild mice: an ever-increasing contribution to a popular mammalian model. Trends Genet 19:24-31 (Pubitemid 35472175)
    • (2003) Trends in Genetics , vol.19 , Issue.1 , pp. 24-31
    • Guenet, J.-L.1    Bonhomme, F.2
  • 47
    • 5844357959 scopus 로고
    • An extreme non-agouti mutant in the mouse
    • Hollander WF, Gowen JW (1956) An extreme non-agouti mutant in the mouse. J Heredity 47:221-224
    • (1956) J Heredity , vol.47 , pp. 221-224
    • Hollander, W.F.1    Gowen, J.W.2
  • 48
  • 52
    • 0032192481 scopus 로고    scopus 로고
    • Mutation of the Angelman ubiquitin ligase in mice causes increased cytoplasmic p53 and deficits of contextual learning and long-term potentiation
    • DOI 10.1016/S0896-6273(00)80596-6
    • Jiang Y, Armstrong D, Albrecht U, Atkins CM, Noebels JL, Eichele G, Sweatt JD, Beaudet AL (1998) Mutation of the Angelman ubiquitin ligase in mice causes increased cytoplasmic p53 and deficits of contextual learning and long-term potentiation. Neuron 21:799-811 (Pubitemid 28498788)
    • (1998) Neuron , vol.21 , Issue.4 , pp. 799-811
    • Jiang, Y.-H.1    Armstrong, D.2    Albrecht, U.3    Atkins, C.M.4    Noebels, J.L.5    Eichele, G.6    Sweatt, J.D.7    Beaudet, A.L.8
  • 53
    • 69149089028 scopus 로고    scopus 로고
    • Lesch-Nyhan disease: From mechanism to model and back again
    • Jinnah HA (2009) Lesch-Nyhan disease: from mechanism to model and back again. Dis Model Mech 2:116-121
    • (2009) Dis Model Mech , vol.2 , pp. 116-121
    • Jinnah, H.A.1
  • 54
    • 0029004532 scopus 로고
    • Ectopic expression of the agouti gene in transgenic mice causes obesity, features of type II diabetes, and yellow fur
    • Klebig ML, Wilkinson JE, Geisler JG, Woychik RP (1995) Ectopic expression of the agouti gene in transgenic mice causes obesity, features of type II diabetes, and yellow fur. Proc Natl Acad Sci USA 92:4728-4732
    • (1995) Proc Natl Acad Sci USA , vol.92 , pp. 4728-4732
    • Klebig, M.L.1    Wilkinson, J.E.2    Geisler, J.G.3    Woychik, R.P.4
  • 55
    • 0023090920 scopus 로고
    • A potential animal model for Lesch-Nyhan syndrome through introduction of HPRT mutations into mice
    • DOI 10.1038/326295a0
    • Kuehn MR, Bradley A, Robertson EJ, Evans MJ (1987) A potential animal model for Lesch-Nyhan syndrome through introduction of HPRT mutations into mice. Nature 326:295-298 (Pubitemid 17053753)
    • (1987) Nature , vol.326 , Issue.6110 , pp. 295-298
    • Kuehn, M.R.1    Bradley, A.2    Robertson, E.J.3    Evans, M.J.4
  • 58
    • 34250724015 scopus 로고    scopus 로고
    • Min/1 on mouse chromosome 18
    • DOI 10.1534/genetics.107.071217
    • Kwong LN, Shedlovsky A, Biehl BS, Clipson L, Pasch CA, Dove WF (2007) Identification of Mom7, a novel modifier of Apc(Min/+) on mouse chromosome 18. Genetics 176:1237-1244 (Pubitemid 46961118)
    • (2007) Genetics , vol.176 , Issue.2 , pp. 1237-1244
    • Kwong, L.N.1    Shedlovsky, A.2    Biehl, B.S.3    Clipson, L.4    Pasch, C.A.5    Dove, W.F.6
  • 59
    • 0035368519 scopus 로고    scopus 로고
    • A transgenic model for listeriosis: Role of internalin in crossing the intestinal barrier
    • DOI 10.1126/science.1059852
    • Lecuit M, Vandormael-Pournin S, Lefort J, Huerre M, Gounon P, Dupuy C, Babinet C, Cossart P (2001) A transgenic model for Listeriosis: role of internalin in crossing the intestinal barrier. Science 292:1722-1725 (Pubitemid 32506246)
    • (2001) Science , vol.292 , Issue.5522 , pp. 1722-1725
    • Lecuit, M.1    Vandormael-Pournin, S.2    Lefort, J.3    Huerre, M.4    Gounon, P.5    Dupuy, C.6    Babinet, C.7    Cossart, P.8
  • 60
    • 63149139634 scopus 로고    scopus 로고
    • Sub-physiological sarcoglycan expression contributes to compensatory muscle protection in mdx mice
    • Li D, Long C, Yue Y, Duan D (2009) Sub-physiological sarcoglycan expression contributes to compensatory muscle protection in mdx mice. Hum Mol Genet 18:1209-1220
    • (2009) Hum Mol Genet , vol.18 , pp. 1209-1220
    • Li, D.1    Long, C.2    Yue, Y.3    Duan, D.4
  • 62
    • 0028998255 scopus 로고
    • The secretory phospholipase A2 gene is a candidate for the Mom1 locus, a major modifier of ApcMininduced intestinal neoplasia
    • MacPhee M, Chepenik KP, Liddell RA, Nelson KK, Siracusa LD, Buchberg AM (1995) The secretory phospholipase A2 gene is a candidate for the Mom1 locus, a major modifier of ApcMininduced intestinal neoplasia. Cell 81:957-966
    • (1995) Cell , vol.81 , pp. 957-966
    • MacPhee, M.1    Chepenik, K.P.2    Liddell, R.A.3    Nelson, K.K.4    Siracusa, L.D.5    Buchberg, A.M.6
  • 63
    • 76049104178 scopus 로고    scopus 로고
    • Phenylketonuria: Defects in amino acid metabolism
    • Madden M (2004) Phenylketonuria: defects in amino acid metabolism. South Carol J Mol Med (SCJMM) 5:57-61
    • (2004) South Carol J Mol Med (SCJMM) , vol.5 , pp. 57-61
    • Madden, M.1
  • 64
    • 0038202901 scopus 로고    scopus 로고
    • FOXP2 in focus: What can genes tell us about speech and language?
    • DOI 10.1016/S1364-6613(03)00104-9
    • Marcus GF, Fisher SE (2003) FOXP2 in focus: what can genes tell us about speech and language? Trends Cogn Sci 7:257-262 (Pubitemid 36683371)
    • (2003) Trends in Cognitive Sciences , vol.7 , Issue.6 , pp. 257-262
    • Marcus, G.F.1    Fisher, S.E.2
  • 65
    • 84870187451 scopus 로고    scopus 로고
    • Animal models of celiac disease
    • Fasano A, Troncone R, Branski D (eds). Pediatr Adolesc Med. Karger, Basel
    • Marietta EM, Murray JA, David CS (2008) Animal models of celiac disease. In: Fasano A, Troncone R, Branski D (eds) Frontiers in celiac disease, vol 12. Pediatr Adolesc Med. Karger, Basel, pp 210-216
    • (2008) Frontiers in Celiac Disease , vol.12 , pp. 210-216
    • Marietta, E.M.1    Murray, J.A.2    David, C.S.3
  • 67
    • 77955364853 scopus 로고    scopus 로고
    • The impact of genetic research on our understanding of Parkinson's disease
    • Martin I, Dawson VL, Dawson TM (2010) The impact of genetic research on our understanding of Parkinson's disease. Prog Brain Res 183:21-41
    • (2010) Prog Brain Res , vol.183 , pp. 21-41
    • Martin, I.1    Dawson, V.L.2    Dawson, T.M.3
  • 71
  • 72
    • 0028158177 scopus 로고
    • Aku, a mutation of the mouse homologous to human alkaptonuria, maps to chromosome 16
    • DOI 10.1006/geno.1994.1004
    • Montagutelli X, Lalouette A, Coudé M, Kamoun P, Forest M, Guénet JL (1994) aku, a mutation of the mouse homologous to human alkaptonuria, maps to chromosome 16. Genomics 19:9-11 (Pubitemid 24034358)
    • (1994) Genomics , vol.19 , Issue.1 , pp. 9-11
    • Montagutelli, X.1    Lalouette, A.2    Coude, M.3    Kamoun, P.4    Forest, M.5    Guenet, J.-L.6
  • 73
    • 0025015168 scopus 로고
    • A dominant mutation that predisposes to multiple intestinal neoplasia in the mouse
    • Moser AR, Pitot HC, Dove WF (1990) A dominant mutation that predisposes to multiple intestinal neoplasia in the mouse. Science 247:322-324
    • (1990) Science , vol.247 , pp. 322-324
    • Moser, A.R.1    Pitot, H.C.2    Dove, W.F.3
  • 74
    • 0026568524 scopus 로고
    • The Min (multiple intestinal neoplasia) mutation: Its effect on gut epithelial cell differentiation and interaction with a modifier system
    • Moser AR, Dove WF, Roth KA, Gordon JI (1992) The Min (multiple intestinal neoplasia) mutation: its effect on gut epithelial cell differentiation and interaction with a modifier system. J Cell Biol 116:1517-1526
    • (1992) J Cell Biol , vol.116 , pp. 1517-1526
    • Moser, A.R.1    Dove, W.F.2    Roth, K.A.3    Gordon, J.I.4
  • 76
    • 0034870684 scopus 로고    scopus 로고
    • Telomere lengths and telomerase activity in dog tissues: A potential model system to study human telomere and telomerase biology
    • DOI 10.1038/sj.neo.7900173
    • Nasir L, Devlin P, Mckevitt T, Rutteman G, Argyle DJ (2001) Telomere lengths and telomerase activity in dog tissues: a potential model system to study human telomere and telomerase biology. Neoplasia 3:351-359 (Pubitemid 32801885)
    • (2001) Neoplasia , vol.3 , Issue.4 , pp. 351-359
    • Nasir, L.1    Devlin, P.2    Mckevitt, T.3    Rutteman, G.4    Argyle, D.J.5
  • 77
    • 0033520364 scopus 로고    scopus 로고
    • Measles virus infection in a transgenic model: Virus-induced immunosuppression and central nervous system disease
    • DOI 10.1016/S0092-8674(00)80050-1
    • Oldstone MB, Lewicki H, Thomas D, Tishon A, Dales S, Patterson J, Manchester M, Homann D, Naniche D, Holz A (1999) Measles virus infection in a transgenic model: virus-induced immunosuppression and central nervous system disease. Cell 98:629-640 (Pubitemid 29418954)
    • (1999) Cell , vol.98 , Issue.5 , pp. 629-640
    • Oldstone, M.B.A.1    Lewicki, H.2    Thomas, D.3    Tishon, A.4    Dales, S.5    Patterson, J.6    Manchester, M.7    Homann, D.8    Naniche, D.9    Holz, A.10
  • 80
    • 1842408336 scopus 로고    scopus 로고
    • Transgenic knockout mice with exclusively human sickle hemoglobin and sickle cell disease
    • DOI 10.1126/science.278.5339.876
    • Pászty C, Brion CM, Manci E, Witkowska HE, Stevens ME, Mohandas N, Rubin EM (1997) Transgenic knockout mice with exclusively human sickle hemoglobin and sickle cell disease. Science 278:876-878 (Pubitemid 27467927)
    • (1997) Science , vol.278 , Issue.5339 , pp. 876-878
    • Paszty, C.1    Brion, C.M.2    Manci, E.3    Witkowska, H.E.4    Stevens, M.E.5    Mohandas, N.6    Rubin, E.M.7
  • 87
    • 79251561841 scopus 로고    scopus 로고
    • Limitations of animal models of Parkinson's disease
    • Potashkin JA, Blume SR, Runkle NK (2010) Limitations of animal models of Parkinson's disease. Parkinsons Dis 2011:658083
    • (2010) Parkinsons Dis , vol.2011 , pp. 658083
    • Potashkin, J.A.1    Blume, S.R.2    Runkle, N.K.3
  • 88
    • 0025183917 scopus 로고
    • Transgenic mice expressing a human poliovirus receptor: A new model for poliomyelitis
    • Ren RB, Costantini F, Gorgacz EJ, Lee JJ, Racaniello VR (1990) Transgenic mice expressing a human poliovirus receptor: a new model for poliomyelitis. Cell 63:353-362
    • (1990) Cell , vol.63 , pp. 353-362
    • Ren, R.B.1    Costantini, F.2    Gorgacz, E.J.3    Lee, J.J.4    Racaniello, V.R.5
  • 89
    • 33645789057 scopus 로고    scopus 로고
    • Understanding the basis for Down syndrome phenotypes
    • Roper RJ, Reeves RH (2006) Understanding the basis for Down syndrome phenotypes. PLoS Genet 2:e50
    • (2006) PLoS Genet , vol.2
    • Roper, R.J.1    Reeves, R.H.2
  • 94
    • 0027295348 scopus 로고
    • Mouse models of human phenylketonuria
    • Shedlovsky A, McDonald JD, Symula D, Dove WF (1993) Mouse models of human phenylketonuria. Genetics 134:1205-1210 (Pubitemid 23220041)
    • (1993) Genetics , vol.134 , Issue.4 , pp. 1205-1210
    • Shedlovsky, A.1    McDonald, J.D.2    Symula, D.3    Dove, W.F.4
  • 96
    • 0036141923 scopus 로고    scopus 로고
    • Identification of the modifier of Min 2 (Mom2) locus, a new mutation that influences Apc-induced intestinal neoplasia
    • DOI 10.1101/gr.206002
    • Silverman KA, Koratkar R, Siracusa LD, Buchberg AM (2002) Identification of the modifier of Min 2 (Mom2) locus, a new mutation that influences Apc-induced intestinal neoplasia. Genome Res 12:88-97 (Pubitemid 34065592)
    • (2002) Genome Research , vol.12 , Issue.1 , pp. 88-97
    • Silverman, K.A.1    Koratkar, R.2    Siracusa, L.D.3    Buchberg, A.M.4
  • 98
    • 52649090787 scopus 로고    scopus 로고
    • Behavioral effects of a deletion in Kcnn2, the gene encoding the SK2 subunit of small-conductance Ca2+ activated K+ channels
    • Szatanik M, Vibert N, Vassias I, Guénet JL, Eugène D, de Waele C, Jaubert J (2008) Behavioral effects of a deletion in Kcnn2, the gene encoding the SK2 subunit of small-conductance Ca2+ activated K+ channels. Neurogenetics 9:237-248
    • (2008) Neurogenetics , vol.9 , pp. 237-248
    • Szatanik, M.1    Vibert, N.2    Vassias, I.3    Guénet, J.L.4    Eugène, D.5    De Waele, C.6    Jaubert, J.7
  • 99
    • 0029906168 scopus 로고    scopus 로고
    • Amelioration of the dystrophic phenotype of mdx mice using a truncated utrophin transgene
    • DOI 10.1038/384349a0
    • Tinsley JM, Potter AC, Phelps SR, Fisher R, Trickett JI, Davies KE (1996) Amelioration of the dystrophic phenotype of mdx mice using a truncated utrophin transgene. Nature 384:349-353 (Pubitemid 26408514)
    • (1996) Nature , vol.384 , Issue.6607 , pp. 349-353
    • Tinsley, J.M.1    Potter, A.C.2    Phelps, S.R.3    Fisher, R.4    Trickett, J.I.5    Davies, K.E.6
  • 101
    • 19744380273 scopus 로고    scopus 로고
    • Loss of wild-type huntingtin influences motor dysfunction and survival in the YAC128 mouse model of Huntington disease
    • DOI 10.1093/hmg/ddi147
    • Van Raamsdonk JM, Pearson J, Rogers DA, Bissada N, Vogl AW, Hayden MR, Leavitt BR (2005) Loss of wild-type huntingtin influences motor dysfunction and survival in the YAC128 mouse model of Huntington disease. Hum Mol Genet 14:1379-1392 (Pubitemid 40744495)
    • (2005) Human Molecular Genetics , vol.14 , Issue.10 , pp. 1379-1392
    • Van Raamsdonk, J.M.1    Pearson, J.2    Rogers, D.A.3    Bissada, N.4    Vogl, A.W.5    Hayden, M.R.6    Leavitt, B.R.7
  • 102
    • 71649101421 scopus 로고    scopus 로고
    • Characterization of an MPS I-H knock-in mouse that carries a nonsense mutation analogous to the human IDUA-W402X mutation
    • Wang D, Shukla C, Liu X, Schoeb TR, Clarke LA, Bedwell DM, Keeling KM (2010) Characterization of an MPS I-H knock-in mouse that carries a nonsense mutation analogous to the human IDUA-W402X mutation. Mol Genet Metab 99:62-71
    • (2010) Mol Genet Metab , vol.99 , pp. 62-71
    • Wang, D.1    Shukla, C.2    Liu, X.3    Schoeb, T.R.4    Clarke, L.A.5    Bedwell, D.M.6    Keeling, K.M.7
  • 103
    • 58149380025 scopus 로고    scopus 로고
    • Animal models of Alzheimer's disease: Therapeutic implications
    • Woodruff-Pak DS (2008) Animal models of Alzheimer's disease: therapeutic implications. J Alzheimer's Dis 15:507-521
    • (2008) J Alzheimer's Dis , vol.15 , pp. 507-521
    • Woodruff-Pak, D.S.1
  • 104
    • 77955278262 scopus 로고    scopus 로고
    • 'Too much good news' - Are Alzheimer mouse models trying to tell us how to prevent, not cure, Alzheimer's disease?
    • Zahs KR, Ashe KH (2010) 'Too much good news' - are Alzheimer mouse models trying to tell us how to prevent, not cure, Alzheimer's disease? Trends Neurosci 33:381-389
    • (2010) Trends Neurosci , vol.33 , pp. 381-389
    • Zahs, K.R.1    Ashe, K.H.2
  • 105
    • 0028139089 scopus 로고
    • Positional cloning of the mouse obese gene and its human homologue
    • DOI 10.1038/372425a0
    • Zhang Y, Proença R, Maffei M, Barone M, Leopold L, Friedman JM (1994) Positional cloning of the mouse obese gene and its human homologue. Nature 372:425-432 (Pubitemid 24365442)
    • (1994) Nature , vol.372 , Issue.6505 , pp. 425-432
    • Zhang, Y.1    Proenca, R.2    Maffei, M.3    Barone, M.4    Leopold, L.5    Friedman, J.M.6


* 이 정보는 Elsevier사의 SCOPUS DB에서 KISTI가 분석하여 추출한 것입니다.