Significantly differential diffusion of neuropathological aggregates in the brain of transgenic mice carrying N-terminal mutant huntingtin fused with green fluorescent protein

Brain Structure and Function

Volumn 218, Issue 1, 2013, Pages 283-294

  Cheng, Pei Hsun ^a   Li, Chia Ling ^b   Her, Lu Shiun ^b   Chang, Yu Fan ^a   Chan, Anthony W S ^c   Chen, Chuan Mu ^d   Yang, Shang Hsun ^a,b  

^a NATIONAL CHENG KUNG UNIVERSITY   (Taiwan)

^b NATIONAL CHENG KUNG UNIVERSITY   (Taiwan)

^c EMORY UNIVERSITY SCHOOL OF MEDICINE   (United States)

^d NATIONAL CHUNG HSING UNIVERSITY   (Taiwan)

Author keywords

Animal model; Differential diffusion of neuropathological aggregates; Green fluorescent protein; Huntington's disease (HD); Transgenic mice

Indexed keywords

GREEN FLUORESCENT PROTEIN; HUNTINGTIN;

AMINO TERMINAL SEQUENCE; ANIMAL BEHAVIOR; ANIMAL CELL; ANIMAL TISSUE; ARTICLE; CAG REPEAT; CONTROLLED STUDY; EXON; FEMALE; GENE; GREEN FLUORESCENT PROTEIN GENE; HUMAN; HUMAN CELL; HUNTINGTIN GENE; HUNTINGTON CHOREA; IMMUNOHISTOCHEMISTRY; MOLECULAR MODEL; MOUSE; NEUROPATHOLOGY; NONHUMAN; PHENOTYPIC VARIATION; PRIORITY JOURNAL; PROTEIN EXPRESSION; SIGNAL TRANSDUCTION; TRANSGENIC MOUSE; TRINUCLEOTIDE REPEAT;

ANIMALS; BEHAVIOR, ANIMAL; BRAIN; DISEASE MODELS, ANIMAL; EXONS; GENOTYPE; GREEN FLUORESCENT PROTEINS; HUMANS; HUNTINGTON DISEASE; IMMUNOHISTOCHEMISTRY; MICE; MICE, TRANSGENIC; MOTOR ACTIVITY; MUSCLE STRENGTH; MUTATION; NERVE TISSUE PROTEINS; PHENOTYPE; PROTEIN STRUCTURE, TERTIARY; RECOMBINANT FUSION PROTEINS; TRINUCLEOTIDE REPEATS;

ANIMALIA; MUS MUSCULUS;

EID: 84872361173     PISSN: 18632653     EISSN: 18632661     Source Type: Journal    
DOI: 10.1007/s00429-012-0401-x     Document Type: Article

References (24)

1. Carter RJ, Lione LA, Humby T et al (1999) Characterization of progressive motor deficits in mice transgenic for the human Huntington's disease mutation. J Neurosci 19:3248-3257
2. Chalfie M, Tu Y, Euskirchen G, Ward WW, Prasher DC (1994) Green fluorescent protein as a marker for gene expression. Science 263:802-805
3. Chan AW, Yang SH (2009) Generation of transgenic monkeys with human inherited genetic disease. Methods 49:78-84
4. Chiang MC, Chern Y, Juo CG (2011) The dysfunction of hepatic transcriptional factors in mice with Huntington's Disease. Biochim Biophys Acta 1812:1111-1120
5. Ciammola A, Sassone J, Sciacco M et al (2011) Low anaerobic threshold and increased skeletal muscle lactate production in subjects with Huntington's disease. Mov Disord 26:130-137
6. Cornett J, Cao F, Wang CE et al (2005) Polyglutamine expansion of huntingtin impairs its nuclear export. Nat Genet 37:198-204
7. Davies SW, Turmaine M, Cozens BA et al (1997) Formation of neuronal intranuclear inclusions underlies the neurological dysfunction in mice transgenic for the HD mutation. Cell 90:537-548
8. DiFiglia M, Sapp E, Chase K et al (1995) Huntingtin is a cytoplasmic protein associated with vesicles in human and rat brain neurons. Neuron 14:1075-1081
9. Hodgson JG, Agopyan N, Gutekunst CA et al (1999) A YAC mouse model for Huntington's disease with full-length mutant huntingtin, cytoplasmic toxicity, and selective striatal neurodegeneration. Neuron 23:181-192
10. Hogan B (1994) Manipulating the mouse embryo: a laboratory manual. Cold Spring Harbor Laboratory Press, Cold Spring Harbor
11. Li S, Li XJ (2006) Multiple pathways contribute to the pathogenesis of Huntington disease. Mol Neurodegener 1:19
12. Lin CH, Tallaksen-Greene S, Chien WM et al (2001) Neurological abnormalities in a knock-in mouse model of Huntington's disease. Hum Mol Genet 10:137-144
13. Maat-Schieman M, Roos R, Losekoot M et al (2007) Neuronal intranuclear and neuropil inclusions for pathological assessment of Huntington's disease. Brain Pathol 17:31-37
14. Mangiarini L, Sathasivam K, Seller M et al (1996) Exon 1 of the HD gene with an expanded CAG repeat is sufficient to cause a progressive neurological phenotype in transgenic mice. Cell 87:493-506
15. McGuire JR, Rong J, Li SH, Li XJ (2006) Interaction of Huntingtin-associated protein-1 with kinesin light chain: implications in intracellular trafficking in neurons. J Biol Chem 281:3552-3559
16. Moffitt H, McPhail GD, Woodman B, Hobbs C, Bates GP (2009) Formation of polyglutamine inclusions in a wide range of non-CNS tissues in the HdhQ150 knock-in mouse model of Huntington's disease. PLoS ONE 4:e8025
17. Schilling G, Becher MW, Sharp AH et al (1999) Intranuclear inclusions and neuritic aggregates in transgenic mice expressing a mutant N-terminal fragment of huntingtin. Hum Mol Genet 8:397-407
18. The Huntington's Disease Collaborative Research Group (1993) A novel gene containing a trinucleotide repeat that is expanded and unstable on Huntington's disease chromosomes. Cell 72:971-983
19. von Horsten S, Schmitt I, Nguyen HP et al (2003) Transgenic rat model of Huntington's disease. Hum Mol Genet 12:617-624
20. Walker FO (2007) Huntington's disease. Lancet 369:218-228
21. Wang CE, Tydlacka S, Orr AL et al (2008) Accumulation of N-terminal mutant huntingtin in mouse and monkey models implicated as a pathogenic mechanism in Huntington's disease. Hum Mol Genet 17:2738-2751
22. Williams A, Sarkar S, Cuddon P et al (2008) Novel targets for Huntington's disease in an mTOR-independent autophagy pathway. Nat Chem Biol 4:295-305
23. Yang SH, Chan AW (2011) Transgenic animal models of Huntington's Disease. Curr Top Behav Neurosci 7:61-85
24. Yang SH, Cheng PH, Banta H et al (2008) Towards a transgenic model of Huntington's disease in a non-human primate. Nature 453:921-924