Depletion of wild-type huntingtin in mouse models of neurologic diseases

Journal of Neurochemistry

Volumn 87, Issue 1, 2003, Pages 101-106

  Zhang, Yu ^a   Li, Mingwei ^a   Drozda, Martin ^a   Chen, Minghua ^a   Ren, Shengjun ^b   Sanchez, Rene O Mejia ^a   Leavitt, Blair R ^c   Cattaneo, Elena ^d   Ferrante, Robert J ^e,f   Hayden, Michael R ^c   Friedlander, Robert M ^a  

^a BRIGHAM AND WOMEN S HOSPITAL   (United States)

^b HARVARD MEDICAL SCHOOL   (United States)

^c UNIVERSITY OF BRITISH COLUMBIA   (Canada)

^d UNIVERSITY OF MILAN   (Italy)

^e VETERANS AFFAIRS MEDICAL CENTER   (United States)

^f BOSTON UNIVERSITY SCHOOL OF MEDICINE   (United States)

Author keywords

BDNF; Brain trauma; Huntingtin; Huntington's disease; Ischemia; Spinal cord injury

Indexed keywords

BRAIN DERIVED NEUROTROPHIC FACTOR; HUNTINGTIN;

ALLELE; ANIMAL EXPERIMENT; ANIMAL MODEL; ANIMAL TISSUE; APOPTOSIS; ARTICLE; AUTOSOMAL DOMINANT INHERITANCE; BRAIN FUNCTION; BRAIN TISSUE; CONTROLLED STUDY; DISEASE COURSE; GENE MUTATION; GENE OVEREXPRESSION; GENETIC CODE; HUNTINGTON CHOREA; MOUSE; NERVE DEGENERATION; NEUROLOGIC DISEASE; NONHUMAN; PRIORITY JOURNAL; PROTEIN FUNCTION; PROTEIN LOCALIZATION; SPINAL CORD INJURY; TRANSGENIC MOUSE; WILD TYPE;

ANIMALS; BRAIN CHEMISTRY; BRAIN INJURIES; BRAIN ISCHEMIA; BRAIN-DERIVED NEUROTROPHIC FACTOR; CASPASES; DISEASE MODELS, ANIMAL; DISEASE PROGRESSION; HUMANS; HUNTINGTON DISEASE; MICE; MICE, INBRED C57BL; MICE, TRANSGENIC; NERVE TISSUE PROTEINS; NUCLEAR PROTEINS; SPINAL CORD INJURIES;

ANIMALIA; MUS MUSCULUS;

EID: 0141531987     PISSN: 00223042     EISSN: None     Source Type: Journal    
DOI: 10.1046/j.1471-4159.2003.01980.x     Document Type: Article

References (23)

1. DiFiglia M., Sapp E., Chase K., Schwarz C., Meloni A., Young C., Martin E., Vonsattel J. P., Carraway R., Reeves S. A., Boyce F. M. and Aronin N. (1995) Huntingtin is a cytoplasmic protein associated with vesicles in human and rat brain neurons. Neuron 14, 1075-1081.
2. Dragatsis I., Levine M. S. and Zeitlin S. (2000) Inactivation of Hdh in the brain and testis results in progressive neurodegeneration and sterility in mice. Nat. Genet. 26, 300-306.
3. Duyao M. P., Auerbach A. B., Ryan A., Persichetti F., Barnes G. T., McNeil S. M., Ge P., Vonsattel J. P., Gusella J. F., Joyner A. L. and MacDonald M. (1995) Inactivation of the mouse Huntington's disease gene homolog Hdh. Science 269, 407-410.
4. Ferrante R. J., Gutekunst C. A., Persichetti F., McNeil S. M., Kowall N. W., Gusella J. F., MacDonald M. E., Beal M. F. and Hersch S. M. (1997) Heterogeneous topographic and cellular distribution of huntingtin expression in the normal human neostriatum J. Neurosci. 17, 3052-3063.
5. Fink K. B., Andrews L. J., Butler W. E., Ona V. O., Li M., Bogdanov M., Endres M., Khan S. Q., Namura S., Stieg P. E., Beal M. F., Moskowitz M. A., Yuan J. and Friedlander R. M. (1999) Reduction of post-traumatic brain injury and free radical production by inhibition of the caspase-1 cascade. Neuroscience 94, 1213-1218.
6. Gafni J. and Ellerby L. M. (2002) Calpairi activation in Huntington's disease. J. Neurosci. 22, 4842-4849.
7. Hodgson J. G., Agopyan N., Gutekunst C. A., Leavitt B. R., LePiane F., Singaraja R., Smith D. J., Bissada N., McCutcheon K., Nasir J., Jamot L., Li X. J., Stevens M. E., Rosemond E., Roder J. C., Phillips A. G., Rubin E. M., Hersch S. M. and Hayden M. R. (1999) A YAC mouse model for Huntington's disease with full-length mutant huntingtin, cytoplasmic toxicity, and selective striatal neurodegeneration. Neuron 23, 181-192.
8. Kim Y. J., Yi Y., Sapp E., Wang Y., Cuiffo B., Kegel K. B., Qin Z. H., Aronin N. and DiFiglia M. (2001) Caspase 3-cleaved N-terminal fragments of wild-type and mutant huntingtin are present in normal and Huntington's disease brains, associate with membranes, and undergo calpain-dependent proteolysis. Proc. Natl Acad. Sci. USA 98, 12784-12789.
9. Li S. H., Schilling G., Young W. S., Li X. J., Margolis R. L., Stine O. C., Wagster M. V., Abbott M. H., Franz M. L., Ranen N. G., Hedreen J., Folstein S. E. and Ross C. A. (1993) Huntington's disease gene (IT 15) is widely expressed in human and, rat tissues. Neuron 11, 985-993.
10. Li M., Ona V. O., Chen M., Kaul M., Tenneti L., Zhang X., Stieg P. E., Lipton S. A. and Friedlander R. M. (2000) Functional role and therapeutic implications of neuronal caspase-1 and -3 in a mouse model of traumatic spinal cord injury. Neuroscience 99, 333-342.
11. Mangiarini L., Sathasivam K., Seller M., Cozens B., Harper A., Hetherington C., Lawton M., Trottier Y., Lehrach H., Davies S. W. and Bates G. P, (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.
12. Nasir J., Floresco S. B., O'Kusky J. R., Diewert V. M., Richman J. M., Zeisler J., Borowski A., Marth J. D., Phillips A. G. and Hayden M. R. (1995) Targeted disruption of the Huntington's disease gene results in embryonic lethality and behavioral and morphological changes in heterozygotes. Cell 81, 811-823.
13. Reddy P. H., Charles V., Williams M., Miller G., Whetsell W. O. Jr and Tagle D. A. (1999) Transgenic mice expressing mutated full-length HD cDNA: a paradigm for locomotor changes and selective neuronal loss in Huntington's disease. Philos. Trans. R. Soc. Lond. B Biol. Sci. 354, 1035-1045.
14. Rigamonti D., Bauer J. H., De-Fraja C., Conti L., Sipione S., Sciorati C., Clementi E., Hackam A., Hayden M. R., Li Y., Cooper J. K., Ross C. A., Govoni S., Vincenz C. and Cattaneo E. (2000) Wild-type huntingtin protects from apoptosis upstream of caspase-3. J. Neurosci. 20, 3705-3713.
15. Schilling G., Becher M. W., Sharp A. H., Jinnah H. A., Duan K., Kotzuk J. A., Slunt H. H., Ratovitski T., Cooper J. K., Jenkins N. A., Copeland N. G., Price D. L., Ross C. A. and Borchelt D. R. (1999) Intranuclear inclusions and neuritic aggregates in transgenic mice expressing a mutant N-terminal fragment of huntingtin. Hum. Mol. Genet. 8, 397-407.
16. Sharp A. H., Loev S. J., Schilling G., Li S. H., Li X. J., Bao J., Wagster M. V., Kotzuk J. A., Steiner J. P., Lo A., Hedreen J., Sisodia S., Snyder S. H., Dawson T. M., Ryugo D. K. and Ross C. A. (1995) Widespread expression of Huntington's disease gene (IT 15) protein product. Neuron 14, 1065-1074.
17. Shen L., Figurov A. and Lu B. (1997) Recent progress in studies of neurotrophic factors and their clinical implications. J. Mol. Med. 75, 637-644.
18. Sun Y., Savanenin A., Reddy P. H. and Liu Y. F. (2001) Polyglutamine-expanded huntingtin promotes sensitization of N-methyl-D-asparrate receptors via post-synaptic density 95. J. Biol. Chem. 276, 24713-24718.
19. 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.
20. Velier J., Kim M., Schwarz C., Kim T. W., Sapp E., Chase K., Aronin N. and DiFiglia M. (1998) Wild-type and mutant huntingtins function in vesicle trafficking in the secretory and endocytic pathways. Exp. Neurol. 152, 34-40.
21. Waelter S., Scherzinger E., Hasenbank R., Nordhoff E., Lurz R., Goehler H., Gauss C., Sathasivam K., Bates G. P., Lehrach, H. and Wanker E. E. (2001) The huntingtin interacting protein HIP1 is a clathrin and α -adaptin-binding protein involved in receptor-mediated endocytosis. Hum. Mol. Genet. 10, 1807-1817.
22. Zeitlin S., Liu J. P., Chapman D. L., Papaioannou V. E. and Efstratiadis A. (1995) Increased apoptosis and early embryonic lethality in mice nullizygous for the Huntington's disease gene homologue. Nat. Genet. 11, 155-163.
23. Zuccato C., Ciammola A., Rigamonti D., Leavitt B. R., Goffredo D., Conti L., MacDonald M. E., Friedlander R. M., Sitani V., Hayden M. R., Timmusk T., Sipione S. and Cattaneo E. (2001) Loss of huntingtin-mediated BDNF gene transcription in Huntington's disease. Science 293, 493-498.