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Huntingtin Comparative Pathway

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Huntington disease is an Autosomally Dominant degenerative disorder resulting from expansion (>37 units) of a polyglutamine repeats in Huntingtin, a 350-kDa protein of unknown function. The polyglutamine repeat is localized in the N-terminal region of Huntingtin and is encoded by exon1 of the HD or Htt (Huntingtin) gene. Huntington disease is characterized by uncontrolled movements, personality changes, and dementia and causes the death of patients within 10–20 years after the appearance of the first symptoms. Huntingtin is highly expressed in the brain, and particularly enriched in Cerebral Cortex and Striatum. It is a cytoplasmic protein that is essential during development for Gastrulation and Neurogenesis, and it is important for Neuronal survival in the adult. Wild-type Huntingtin is anti-apoptotic in neurons [...]


1.Huntingtin processing in pathogenesis of Huntington disease.
Qin ZH, Gu ZL.
Acta Pharmacol Sin. 2004 Oct;25(10):1243-9.
2.Huntingtin is present in the nucleus, interacts with the transcriptional corepressor C-terminal binding protein, and represses transcription.
Kegel KB, Meloni AR, Yi Y, Kim YJ, Doyle E, Cuiffo BG, Sapp E, Wang Y, Qin ZH, Chen JD, Nevins JR, Aronin N, DiFiglia M.
J Biol Chem. 2002 Mar 1;277(9):7466-76. Epub 2001 Dec 05.
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