Protein Information

Name huntingtin
Synonyms HD; HTT; HD protein; Huntingtin; Huntington disease protein; Huntington's disease protein; IT15; huntingtin (Huntington disease)…

Compound Information

Name rotenone
CAS

Reference List

PubMed Abstract RScore(About this table)
15983033 Milakovic T, Johnson GV: Mitochondrial respiration and ATP production are significantly impaired in striatal cells expressing mutant huntingtin. J Biol Chem. 2005 Sep 2;280(35):30773-82. Epub 2005 Jun 27.

3(0,0,0,3) Details
14962977 Ruan Q, Lesort M, MacDonald ME, Johnson GV: Striatal cells from mutant huntingtin knock-in mice are selectively vulnerable to mitochondrial complex II inhibitor-induced cell death through a non-apoptotic pathway. Hum Mol Genet. 2004 Apr 1;13(7):669-81. Epub 2004 Feb 12.

In contrast, the extent of cell death induced by rotenone, a complex I inhibitor, was similar in both cell lines.
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19076450 Knott AB, Bossy-Wetzel E: Impairing the mitochondrial fission and fusion balance: a new mechanism of neurodegeneration. Ann N Y Acad Sci. 2008 Dec;1147:283-92.

Rotenone, a pesticide and inducer of PD symptoms, and amyloid-beta peptide, which is causally linked to Alzheimer's disease, initiate mitochondrial fission.
Furthermore, mutant huntingtin, the disease-causing protein in Huntington's disease, alters mitochondrial morphology and dynamics.
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17726098 Varma H, Cheng R, Voisine C, Hart AC, Stockwell BR: Inhibitors of metabolism rescue cell death in Huntington's disease models. Proc Natl Acad Sci U S A. 2007 Sep 4;104(36):14525-30. Epub 2007 Aug 28.


HD is caused by polyglutamine expansions in the huntingtin (htt) protein that result in neuronal loss and contribute to HD pathology.
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