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Article

HD CAG repeat implicates a dominant property of huntingtin in mitochondrial energy metabolism.

Human molecular genetics - 1 Oct 2005

Seong Ihn Sik, Ivanova Elena, Lee Jong-Min, Choo Yeun Su, Fossale Elisa, Anderson MaryAnne, Gusella James F, Laramie Jason M, Myers Richard H, Lesort Mathieu, MacDonald Marcy E

Abstract excerpt

The 'expanded' HD CAG repeat that causes Huntington's disease (HD) encodes a polyglutamine tract in huntingtin, which first targets the death of medium-sized spiny striatal neurons. Mitochondrial energetics, related to N-methyl-d-aspartate (NMDA) Ca2+-signaling, has long been implicated in this neuronal specificity, implying an integral role for huntingtin in mitochondrial energy metabolism. As a genetic test of...

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Identifiers

PMID: 16115812
DOI: 10.1093/hmg/ddi319

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PubMed metadata and Literature Corpus relationships. Corpus snapshot updated 7 Sept 2026. Manifest publication-graph-cohort-2026-09-07.1.

Source receipt: pubmed, retrieved 7 Sept 2026.

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