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Severe ultrastructural mitochondrial changes in lymphoblasts homozygous for Huntington disease mutation.

Mechanisms of ageing and development - 1 Feb 2006

Squitieri Ferdinando, Cannella Milena, Sgarbi Gianluca, Maglione Vittorio, Falleni Alessandra, Lenzi Paola, Baracca Alessandra, Cislaghi Giuliana, Saft Carsten, Ragona Giuseppe, Russo Matteo A, Thompson Leslie M, Solaini Giancarlo, Fornai Francesco

Abstract excerpt

Mutated huntingtin is expressed in nervous and non nervous system included lymphoblasts. Eneregetic metabolism is impaired in Huntington's disease (HD) and other neurodegenerative diseases. Human HD lymphoblasts have provided clear-cut data on mitochondnal disruption. Here we report morphological, morphometric and membrane potential differences in mitochondria from lymphoblasts obtained from patients homozygous...

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Identifiers

PMID: 16289240
DOI: 10.1016/j.mad.2005.09.010

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PubMed metadata and Literature Corpus relationships. Corpus snapshot updated 6 Sept 2026. Manifest publication-graph-cohort-2026-09-06.1.

Source receipt: pubmed, retrieved 6 Sept 2026.

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