Document Detail

Genotype-dependent priming to self- and xeno-cannibalism in heterozygous and homozygous lymphoblasts from patients with Huntington's disease.
MedLine Citation:
PMID:  16895579     Owner:  NLM     Status:  MEDLINE    
In the present work, we studied the mitochondrial function and cell death pathway(s) in heterozygous and homozygous immortalized cell lines from patients with Huntington's disease (HD). Heterozygosis was characterized by specific alterations in mitochondrial membrane potential, a constitutive hyperpolarization state of mitochondria, and was correlated with an increased susceptibility to apoptosis. Lymphoblasts from homozygous patients, on the other hand, were characterized by a significant percentage of cells displaying autophagic vacuoles. These cells also demonstrated a striking attitude towards significant cannibalistic activity. Considering the pathogenic role of cell death in HD, our study provides new and useful insights into the role of mitochondrial dysfunction, i.e. hyperpolarization, in hijacking HD heterozygous cells towards apoptosis and HD homozygous cells towards a peculiar phenotype characterized by both self- and xeno-cannibalism. These events can, however, be viewed as an ultimate attempt to survive rather than a way to die. The present work underlines the possibility that HD-associated mitochondrial defects could tentatively be by-passed by the cells by activating cellular 'phagic' activities, including so-called 'mitophagy' and 'cannibalism', that only finally lead to cell death.
Elisabetta Mormone; Paola Matarrese; Antonella Tinari; Milena Cannella; Vittorio Maglione; Maria Grazia Farrace; Mauro Piacentini; Luigi Frati; Walter Malorni; Ferdinando Squitieri
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Publication Detail:
Type:  Journal Article    
Journal Detail:
Title:  Journal of neurochemistry     Volume:  98     ISSN:  0022-3042     ISO Abbreviation:  J. Neurochem.     Publication Date:  2006 Aug 
Date Detail:
Created Date:  2006-08-09     Completed Date:  2006-09-21     Revised Date:  -    
Medline Journal Info:
Nlm Unique ID:  2985190R     Medline TA:  J Neurochem     Country:  England    
Other Details:
Languages:  eng     Pagination:  1090-9     Citation Subset:  IM    
Department of Drug Research and Evaluation, Istituto Superiore di Sanità, Rome, Italy.
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MeSH Terms
Annexin A5 / metabolism
Cell Death / physiology
Cell Line
Cell Survival / physiology
Flow Cytometry
Huntington Disease / genetics*,  pathology*
Lymphocytes / immunology*
Membrane Potentials / physiology
Microscopy, Electron, Transmission
Microscopy, Fluorescence
Mitochondria / metabolism
Phagocytosis / genetics*
Reg. No./Substance:
0/Annexin A5

From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine

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