Mitochondrial Functional Alterations in Relation to Pathophysiology of Huntington’s Disease
IR@IICB: CSIR-Indian Institute of Chemical Biology, Kolkata
View Archive Info| Field | Value | |
| Title |
Mitochondrial Functional Alterations in Relation
to Pathophysiology of Huntington’s Disease
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| Creator |
Pandey, Mritunjay
Mohanakumar , K P Usha, Rajamma |
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| Subject |
Cell Biology & Physiology
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| Description |
Huntington’s disease (HD) is an autosomal
dominant neurodegenerative disease which is characterized
by psychiatric symptoms, involuntary choreiform movements
and dementia with maximum degeneration occurring
in striatum and cerebral cortex. Several studies implicate
mitochondrial dysfunction to the selective neurodegeneration
happening in this disorder. Calcium buffering imbalance
and oxidative stress in the mitochondria, critically
impaired movement across axons and abnormal fission or
fusion of this organelle in the cells are some of the salient
features that results in the loss of mitochondrial electron
transport chain (ETC) complex function in HD. Although
several models involving mutant huntingtin, excitotoxins
and mitochondrial complex-II inhibitors have been used to
explore the disease, it is not clear how disturbances in
mitochondrial functioning is associated with such selective
neurodegeneration, or in the expression of huntingtonian
phenotypes in animals or man. We have carefully assessed
various mitochondrial abnormalities observed in human
patient samples, postmortem HD brains, cellular, vertebrate
and invertebrate models of the disease, to conclude that
ETC dysfunction is an integral part of the disease and justify a causal role of mitochondrial ETC dysfunction for
the genesis of this disorder
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| Publisher |
Kluwer
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| Date |
2010
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| Type |
Article
PeerReviewed |
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| Format |
application/pdf
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| Identifier |
http://www.eprints.iicb.res.in/69/1/JOURNAL_OF_BIOENERGETICS_AND_BIOMEMBRANES_42(3)_217%2D226_;2010[72].pdf
Pandey, Mritunjay and Mohanakumar , K P and Usha, Rajamma (2010) Mitochondrial Functional Alterations in Relation to Pathophysiology of Huntington’s Disease. Journal of Bioenergetics and Biomembranes, 42. pp. 217-226. |
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| Relation |
http://dx.doi.org/10.1007/s10863-010-9288-5
http://www.eprints.iicb.res.in/69/ |
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