Early cerebellar deficits in mitochondrial biogenesis and respiratory chain complexes in the KIKO mo...
Early cerebellar deficits in mitochondrial biogenesis and respiratory chain complexes in the KIKO mouse model of Friedreich ataxia
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Publisher
England: The Company of Biologists Ltd
Journal title
Language
English
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Publisher
England: The Company of Biologists Ltd
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Scope and Contents
Contents
Friedreich ataxia (FRDA), the most common recessive inherited ataxia, results from deficiency of frataxin, a small mitochondrial protein crucial for iron-sulphur cluster formation and ATP production. Frataxin deficiency is associated with mitochondrial dysfunction in FRDA patients and animal models; however, early mitochondrial pathology in FRDA ce...
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Full title
Early cerebellar deficits in mitochondrial biogenesis and respiratory chain complexes in the KIKO mouse model of Friedreich ataxia
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TN_cdi_doaj_primary_oai_doaj_org_article_d79fcb553b9b47c5bc781c43cb423749
Permalink
https://devfeature-collection.sl.nsw.gov.au/record/TN_cdi_doaj_primary_oai_doaj_org_article_d79fcb553b9b47c5bc781c43cb423749
Other Identifiers
ISSN
1754-8403
E-ISSN
1754-8411
DOI
10.1242/dmm.030502
