Inter-mitochondrial complementation: Mitochondria-specific system preventing mice from expression of disease phenotypes by mutant mtDNA
Article Abstract:
The pathogenesis of deleting mutant mitochondrial (mt) DNA by generating mice with mutant mtDNA carrying a 4696-basepair deletion (mtDNA4696), and by using cytochrome c oxidase electron micrographs to identify COX activity at the individual mitochondrial level is investigated. The results in this context indicate the occurrence of inter-mitochondrial complementation through exchange of genetic contents between exogenously introduced mitochondria with mtDNA4696 and host mitochondria with normal mtDNA.
Publication Name: Nature Medicine
Subject: Health
ISSN: 1078-8956
Year: 2001
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A crucial role of mitochondrial Hsp40 in preventing dilated cardiomyopathy
Article Abstract:
The heat shock protein (Hsp), mitochondrial chaperone Hsp40 plays a very important role in inhibiting the aggregation of unfolded polypepetides and refold denatured proteins, which later results in protein aggregation and protein instability. The results how that the Hsp40, also known as Dnaja3 is a very important part of mitochondrial biogenesis in preventing dilated cardiomyopathy, as it also provides genetic evidence of the necessity for mitochondrial Hsp40 required in it.
Publication Name: Nature Medicine
Subject: Health
ISSN: 1078-8956
Year: 2006
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Inbred Israeli families aid research on rare diseases
Article Abstract:
Genetic studies have revealed an obscure subgroup of mitochondrial metabolic disorders among the Israeli families in which marriages take place between close relatives, which include diseases like kidney dysfunction, high blood pressure and dementia. Various DNA analyses are being done to identify the genes responsible for such disorders, though an effective treatment has not yet been developed because of their diverse and varying symptoms.
Publication Name: Nature Medicine
Subject: Health
ISSN: 1078-8956
Year: 2006
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