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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.

Author: Nonaka, Ikuya, Ogura, Atsuo, Nakada, Kazuto, Inoue, Kimiko, Ono, Tomoko, Isobe, Kotoyo, Goto, Yu-Ichi, Hayashi, Jun-Ichi
Publisher: Nature America, Inc.
Publication Name: Nature Medicine
Subject: Health
ISSN: 1078-8956
Year: 2001
Care and treatment, Usage

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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.

Author: Hayashi, Masaaki, Imanaka-Yoshida, Kyoko, Yoshida, Toshimichi, Wood, Malcolm, Fearns, Colleen, Tatake, Revati J., Jiing-Dwan Lee
Publisher: Nature America, Inc.
Publication Name: Nature Medicine
Subject: Health
ISSN: 1078-8956
Year: 2006
Connecticut, Health aspects, Causes of, Heat shock proteins, Cardiomyopathy, Hypertrophic, Hypertrophic cardiomyopathy, Tumor proteins, Tumour proteins

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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.

Author: Vikhanski, Luba
Publisher: Nature America, Inc.
Publication Name: Nature Medicine
Subject: Health
ISSN: 1078-8956
Year: 2006
Physiological aspects, Genetic aspects, Israelis, DNA synthesis

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Subjects list: Research, United States, Mitochondrial DNA, Mitochondrial diseases
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