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Mitochondrial myopa...
Mitochondrial myopathy induces a starvation-like response
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- Tyynismaa, Henna (författare)
- Research Program of Molecular Neurology, Biomedicum-Helsinki, Helsinki, Finland
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- Carroll, Christopher J. (författare)
- Research Program of Molecular Neurology, Biomedicum-Helsinki, Helsinki, Finland
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- Raimundo, Nuno (författare)
- Research Program of Molecular Neurology, Biomedicum-Helsinki, Helsinki, Finland
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- Ahola-Erkkilä, Sofia (författare)
- Research Program of Molecular Neurology, Biomedicum-Helsinki, Helsinki, Finland
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- Wenz, Tina (författare)
- Department of Neurology and Cell Biology and Anatomy, University of Miami, Miller School of Medicine, Miami, USA
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- Ruhanen, Heini (författare)
- Research Program of Molecular Neurology, Biomedicum-Helsinki, Helsinki, Finland
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- Guse, Kilian (författare)
- Molecular Cancer Biology Program & Transplantation Laboratory, Haartman Institute & Finnish Institute for Molecular Medicine & HUSLAB & Helsinki University Central Hospital, Cancer Gene Therapy Group, Helsinki, Finland
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- Hemminki, Akseli (författare)
- Molecular Cancer Biology Program & Transplantation Laboratory, Haartman Institute & Finnish Institute for Molecular Medicine & HUSLAB & Helsinki University Central Hospital, Cancer Gene Therapy Group, Helsinki, Finland
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- Peltola-Mjøsund, Katja E. (författare)
- Research Program of Molecular Neurology, Biomedicum-Helsinki, Helsinki, Finland
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- Tulkki, Valtteri (författare)
- Research Program of Molecular Neurology, Biomedicum-Helsinki, Helsinki, Finland
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- Oresic, Matej, 1967- (författare)
- Institute for Molecular Medicine Finland FIMM, University of Helsinki, Helsinki, Finland; VTT Technical Research Centre of Finland, Espoo, VTT, Finland
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- Moraes, Carlos T. (författare)
- Department of Neurology and Cell Biology and Anatomy, University of Miami, Miller School of Medicine, Miami, FL, USA
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- Pietiläinen, Kirsi (författare)
- Obesity Research Unit, Department of Psychiatry, Helsinki University Central Hospital, Helsinki, Finland
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- Hovatta, Iiris (författare)
- Research Program of Molecular Neurology, Biomedicum-Helsinki, Helsinki, Finland; Department of Mental Health and Substance Abuse Services, National Institute for Health and Welfare and Department of Medical Genetics, University of Helsinki, Helsinki, Finland
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- Suomalainen, Anu (författare)
- Research Program of Molecular Neurology, Biomedicum-Helsinki, Helsinki, Finland; Institute for Molecular Medicine Finland FIMM, University of Helsinki, Helsinki, Finland; Department of Neurology, Helsinki, University Central Hospital, Helsinki, Finland
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(creator_code:org_t)
- 2010-07-23
- 2010
- Engelska.
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Ingår i: Human Molecular Genetics. - : Oxford University Press. - 0964-6906 .- 1460-2083. ; 19:20, s. 3948-3958
- Relaterad länk:
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https://doi.org/10.1...
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http://hmg.oxfordjou...
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https://urn.kb.se/re...
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https://doi.org/10.1...
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Abstract
Ämnesord
Stäng
- Mitochondrial respiratory chain (RC) deficiency is among the most common causes of inherited metabolic disease, but its physiological consequences are poorly characterized. We studied the skeletal muscle gene expression profiles of mice with late-onset mitochondrial myopathy. These animals express a dominant patient mutation in the mitochondrial replicative helicase Twinkle, leading to accumulation of multiple mtDNA deletions and progressive subtle RC deficiency in the skeletal muscle. The global gene expression pattern of the mouse skeletal muscle showed induction of pathways involved in amino acid starvation response and activation of Akt signaling. Furthermore, the muscle showed induction of a fasting-related hormone, fibroblast growth factor 21 (Fgf21). This secreted regulator of lipid metabolism was also elevated in the mouse serum, and the animals showed widespread changes in their lipid metabolism: small adipocyte size, low fat content in the liver and resistance to high-fat diet. We propose that RC deficiency induces a mitochondrial stress response, with local and global changes mimicking starvation, in a normal nutritional state. These results may have important implications for understanding the metabolic consequences of mitochondrial myopathies.
Ämnesord
- MEDICIN OCH HÄLSOVETENSKAP -- Medicinska och farmaceutiska grundvetenskaper -- Andra medicinska och farmaceutiska grundvetenskaper (hsv//swe)
- MEDICAL AND HEALTH SCIENCES -- Basic Medicine -- Other Basic Medicine (hsv//eng)
Nyckelord
- Mitochondrial myopathies
- signal transduction
- mutation
- mitochondria
- amino acids
- stress response
- diet
- hormones
- adipocytes
- dna
- mitochondrial
- fasting
- gene expression profiling
- skeletal muscles
- liver
- mice
- proto-oncogene proteins c-akt
Publikations- och innehållstyp
- ref (ämneskategori)
- art (ämneskategori)
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- Av författaren/redakt...
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Tyynismaa, Henna
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Carroll, Christo ...
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Raimundo, Nuno
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Ahola-Erkkilä, S ...
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Wenz, Tina
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Ruhanen, Heini
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Guse, Kilian
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Hemminki, Akseli
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Peltola-Mjøsund, ...
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Tulkki, Valtteri
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Oresic, Matej, 1 ...
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Moraes, Carlos T ...
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Pietiläinen, Kir ...
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Hovatta, Iiris
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Suomalainen, Anu
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Örebro universitet