Document Detail

Alteration of mitochondrial DNA and RNA level in human fibroblasts with impaired vitamin B12 coenzyme synthesis.
MedLine Citation:
PMID:  9720919     Owner:  NLM     Status:  MEDLINE    
Alterations of mitochondrial (mt) nucleic acid metabolism in methylmalonic aciduria (MMA) were studied in two cell lines from skin fibroblasts of patients with mitochondrial (GM00595) or cytosolic (GM10011) defects in the biosynthesis pathways of cobalamin coenzymes. The mtDNA level increased two-fold in GM00595 cells, which carry a mt defect in the adenosylcobalamin synthesis, whereas no appreciable change was found in GM10011 cells. The content of the two rRNAs 16S and 12S mtRNAs, normalized for the mtDNA copy number, decreased by 70% and 50% in GM00595 and GM10011, respectively. The normalized content of ND1, ND2 and CO I mRNAs decreased in GM00595, but was unchanged in GM10011. Respiratory chain complex activities measured in these two cell lines were not different from control activities. These data suggest that the maintenance of the mt function is due to doubling of mtDNA and that this compensatory response takes place only in those cells in which the greater reduction of the level of rRNA might have brought the content of these transcripts below the threshold value for optimal expression of the mt genome.
P Cantatore; V Petruzzella; C Nicoletti; F Papadia; F Fracasso; P Rustin; M N Gadaleta
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Publication Detail:
Type:  Journal Article; Research Support, Non-U.S. Gov't    
Journal Detail:
Title:  FEBS letters     Volume:  432     ISSN:  0014-5793     ISO Abbreviation:  FEBS Lett.     Publication Date:  1998 Aug 
Date Detail:
Created Date:  1998-09-18     Completed Date:  1998-09-18     Revised Date:  2012-02-22    
Medline Journal Info:
Nlm Unique ID:  0155157     Medline TA:  FEBS Lett     Country:  NETHERLANDS    
Other Details:
Languages:  eng     Pagination:  173-8     Citation Subset:  IM    
Department of Biochemistry and Molecular Biology, University of Bari and Centro Studi sui Mitocondri e Metabolismo Energetico, CNR, Italy.
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MeSH Terms
Amino Acid Metabolism, Inborn Errors / genetics,  metabolism
Cell Line
Cell Respiration / genetics,  physiology
Cobamides / biosynthesis*
DNA, Mitochondrial / genetics,  metabolism*
Electron Transport / genetics,  physiology
Fibroblasts / chemistry,  cytology,  metabolism*
Gene Expression Regulation
Methylmalonic Acid / urine
Mitochondria / chemistry,  enzymology,  metabolism
Multienzyme Complexes / genetics,  metabolism
RNA / genetics,  metabolism*
Transcription, Genetic / genetics
Grant Support
Reg. No./Substance:
0/Cobamides; 0/DNA, Mitochondrial; 0/Multienzyme Complexes; 0/RNA, mitochondrial; 516-05-2/Methylmalonic Acid; 63231-63-0/RNA

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