Document Detail


Low brain intracellular free magnesium in mitochondrial cytopathies.
MedLine Citation:
PMID:  10326720     Owner:  NLM     Status:  MEDLINE    
Abstract/OtherAbstract:
The authors studied, by in vivo phosphorus magnetic resonance spectroscopy (31P-MRS), the occipital lobes of 19 patients with mitochondrial cytopathies to clarify the functional relation between energy metabolism and concentration of cytosolic free magnesium. All patients displayed defective mitochondrial respiration with low phosphocreatine concentration [PCr] and high inorganic phosphate concentration [Pi] and [ADP]. Cytosolic free [Mg2+] and the readily available free energy (defined as the actual free energy released by the exoergonic reaction of ATP hydrolysis, i.e., deltaG(ATPhyd)) were abnormally low in all patients. Nine patients were treated with coenzyme Q10 (CoQ), which improved the efficiency of the respiratory chain, as shown by an increased [PCr], decreased [Pi] and [ADP], and increased availability of free energy (more negative value of deltaG(ATPhyd)). Treatment with CoQ also increased cytosolic free [Mg2+] in all treated patients. The authors findings demonstrate low brain free [Mg2+] in our patients and indicate that it resulted from failure of the respiratory chain. Free Mg2+ contributes to the absolute value of deltaG(ATPhyd). The results also are consistent with the view that cytosolic [Mg2+] is regulated in the intact brain cell to equilibrate, at least in part, any changes in rapidly available free energy.
Authors:
B Barbiroli; S Iotti; P Cortelli; P Martinelli; R Lodi; V Carelli; P Montagna
Publication Detail:
Type:  Clinical Trial; Journal Article; Research Support, Non-U.S. Gov't    
Journal Detail:
Title:  Journal of cerebral blood flow and metabolism : official journal of the International Society of Cerebral Blood Flow and Metabolism     Volume:  19     ISSN:  0271-678X     ISO Abbreviation:  J. Cereb. Blood Flow Metab.     Publication Date:  1999 May 
Date Detail:
Created Date:  1999-05-25     Completed Date:  1999-05-25     Revised Date:  2007-11-15    
Medline Journal Info:
Nlm Unique ID:  8112566     Medline TA:  J Cereb Blood Flow Metab     Country:  UNITED STATES    
Other Details:
Languages:  eng     Pagination:  528-32     Citation Subset:  IM    
Affiliation:
Biochimica Clinica, Dipartimento di Medicina Clinica e Biotecnologia Applicata D. Campanacci, Bologna, Italy.
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MeSH Terms
Descriptor/Qualifier:
Adolescent
Adult
Aged
Brain / metabolism*
Coenzymes
Cytoprotection
Cytosol / metabolism*
Energy Metabolism / physiology
Female
Humans
Magnesium / metabolism*
Magnetic Resonance Spectroscopy / methods
Male
Middle Aged
Mitochondrial Myopathies / drug therapy,  metabolism*
Phosphorus
Ubiquinone / analogs & derivatives,  therapeutic use
Chemical
Reg. No./Substance:
0/Coenzymes; 1339-63-5/Ubiquinone; 303-98-0/coenzyme Q10; 7439-95-4/Magnesium; 7723-14-0/Phosphorus

From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine


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