Document Detail

Genetic disease of mitochondrial function evaluated by NMR and NIR spectroscopy of skeletal tissue.
MedLine Citation:
PMID:  7599229     Owner:  NLM     Status:  MEDLINE    
Bioenergetic sufficiency can be quantitatively assayed by nuclear magnetic resonance spectroscopy (MRS) and on a relative basis by tissue optical spectroscopy (NIRS). Nuclear magnetic resonance measures quantitatively the fall of phosphocreatine and the rise of inorganic phosphate necessary to raise mitochondrial adenosine diphosphate and activate ATP synthesis to adequate level to meet metabolic demands. This relationship is readily demonstrated in skeletal muscle where the quality of supply and demand for ATP is observed over a wide range of aerobic exercise. Metabolic and genetic disease of mitochondria is readily detected by the rapid fall of PCR and rise of Pi during mild exercise and has been essential in the diagnosis and therapy of deficiency of cytochrome bc1 in human skeletal muscle. Insufficiencies of oxygen utilization in relation to oxygen delivery are readily measured optically by the simplest of dual wavelength spectrometers. Instead of deoxygenating hemoglobin during exercise in cases of normal bioenergetic function, a luxury perfusion or hyperoxygenation of skeletal muscles occurs in exercising the energetically deficient skeletal tissue. In this way, a simple screen for metabolic and mitochondrial disease of energy production has been established and demonstrated in a number of clinical cases. Thus, the combination of the absolute evaluations by NMR and the relative indications of light of spectroscopy (NIRS) form essential tools in detection of mitochondrial defects.
B Chance; W Bank
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Publication Detail:
Type:  Journal Article; Research Support, U.S. Gov't, P.H.S.    
Journal Detail:
Title:  Biochimica et biophysica acta     Volume:  1271     ISSN:  0006-3002     ISO Abbreviation:  Biochim. Biophys. Acta     Publication Date:  1995 May 
Date Detail:
Created Date:  1995-08-07     Completed Date:  1995-08-07     Revised Date:  2007-11-14    
Medline Journal Info:
Nlm Unique ID:  0217513     Medline TA:  Biochim Biophys Acta     Country:  NETHERLANDS    
Other Details:
Languages:  eng     Pagination:  7-14     Citation Subset:  IM    
Johnson Research Foundation, Department Biochemistry and Biophysics, University of Pennsylvania, Philadelphia 19104-6089, USA.
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MeSH Terms
Adenosine Triphosphate / metabolism
Energy Metabolism*
Exercise Test
Genetic Diseases, Inborn / metabolism*
Magnetic Resonance Spectroscopy / diagnostic use,  instrumentation,  methods
Mitochondria, Muscle / metabolism*
Mitochondrial Myopathies / diagnosis,  genetics*,  metabolism*
Muscle, Skeletal / metabolism*
Phosphates / metabolism
Phosphocreatine / metabolism
Reproducibility of Results
Spectrophotometry / instrumentation,  methods
Grant Support
Reg. No./Substance:
0/Phosphates; 56-65-5/Adenosine Triphosphate; 67-07-2/Phosphocreatine

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