Document Detail

Low succinate dehydrogenase (SDH) activity in a patient with a hereditary myopathy with paroxysmal myoglobinuria.
MedLine Citation:
PMID:  2384736     Owner:  NLM     Status:  MEDLINE    
An unusual hereditary myopathy with paroxysmal myoglobinuria has been described previously. We have studied muscle biopsy specimens taken before and after exercise to exhaustion (24 min at 20-25 W) in a young woman with this condition. Marked glycogenolysis with lactate production and marked phosphagen breakdown (ATP + CP) were observed after exercise, and almost all type I fibres were found to be depleted of glycogen. Succinate dehydrogenase (SDH) activity was low, while the activities of 3-OH-acyl-CoA-dehydrogenase, phosphofructokinase, phosphorylase and lactate dehydrogenase were normal. On electron microscopy, the mitochondria showed abnormalities typical of mitochondrial myopathy. The findings in our patient suggest a limitation of mitochondrial function, probably related to SDH in the tricarboxylic acid cycle and complex II in the electron transport chain. This may explain the inability of ATP regeneration to keep pace with ATP utilization during exercise. Other metabolic defects may coexist.
H Linderholm; B Essén-Gustavsson; L E Thornell
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Publication Detail:
Type:  Case Reports; Journal Article; Research Support, Non-U.S. Gov't    
Journal Detail:
Title:  Journal of internal medicine     Volume:  228     ISSN:  0954-6820     ISO Abbreviation:  J. Intern. Med.     Publication Date:  1990 Jul 
Date Detail:
Created Date:  1990-09-18     Completed Date:  1990-09-18     Revised Date:  2006-11-15    
Medline Journal Info:
Nlm Unique ID:  8904841     Medline TA:  J Intern Med     Country:  ENGLAND    
Other Details:
Languages:  eng     Pagination:  43-52     Citation Subset:  IM    
Department of Clinical Physiology, University of Umeå, Sweden.
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MeSH Terms
Exercise Test
Glycerolphosphate Dehydrogenase / metabolism
Muscles / pathology,  ultrastructure
Muscular Diseases / enzymology*,  pathology,  physiopathology
Myoglobinuria / enzymology*,  genetics,  pathology,  physiopathology
NADH Tetrazolium Reductase / metabolism
Rhabdomyolysis / enzymology*
Succinate Dehydrogenase / deficiency*
Reg. No./Substance:
EC 1.1.-/Glycerolphosphate Dehydrogenase; EC Dehydrogenase; EC 1.6.-/NADH Tetrazolium Reductase

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