Document Detail


Ragged-red fibers. A biochemical and morphological study.
MedLine Citation:
PMID:  128619     Owner:  NLM     Status:  MEDLINE    
Abstract/OtherAbstract:
Histochemical, ultrastructural and biochemical studies were performed on muscle biopsy specimens from a 30-year-old man with proximal limb weakness. Modified Gomori trichome stains of anterior tibial muscle revealed accumulations of red-staining material in the subsarcolemmal and intermyofibrillar regions of virtually every fiber (ragged-red fibers); these accumulations were rich in oxidative enzymes. Electron microscopy of this muscle showed that the red-staining areas consisted of large collections of abnormal-appearing mitochondria. Mitochondria isolated from the quadriceps muscle showed lack of respiratory control with alpha-glycerophosphate as substrate. However, the lack of respiratory control with alpha-glycerophosphate must be interpreted with caution since the quadriceps muscle was severely degenerated, and biochemical alterations of mitochondria may occur secondary to muscle degeneration itself. Nevertheless, this is the second reported case of lack of respiratory control with alpha-glycerophosphate in a patient with ragged-red fibers. Further interpretation of this defect and its significance must await more studies to determine whether this muscle mitochondrial abnormality is a common finding in the disorders in which ragged-red fibers are encountered.
Authors:
J T Black; D Judge; L Demers; S Gordon
Related Documents :
12767499 - Clinical progression of mitochondrial myopathy is associated with the random accumulati...
21243969 - Magnetic resonance imaging (mri) for assessment of differences in geometrical parameter...
9271809 - Structural and biochemical characteristics of locomotory muscles of emperor penguins, a...
16778569 - Critical illness myopathy: what is happening?
8440289 - Infantile mitochondria encephalomyopathies: report on 4 cases.
9128179 - Application of inhibitor titrations for the detection of oxidative phosphorylation defe...
8739379 - Motor evoked potentials during brain surgery.
20639419 - On muscle, tendon and high heels.
22063949 - An evaluation of the growth, carcass and meat quality characteristics of omani goat bre...
Publication Detail:
Type:  Case Reports; Journal Article    
Journal Detail:
Title:  Journal of the neurological sciences     Volume:  26     ISSN:  0022-510X     ISO Abbreviation:  J. Neurol. Sci.     Publication Date:  1975 Dec 
Date Detail:
Created Date:  1976-03-24     Completed Date:  1976-03-24     Revised Date:  2006-11-15    
Medline Journal Info:
Nlm Unique ID:  0375403     Medline TA:  J Neurol Sci     Country:  NETHERLANDS    
Other Details:
Languages:  eng     Pagination:  479-88     Citation Subset:  IM    
Export Citation:
APA/MLA Format     Download EndNote     Download BibTex
MeSH Terms
Descriptor/Qualifier:
Adenosine Triphosphatases / metabolism
Adult
Glycerophosphates / metabolism
Glycogen / metabolism
Histocytochemistry
Humans
Male
Microscopy, Electron
Mitochondria, Muscle / metabolism*
Muscles / enzymology
Muscular Diseases / enzymology,  metabolism,  pathology*
Myofibrils
Oxidative Phosphorylation
Oxygen Consumption
Chemical
Reg. No./Substance:
0/Glycerophosphates; 9005-79-2/Glycogen; EC 3.6.1.-/Adenosine Triphosphatases

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


Previous Document:  The effect of canaline on some events in vaccinia virus replication.
Next Document:  A new approach to the surgical treatment of lumbar spondylosis.