High resolution respirometry of permeabilized skeletal muscle fibers in the diagnosis of neuromuscular disorders

Molecular and Cellular Biochemistry

Volumn 174, Issue 1-2, 1997, Pages 71-78

  Sperl, Wolfgang ^a,c   Skladal, Daniela ^a   Gnaiger, Erich ^a   Wyss, Markus ^a   Mayr, Udo ^a   Hager, Josef ^a   Gellerich, Frank Norbert ^b  

^a UNIVERSITY OF INNSBRUCK   (Austria)

^b MARTIN LUTHER UNIVERSITY HALLE WITTENBERG   (Germany)

^c Children's Hospital Salzburg   (Austria)

Author keywords

Mitochondrial encephalomyopathy; Muscular dystrophy duchenne; Respirometry; Skinned muscle fibers; Spinal muscular atrophy

Indexed keywords

ASCORBIC ACID; CARNITINE DERIVATIVE; CYTOCHROME C OXIDASE; MALIC ACID; MITOCHONDRIAL ENZYME; PALMITOYLCARNITINE; PYRUVATE DEHYDROGENASE COMPLEX; PYRUVIC ACID; REDUCED NICOTINAMIDE ADENINE DINUCLEOTIDE DEHYDROGENASE (UBIQUINONE); SAPONIN; SUCCINIC ACID;

ARTICLE; CHILD; CLINICAL ARTICLE; CONTROLLED STUDY; DUCHENNE MUSCULAR DYSTROPHY; ENCEPHALOMYOPATHY; ENZYME DEFECT; ENZYME DEFICIENCY; ENZYME INHIBITION; ENZYME SUBSTRATE; HUMAN; HUMAN TISSUE; MITOCHONDRIAL RESPIRATION; MUSCLE BIOPSY; MUSCLE MITOCHONDRION; MUSCLE TISSUE; NEUROMUSCULAR DISEASE; OXIDATIVE PHOSPHORYLATION; PERMEABILITY; SKELETAL MUSCLE; SKINNED MUSCLE FIBER; SPINAL MUSCULAR ATROPHY; SPIROMETRY;

CHILD; CHILD, PRESCHOOL; ELECTRON TRANSPORT; HUMANS; MITOCHONDRIA, MUSCLE; MITOCHONDRIAL ENCEPHALOMYOPATHIES; MUSCLE, SKELETAL; MUSCULAR DYSTROPHIES; MYOFIBRILS; PERMEABILITY;

EID: 0030869158     PISSN: 03008177     EISSN: None     Source Type: Journal    
DOI: 10.1023/A:1006880529195     Document Type: Article

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