Blue native polyacrylamide gel electrophoresis: A powerful tool in diagnosis of oxidative phosphorylation defects

Pediatric Research

Volumn 50, Issue 5, 2001, Pages 658-665

  Van Coster, Rudy ^a   Smet, Joel ^a   George, Edith ^a   De Meirleir, Linda ^b   Seneca, Sara ^b   Van Hove, Johan ^c   Sebire, Guillaume ^d   Verhelst, Helene ^a   De Bleecker, Jan ^a   Van Vlem, Bruno ^a   Verloo, Patrick ^a   Leroy, Jules ^a  

^a GHENT UNIVERSITY HOSPITAL   (Belgium)

^b VRIJE UNIVERSITEIT BRUSSEL   (Belgium)

^c UNIVERSITY HOSPITALS LEUVEN   (Belgium)

^d CLINIQUES UNIVERSITAIRES SAINT LUC   (Belgium)

Author keywords

[No Author keywords available]

Indexed keywords

ANAZOLENE SODIUM; CELL NUCLEUS DNA; COMPLEX V; CYTOCHROME C OXIDASE; MITOCHONDRIAL DNA; PYROXYLIN; REDUCED NICOTINAMIDE ADENINE DINUCLEOTIDE DEHYDROGENASE (UBIQUINONE); SUCCINATE DEHYDROGENASE (UBIQUINONE); UNCLASSIFIED DRUG;

ARTICLE; CATALYSIS; CELL LEVEL; CLINICAL ARTICLE; ENZYME ACTIVITY; ENZYME DEFICIENCY; HEART; HUMAN; HUMAN CELL; HUMAN TISSUE; IMMUNOBLOTTING; IMMUNOHISTOCHEMISTRY; LIVER; OXIDATIVE PHOSPHORYLATION; POLYACRYLAMIDE GEL ELECTROPHORESIS; PRIORITY JOURNAL; SILVER STAINING; SKELETAL MUSCLE; SKIN FIBROBLAST; SPECTROPHOTOMETRY; TISSUE LEVEL;

EID: 0034775551     PISSN: 00313998     EISSN: None     Source Type: Journal    
DOI: 10.1203/00006450-200111000-00020     Document Type: Article

References (13)

1. Blue native electrophoresis for isolation of membrane protein complexes in enzymatically active form
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3. Reference charts for respiratory chain activities in human tissues
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5. Preparation of succinate dehydrogenase and reconstruction of succinate oxidase
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7. Citrate synthase
8. Assessment of deficiencies of fatty acyl-CoA dehydrogenases in fibroblasts, muscle and liver
9. A rapid and sensitive method for the quantitation of microgram quantities of protein utilizing the principle of protein-dye binding
10. SURFEIT-1 gene analysis and two-dimensional blue native gel electrophoresis in cytochrome c oxidase deficiency
11. Fatal infantile cardioencephalomyopathy with COX deficiency and mutations in SCO2, a COX assembly gene
12. Sudden deterioration in a 5-month-old baby with complex II deficiency and homozygous mutation in the 70-kDa flavoprotein gene (Gly555Glu)
13. Combined enzymatic complex I and III deficiency associated with mutations in the nuclear encoded NDUFS4 gene