Carnitine stimulation of pyruvate dehydrogenase complex (PDHC) in isolated human skeletal muscle mitochondria

Muscle & Nerve

Volumn 11, Issue 7, 1988, Pages 720-724

  Uziel, Graziella ^a   Garavaglia, Barbara ^a   Di Donato, Stefano ^a  

^a DEPARTMENT OF NEUROSURGERY   (Italy)

Author keywords

[No Author keywords available]

Indexed keywords

ACETYL COENZYME A; ALPHA CYANOHYDROXYCINNAMIC ACID; CARNITINE; GAMMA BUTYROBETAINE; PYRUVATE DEHYDROGENASE; PYRUVIC ACID; RADIOISOTOPE; SULFOBETAINE; UNCLASSIFIED DRUG;

HISTOCHEMISTRY; HUMAN; HUMAN CELL; MUSCLE MITOCHONDRION; PRIORITY JOURNAL;

ACETYL COENZYME A; ACETYLCARNITINE; CARNITINE; CARNITINE O-ACETYLTRANSFERASE; HUMAN; MITOCHONDRIA, MUSCLE; OXIDATION-REDUCTION; PYRUVATE DEHYDROGENASE COMPLEX; PYRUVATES; PYRUVIC ACID; STIMULATION, CHEMICAL; SUPPORT, NON-U.S. GOV'T;

EID: 0023904816     PISSN: 0148639X     EISSN: 10974598     Source Type: Journal    
DOI: 10.1002/mus.880110708     Document Type: Article

References (24)

1. A rapid and sensitive method for the quantification of microgram quantities of protein utilizing the principle of protein dye binding
2. Carnitine in intermediary metabolism. Reversible acetylation of carnitine by mitochondria
3. Function and regulation of mammalian pyruvate‐dehydrogenase complex
4. Secondary carnitine insufficiency in disorders of organic acid metabolism: modulation of acylCoA/CoA ratios by L‐carnitine in vivo
5. Function of carnitine in the fatty acid oxidase deficient insect fly muscle
6. On the oxidation of acetate and pyruvate by guinea‐pig heart sarcomes
7. Propionylcarnitine excretion in propionic and methylmalonic acidurias: a cause of carnitine deficiency
8. Fatal ataxic encephalopathy and carnitine acetyltransferase deficiency: a functional defect of pyruvate oxidation?
9. Fatal infantile mitochondrial myopathy and renal dysfunction due to cytochrome‐c‐oxidase deficiency
10. Carnitine‐acetyltransferase II. Inhibition by carnitine analogues and sulphidryl reagents
11. Long‐chain carnitine acyltransferase and the role of acylcarnitine derivates in the catalytic increase of fatty acid oxidations induced by carnitine
12. Carnitine‐palmitoyltransferase. Location of two enzymatic activities in rat liver mitochondria
13. Alpha‐ketoacid‐dehydrogenase complexes. X. Regulation of the activity of the pyruvate‐dehydrogenase complex from beef kidney mitochondria by phosphorylation and dephosphorylation
14. Biochemical studies of skeletal muscle mitochondria
15. Role of carnitine in hepatic ketogenesis
16. Mechanism of carnitine acylcarnitine translocase catalyzed import of acylcarnitines into mitochondria
17. A simple radioisotopic assay of acetylcarnitine and acetylCoA at picomolr levels
18. Carnitine‐acylcarnitine translocase. Inhibition by alpha‐cyano‐4‐hydroxycinnamate and evidence for separate identity from the pyruvate transporting system of mitochondria
19. Cytochrome‐c‐oxidase deficiency in muscles of a floppy infant without mitochondrial myopathy
20. Evidence for lipoic acid mediated NADH and acetylCoA stimulation of liver and kidney pyruvate dehydrogenase kinase
21. L‐carnitine therapy in propionic acidaemia
22. Medium‐chain acylCoA dehydrogenase deficiency in children with non‐ketotic hypoglycemia and low carnitine levels
23. Pyruvate dehydrogenase complex in ataxic patients: enzyme deficiency in ataxic encephalopathy plus lactic acidosis and normal activity in Friedreich ataxia
24. Biological oxidation