Localization | Comment | Organism | GeneOntology No. | Textmining |
---|---|---|---|---|
mitochondrion | - |
Rattus norvegicus | 5739 | - |
Organism | UniProt | Comment | Textmining |
---|---|---|---|
Rattus norvegicus | Q64428 | - |
- |
Rattus norvegicus Wistar | Q64428 | - |
- |
Source Tissue | Comment | Organism | Textmining |
---|---|---|---|
skeletal muscle | - |
Rattus norvegicus | - |
Synonyms | Comment | Organism |
---|---|---|
LCHAD | - |
Rattus norvegicus |
long-chain 3-hydroxy-acyl-CoA dehydrogenase | - |
Rattus norvegicus |
General Information | Comment | Organism |
---|---|---|
malfunction | analysis of the effects of the major long-chain monocarboxylic 3-hydroxylated fatty acids (LCHFA) accumulating in the disorders mitochondrial trifunctional protein (MTP) and long-chain 3-hydroxy-acyl-CoA dehydrogenase (LCHAD) deficiencies, namely 3-hydroxytetradecanoic (3HTA) and 3-hydroxypalmitic (3HPA) acids, on important mitochondrial functions in rat skeletal muscle mitochondria 3HTA and 3HPA markedly increases resting and decrease ADP-stimulated and CCCP-stimulated (uncoupled) respiration. 3HPA provokes similar effects in permeabilized skeletal muscle fibers, validating the results obtained in purified mitochondria. 3HTA and 3HPA markedly diminish mitochondrial membrane potential, NAD(P)H content, and Ca2+ retention capacity in Ca2+-loaded mitochondria. Mitochondrial permeability transition (mPT) induction probably underlies these effects since they are totally prevented by cyclosporin A and ADP. In contrast, the dicarboxylic analogue of 3HTA does not alter the tested parameters. 3HTA and 3HPA behave as metabolic inhibitors, uncouplers of oxidative phosphorylation and mPT inducers in skeletal muscle. The pathomechanisms disrupting mitochondrial homeostasis may be involved in the muscle alterations characteristic ofMTP and LCHAD deficiencies | Rattus norvegicus |