Localization | Comment | Organism | GeneOntology No. | Textmining |
---|---|---|---|---|
mitochondrion | - |
Rattus norvegicus | 5739 | - |
Natural Substrates | Organism | Comment (Nat. Sub.) | Natural Products | Comment (Nat. Pro.) | Rev. | Reac. |
---|---|---|---|---|---|---|
a long-chain (S)-3-hydroxyacyl-CoA + NAD+ | Rattus norvegicus | - |
a long-chain 3-oxoacyl-CoA + NADH + H+ | - |
? | |
a long-chain (S)-3-hydroxyacyl-CoA + NAD+ | Rattus norvegicus Wistar | - |
a long-chain 3-oxoacyl-CoA + NADH + H+ | - |
? |
Organism | UniProt | Comment | Textmining |
---|---|---|---|
Rattus norvegicus | Q64428 | - |
- |
Rattus norvegicus Wistar | Q64428 | - |
- |
Source Tissue | Comment | Organism | Textmining |
---|---|---|---|
liver | - |
Rattus norvegicus | - |
Substrates | Comment Substrates | Organism | Products | Comment (Products) | Rev. | Reac. |
---|---|---|---|---|---|---|
a long-chain (S)-3-hydroxyacyl-CoA + NAD+ | - |
Rattus norvegicus | a long-chain 3-oxoacyl-CoA + NADH + H+ | - |
? | |
a long-chain (S)-3-hydroxyacyl-CoA + NAD+ | - |
Rattus norvegicus Wistar | a long-chain 3-oxoacyl-CoA + NADH + H+ | - |
? |
Synonyms | Comment | Organism |
---|---|---|
LCHAD | - |
Rattus norvegicus |
long-chain 3-hydroxy-acyl-CoA dehydrogenase | - |
Rattus norvegicus |
Cofactor | Comment | Organism | Structure |
---|---|---|---|
NAD+ | - |
Rattus norvegicus |
General Information | Comment | Organism |
---|---|---|
malfunction | effects of long-chain 3-hydroxylated fatty acids (LCHFA) that accumulate in LCHAD deficiency on liver bioenergetics in mitochondrial preparations from young rats: 3-hydroxytetradecanoic (3HTA) and 3-hydroxypalmitic (3HPA) acids, the monocarboxylic acids that are found at the highest tissue concentrations in this disorder, act as metabolic inhibitors and uncouplers of oxidative phosphorylation. These fatty acids decrease ADP-stimulated and uncoupled respiration, mitochondrial membrane potential and NAD(P)H content, and, in contrast, increased resting respiration. 3HTA and 3HPA markedly reduce Ca2+ retention capacity and induce swelling in C2+-loaded mitochondria. The effects are mediated by mitochondrial permeability transition (MPT) induction since they are totally prevented by the classical MPT inhibitors cyclosporin A and ADP, as well as by ruthenium red, a Ca2+ uptake blocker. Long-chain monocarboxylic 3-hydroxylated fatty acids alter oxygen consumption in liver mitochondria. The major monocarboxylic LCHFA accumulating in LCHAD deficiency disrupt energy mitochondrial homeostasis in the liver leading to liver dysfunction | Rattus norvegicus |