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+ | - |
? |
Organism | UniProt | Comment | Textmining |
---|---|---|---|
Rattus norvegicus | Q64428 | - |
- |
Source Tissue | Comment | Organism | Textmining |
---|---|---|---|
brain | - |
Rattus norvegicus | - |
heart | - |
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+ | - |
? |
Cofactor | Comment | Organism | Structure |
---|---|---|---|
NAD+ | - |
Rattus norvegicus |
General Information | Comment | Organism |
---|---|---|
malfunction | mitochondrial trifunctional protein and long-chain 3-hydroxyacyl-CoA dehydrogenase deficiencies are fatty acid oxidation disorders biochemically characterized by tissue accumulation of long-chain fatty acids and derivatives, including the monocarboxylic long-chain 3-hydroxy fatty acids (LCHFAs) 3-hydroxytetradecanoic acid (3HTA) and 3-hydroxypalmitic acid (3HPA). Deregulation of mitochondrial functions can be provoked by long-chain fatty acid accumulating in long-chain 3-hydroxyacyl-CoA dehydrogenase and mitochondrial permeability transition deficiencies in rat heart. 3HTA and 3HPA significantly decrease mitochondrial membrane potential, the matrix NAD(P)H pool and Ca2+ retention capacity, and also induced mitochondrial swelling. These fatty acids also provoke a marked decrease of ATP production reflecting severe energy dysfunction. 3HTA-induced mitochondrial alterations are completely prevented by the classical mitochondrial permeability transition (mPT) inhibitors cyclosporin A and ADP, as well as by ruthenium red, a Ca2+ uptake blocker, indicating that LCHFAs induce Ca2+-dependent mPT pore opening. Comparison of the susceptibility of heart and brain to the toxic effects of these hydroxylated fatty acids, phenotypes, overview | Rattus norvegicus |