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Literature summary extracted from

  • Hickmann, F.H.; Cecatto, C.; Kleemann, D.; Monteiro, W.O.; Castilho, R.F.; Amaral, A.U.; Wajner, M.
    Uncoupling, metabolic inhibition and induction of mitochondrial permeability transition in rat liver mitochondria caused by the major long-chain hydroxyl monocarboxylic fatty acids accumulating in LCHAD deficiency (2015), Biochim. Biophys. Acta, 1847, 620-628.
    View publication on PubMed

Localization

EC Number Localization Comment Organism GeneOntology No. Textmining
1.1.1.211 mitochondrion
-
 Rattus norvegicus 5739
-

Natural Substrates/ Products (Substrates)

EC Number Natural Substrates Organism Comment (Nat. Sub.) Natural Products Comment (Nat. Pro.) Rev. Reac.
1.1.1.211 a long-chain (S)-3-hydroxyacyl-CoA + NAD+ Rattus norvegicus
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 a long-chain 3-oxoacyl-CoA + NADH + H+
-
 ?
1.1.1.211 a long-chain (S)-3-hydroxyacyl-CoA + NAD+ Rattus norvegicus Wistar
-
 a long-chain 3-oxoacyl-CoA + NADH + H+
-
 ?

Organism

EC Number Organism UniProt Comment Textmining
1.1.1.211 Rattus norvegicus Q64428
-
-
1.1.1.211 Rattus norvegicus Wistar Q64428
-
-

Source Tissue

EC Number Source Tissue Comment Organism Textmining
1.1.1.211 liver
-
 Rattus norvegicus
-

Substrates and Products (Substrate)

EC Number Substrates Comment Substrates Organism Products Comment (Products) Rev. Reac.
1.1.1.211 a long-chain (S)-3-hydroxyacyl-CoA + NAD+
-
 Rattus norvegicus a long-chain 3-oxoacyl-CoA + NADH + H+
-
 ?
1.1.1.211 a long-chain (S)-3-hydroxyacyl-CoA + NAD+
-
 Rattus norvegicus Wistar a long-chain 3-oxoacyl-CoA + NADH + H+
-
 ?

Synonyms

EC Number Synonyms Comment Organism
1.1.1.211 LCHAD
-
 Rattus norvegicus
1.1.1.211 long-chain 3-hydroxy-acyl-CoA dehydrogenase
-
 Rattus norvegicus

Cofactor

EC Number Cofactor Comment Organism Structure
1.1.1.211 NAD+
-
 Rattus norvegicus

General Information

EC Number General Information Comment Organism
1.1.1.211 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