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Literature summary extracted from

  • Tonin, A.M.; Amaral, A.U.; Busanello, E.N.; Gasparotto, J.; Gelain, D.P.; Gregersen, N.; Wajner, M.
    Mitochondrial bioenergetics deregulation caused by long-chain 3-hydroxy fatty acids accumulating in LCHAD and MTP deficiencies in rat brain: a possible role of mPTP opening as a pathomechanism in these disorders? (2014), Biochim. Biophys. Acta, 1842, 1658-1667.
    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
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 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 brain
-
 Rattus norvegicus
-
1.1.1.211 cerebral cortex
-
 Rattus norvegicus
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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

Temperature Optimum [°C]

EC Number Temperature Optimum [°C] Temperature Optimum Maximum [°C] Comment Organism
1.1.1.211 37
-
 assay at Rattus norvegicus

pH Optimum

EC Number pH Optimum Minimum pH Optimum Maximum Comment Organism
1.1.1.211 7.2
-
 assay at Rattus norvegicus

Cofactor

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

Expression

EC Number Organism Comment Expression
1.1.1.211 Rattus norvegicus nickel strongly represses mitochondrial fatty acid oxidation, the pathway by which fatty acids are catabolized for energy, in both primary human lung fibroblasts and mouse embryonic fibroblasts down

General Information

EC Number General Information Comment Organism
1.1.1.211 malfunction Long-chain 3-hydroxylated fatty acids (LCHFA) accumulate in long-chain 3-hydroxy-acyl-CoA dehydrogenase (LCHAD) and mitochondrial trifunctional protein (MTP) deficiencies. 3-Hydroxytetradecanoic acid (3 HTA) reduces mitochondrial membrane potential, NAD(P)H levels, Ca2+ retention capacity and ATP content, besides inducing swelling, cytochrome c release and H2O2 production in Ca2+-loaded mitochondrial preparations. Cyclosporine A plus ADP, as well as ruthenium red, a Ca2+ uptake blocker, prevent these effects, suggesting the involvement of the mitochondrial permeability transition pore (mPTP) and an important role for Ca2+, respectively. 3-Hydroxydodecanoic and 3-hydroxypalmitic acids, that also accumulate in LCHAD and MTP deficiencies, similarly induce mitochondrial swelling and decrease ATP content, but to a variable degree pending on the size of their carbon chain. Pathological neurological phenotype, detailed overview Rattus norvegicus
1.1.1.211 physiological function long-chain 3-hydroxy-acyl-CoA dehydrogenase (LCHAD) is part of the mitochondrial trifunctional protein (MTP) complex that also comprises other two enzyme activities, long-chain enoyl-CoA hydratase and long-chain ketoacyl-CoA thiolase (LCKT). This complex is responsible for mitochondrial oxidation of long-chain fatty acids (LCFA) Rattus norvegicus