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Literature summary for 1.1.1.211 extracted from

  • Cecatto, C.; Hickmann, F.H.; Rodrigues, M.D.; Amaral, A.U.; Wajner, M.
    Deregulation of mitochondrial functions provoked by long-chain fatty acid accumulating in long-chain 3-hydroxyacyl-CoA dehydrogenase and mitochondrial permeability transition deficiencies in rat heart - mitochondrial permeability transition pore opening as a potential contributing pathomechanism of cardiac alterations in these disorders (2015), FEBS J., 282, 4714-4726.
    View publication on PubMed
Search for more literature for 1.1.1.211

Localization

Localization Comment Organism GeneOntology No. Textmining
mitochondrion
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 Rattus norvegicus 5739
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Natural Substrates/ Products (Substrates)

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

Organism

Organism UniProt Comment Textmining
Rattus norvegicus Q64428
-
-

Source Tissue

Source Tissue Comment Organism Textmining
brain
-
 Rattus norvegicus
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heart
-
 Rattus norvegicus
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Substrates and Products (Substrate)

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

Cofactor Comment Organism Structure
NAD+
-
 Rattus norvegicus

General Information

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