Literature summary for 1.1.1.211 extracted from

Cecatto, C.; Godoy, K.d.o.s..S.; da Silva, J.C.; Amaral, A.U.; Wajner, M.
Disturbance of mitochondrial functions provoked by the major long-chain 3-hydroxylated fatty acids accumulating in MTP and LCHAD deficiencies in skeletal muscle (2016), Toxicol. In Vitro, 36, 1-9.

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Localization

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

Organism

Organism	UniProt	Comment	Textmining
Rattus norvegicus	Q64428	-	-
Rattus norvegicus Wistar	Q64428	-	-

Source Tissue

Source Tissue	Comment	Organism	Textmining
skeletal muscle	-	Rattus norvegicus	-

Synonyms

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

General Information

General Information	Comment	Organism
malfunction	analysis of the effects of the major long-chain monocarboxylic 3-hydroxylated fatty acids (LCHFA) accumulating in the disorders mitochondrial trifunctional protein (MTP) and long-chain 3-hydroxy-acyl-CoA dehydrogenase (LCHAD) deficiencies, namely 3-hydroxytetradecanoic (3HTA) and 3-hydroxypalmitic (3HPA) acids, on important mitochondrial functions in rat skeletal muscle mitochondria 3HTA and 3HPA markedly increases resting and decrease ADP-stimulated and CCCP-stimulated (uncoupled) respiration. 3HPA provokes similar effects in permeabilized skeletal muscle fibers, validating the results obtained in purified mitochondria. 3HTA and 3HPA markedly diminish mitochondrial membrane potential, NAD(P)H content, and Ca2+ retention capacity in Ca2+-loaded mitochondria. Mitochondrial permeability transition (mPT) induction probably underlies these effects since they are totally prevented by cyclosporin A and ADP. In contrast, the dicarboxylic analogue of 3HTA does not alter the tested parameters. 3HTA and 3HPA behave as metabolic inhibitors, uncouplers of oxidative phosphorylation and mPT inducers in skeletal muscle. The pathomechanisms disrupting mitochondrial homeostasis may be involved in the muscle alterations characteristic ofMTP and LCHAD deficiencies	Rattus norvegicus

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Release 2023.1 (January 2023)