Literature summary for 1.1.1.211 extracted from

Haglind, C.; Nordenstroem, A.; Ask, S.; von Doebeln, U.; Gustafsson, J.; Stenlid, M.
Increased and early lipolysis in children with long-chain 3-hydroxyacyl-CoA dehydrogenase (LCHAD) deficiency during fast (2015), J. Inherit. Metab. Dis., 38, 315-322.

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Organism

Organism	UniProt	Comment	Textmining
Homo sapiens	P40939	-	-

Synonyms

Synonyms	Comment	Organism
LCHAD	-	Homo sapiens
long-chain 3-hydroxyacyl-CoA dehydrogenase	-	Homo sapiens

General Information

General Information	Comment	Organism
malfunction	children with long-chain 3-hydroxyacyl-CoA dehydrogenasedeficiency (LCHAD) have a defect in the degradation of long-chain fatty acids and are at risk of hypoketotic hypoglycemia and insufficient energy production as well as accumulation of toxic fatty acid intermediates. lipolysis and accumulation of long chain acylcarnitines occurs before hypoglycemia in fasting children with LCHAD, which may indicate more limited fasting tolerance than previously suggested, phenotypes, overview. Early and increased lipolysis and accumulation of long chain acylcarnitines after 4 h of fasting, albeit no patients developed hypoglycemia	Homo sapiens

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Release 2023.1 (January 2023)