Organism | UniProt | Comment | Textmining |
---|---|---|---|
Homo sapiens | P40939 | - |
- |
Synonyms | Comment | Organism |
---|---|---|
LCHAD | - |
Homo sapiens |
long-chain 3-hydroxyacyl-CoA dehydrogenase | - |
Homo sapiens |
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 |