Cloned (Comment) | Organism |
---|---|
gene HADHSC, real-time quantitative PCR expression analysis in in INS832/13 beta-cells | Homo sapiens |
Protein Variants | Comment | Organism |
---|---|---|
additional information | knockdown of HADHSC expression by RNA interference in INS832/13 beta-cells using short hairpin RNA and short interference RNA | Homo sapiens |
Organism | UniProt | Comment | Textmining |
---|---|---|---|
Homo sapiens | - |
- |
- |
Source Tissue | Comment | Organism | Textmining |
---|---|---|---|
INS-1 823/13 cell | - |
Homo sapiens | - |
Synonyms | Comment | Organism |
---|---|---|
HADHSC | - |
Homo sapiens |
short-chain 3-hydroxyacyl-CoA dehydrogenase | - |
Homo sapiens |
General Information | Comment | Organism |
---|---|---|
malfunction | patients with mutated beta-oxidation enzyme short-chain 3-hydroxyacyl-CoA dehydrogenase show hyperinsulinemia associated with non-ketotic hypoglycemia, analysis of the mechanism underlying HADHSC-mediated regulation of insulin secretion, overview. Enhanced glucose-stimulated insulin secretion induced by HADHSC knockdown is independent of changes in cytosolic Ca2+ and also occurs in the presence of fatty acids. The pan transaminase inhibitor amino-oxyacetate reverses HADHSC knockdown-mediated increases in glucose-stimulated insulin secretion. Oxidation of palmitate and octanoate is not reduced in HADHSC knockdown cells. L-3-Hydroxybutyryl-carnitine and L-3-hydroxyglutarate, which accumulate in blood and urine, respectively, of HADHSC-deficient patients, do not change insulin secretion. Transamination reaction(s) and the formation of short-chain acylcarnitines and CoAs may be implicated in the mechanism whereby HADHSC deficiency results in enhanced insulin secretion and hyperinsulinemia | Homo sapiens |