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Literature summary for 1.1.1.35 extracted from

  • Kapoor, R.R.; James, C.; Flanagan, S.E.; Ellard, S.; Eaton, S.; Hussain, K.
    3-Hydroxyacyl-coenzyme A dehydrogenase deficiency and hyperinsulinemic hypoglycemia: characterization of a novel mutation and severe dietary protein sensitivity (2009), J. Clin. Endocrinol. Metab., 94, 2221-2225.
    View publication on PubMed
Search for more literature for 1.1.1.35

Application

Application Comment Organism
medicine HADH protects against excess amino acid-induced insulin secretion Homo sapiens

Organism

Organism UniProt Comment Textmining
Homo sapiens
-
-
-

Source Tissue

Source Tissue Comment Organism Textmining
leukocyte
-
 Homo sapiens
-

Substrates and Products (Substrate)

Substrates Comment Substrates Organism Products Comment (Products) Rev. Reac.
3-oxohexadecanoyl-CoA + NADH + H+
-
 Homo sapiens (S)-3-hydroxhexadecanoyl-CoA + NAD+
-
 ?
3-oxooctanoyl-CoA + NADH + H+
-
 Homo sapiens (S)-3-hydroxyoctanoyl-CoA + NAD+
-
 ?
acetoacetyl-CoA + NADH + H+
-
 Homo sapiens 3-hydroxybutyryl-CoA + NAD+
-
 ?
additional information HADH gene with a novel homozygous missense mutation M188V. Mutations in the HADH gene are associated with significantly decreased short-chain L-HADH activity, mildly decreased medium- and long-chain L-HADH activity, protein-induced hyperinsulinemic hypoglycemia. Patients with hyperinsulinemic hypoglycemia due to HADH gene mutations may have normal acylcarnitines and urine organic acids Homo sapiens ?
-
 ?

Synonyms

Synonyms Comment Organism
3-hydroxyacyl-coenzyme A dehydrogenase
-
 Homo sapiens
HADH
-
 Homo sapiens