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Literature summary for 1.1.1.211 extracted from

  • Stinton, C.; Fraser, H.; Geppert, J.; Johnson, R.; Connock, M.; Johnson, S.; Clarke, A.; Taylor-Phillips, S.
    Newborn screening for long-chain 3-hydroxyacyl-CoA dehydrogenase and mitochondrial trifunctional protein deficiencies using acylcarnitines measurement in dried blood spots - a systematic review of test accuracy (2021), Front. Pediatr., 9, 606194 .
    View publication on PubMedView publication on EuropePMC
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Natural Substrates/ Products (Substrates)

Natural Substrates Organism Comment (Nat. Sub.) Natural Products Comment (Nat. Pro.) Rev. Reac.
a long-chain (S)-3-hydroxyacyl-CoA + NAD+ Homo sapiens
-
 a long-chain 3-oxoacyl-CoA + NADH + H+
-
 ?

Organism

Organism UniProt Comment Textmining
Homo sapiens
-
-
-

Source Tissue

Source Tissue Comment Organism Textmining
fibroblast
-
 Homo sapiens
-
lymphocyte
-
 Homo sapiens
-

Substrates and Products (Substrate)

Substrates Comment Substrates Organism Products Comment (Products) Rev. Reac.
a long-chain (S)-3-hydroxyacyl-CoA + NAD+
-
 Homo sapiens a long-chain 3-oxoacyl-CoA + NADH + H+
-
 ?

Synonyms

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

Cofactor

Cofactor Comment Organism Structure
NAD+
-
 Homo sapiens

General Information

General Information Comment Organism
malfunction long-chain 3-hydroxyacyl-CoA dehydrogenase deficiency is characterized by lethargy, hypoglycemia, hypotonia, cardiomyopathy, and acute metabolic crisis Homo sapiens