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Literature summary for 4.1.2.63 extracted from

  • Mezzar, S.; De Schryver, E.; Asselberghs, S.; Meyhi, E.; Morvay, P.L.; Baes, M.; Van Veldhoven, P.P.
    Phytol-induced pathology in 2-hydroxyacyl-CoA lyase (HACL1) deficient mice. Evidence for a second non-HACL1-related lyase (2017), Biochim. Biophys. Acta Mol. Cell Biol. Lipids, 1862, 972-990 .
    View publication on PubMed

Localization

Localization Comment Organism GeneOntology No. Textmining
peroxisome
-
Mus musculus 5777
-

Metals/Ions

Metals/Ions Comment Organism Structure
Mg2+ required Mus musculus

Natural Substrates/ Products (Substrates)

Natural Substrates Organism Comment (Nat. Sub.) Natural Products Comment (Nat. Pro.) Rev. Reac.
2-hydroxyphytanoyl-CoA Mus musculus
-
pristanal + formyl-CoA
-
?

Organism

Organism UniProt Comment Textmining
Mus musculus Q9QXE0
-
-

Source Tissue

Source Tissue Comment Organism Textmining
brain
-
Mus musculus
-
hepatocyte
-
Mus musculus
-
liver
-
Mus musculus
-

Substrates and Products (Substrate)

Substrates Comment Substrates Organism Products Comment (Products) Rev. Reac.
2-hydroxyoctadecanoyl-CoA
-
Mus musculus heptadecanal + formyl-CoA
-
?
2-hydroxyphytanoyl-CoA
-
Mus musculus pristanal + formyl-CoA
-
?

Subunits

Subunits Comment Organism
homotetramer 4 * 60000, calculated from amino acid sequence Mus musculus

Synonyms

Synonyms Comment Organism
2-hydroxyacyl-CoA lyase
-
Mus musculus
HACL1
-
Mus musculus

Cofactor

Cofactor Comment Organism Structure
thiamine diphosphate
-
Mus musculus

General Information

General Information Comment Organism
malfunction enzyme deficiency is not associated with a severe phenotype, but in combination with phytanic acid intake it might present with phytanic acid elevation and resemble a Refsum like disorder Mus musculus