Information on EC 4.1.2.63 - 2-hydroxyacyl-CoA lyase

2-HPCL

3 entries

2-hydroxyacyl-CoA lyase

3 entries

2-hydroxyphytanoyl-CoA lyase

HACL1

HPL

-

a (2R)-2-hydroxy-Cn-long-chain fatty acyl-CoA = a Cn-1-long-chain fatty aldehyde + formyl-CoA

(2)

-

a 2-hydroxy-3-methyl-Cn-fatty-acyl-CoA = a 2-methyl-branched Cn-1-fatty aldehyde + formyl-CoA

(1)

-

,

2-hydroxy-3-methyl fatty-CoA formyl-CoA lyase (2-methyl branched fatty aldehyde-forming)

Requires Mg2+ and thiamine diphosphate. This peroxisomal enzyme, found in animals, is involved in the alpha-oxidation of 3-methyl-branched fatty acids like phytanic acid and the shortening of 2-hydroxy long-chain fatty acids.

2-hydroxy-3-methylhexadecanoyl-CoA

2-hydroxy-3-methylpentadecanal + formyl-CoA

Rattus norvegicus

Q8CHM7

-

761438

-

?

2-hydroxy-3-methylhexadecanoyl-CoA

2-methylpentadecanal + formyl-CoA

2 entries

2-hydroxy-3-methylhexadecanoyl-CoA

3-methylpentadecanal + formyl-CoA

Homo sapiens

Q9UJ83

-

760615

-

?

2-hydroxyoctadecanoyl-CoA

heptadecanal + formyl-CoA

3 entries

2-hydroxyphytanoyl-CoA

pristanal + formyl-CoA

4 entries

a 2-hydroxy-3-methyl-Cn-fatty-acyl-CoA

a 2-methyl-branched Cn-1-fatty aldehyde + formyl-CoA

4 entries

additional information

?

-

Rattus norvegicus

Q8CHM7

no activity toward 3-methylhexadecanoyl-CoA, hexadecanoyl-CoA, 3-hydroxyhexadecanoyl-CoA, octadecanoyl-CoA, 2-hydroxy-3-methylhexadecanoic acid, 3-methylhexadecanoic acid, hexadecanoic acid, and 2-hydroxyhexadecanoic acid. The addition of 0.05 mM 2-hydroxyhexadecanoyl-CoA or 2-hydroxyoctadecanoyl-CoA to incubations with 0.01 mM 2-hydroxy-3-methylhexadecanoyl-CoA reduces the cleavage rates to a major extent

761438

-

2-hydroxyphytanoyl-CoA

pristanal + formyl-CoA

4 entries

a 2-hydroxy-3-methyl-Cn-fatty-acyl-CoA

a 2-methyl-branched Cn-1-fatty aldehyde + formyl-CoA

4 entries

ATP

Rattus norvegicus

Q8CHM7

dependent on. No oxidation is detected upon the addition of GTP or AMP

761438

CoA

Rattus norvegicus

Q8CHM7

dependent on. CoA cannot be replaced by desulfo-CoA or dephospho-CoA

761438

NAD+

Rattus norvegicus

Q8CHM7

dependent on. Extra addition of NAD+ results in a 20% increase of total oxidation

761438

thiamine diphosphate

6 entries

Mg2+

6 entries

additional information

Rattus norvegicus

Q8CHM7

not inhibited in the presence of 2-keto-octanoate, 2-hydroxyhexadecanoic acid, 2-methylhexadecanoyl-CoA, 2-methylhexadecanoic acid, 3-methylhexadecanoyl-CoA, 3-hydroxy-3-methylhexadecanoyl-CoA, or 3-hydroxy-2-methylhexadecanoyl-CoA. 2-Hydroxy straight chain acyl-CoA esters compete with 2-hydroxy-3-methyl-branched acyl-CoAs for cleavage by the enzyme

761438

-

0.015

2-hydroxy-3-methylhexadecanoyl-CoA

at pH 7.5 and 37°C

500

recombinant wild type enzyme, with 2-hydroxy-3-methylhexadecanoyl-CoA as substrate, pH and temperature not specified in the publication

7.5 - 8

8

-

-

760544, 760615, 762299

SwissProt

-

SwissProt

-

760472, 760544, 760613, 761438, 762299

SwissProt

-

highest expression in liver and kidney

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

metabolism

?

homotetramer

D455S

the mutation within the thiamine diphosphate binding domain of the enzyme does not affect the targeting upon expression in transfected CHO cells, although enzyme activity is reduced to 5.38% of wild type activity

760615

D455S/S456R

Homo sapiens

Q9UJ83

the mutation within the thiamine diphosphate binding domain of the enzyme does not affect the targeting upon expression in transfected CHO cells, although enzyme activity is reduced to 2.43% of wild type activity

760615

S456R

Homo sapiens

Q9UJ83

the mutation within the thiamine diphosphate binding domain of the enzyme does not affect the targeting upon expression in transfected CHO cells, although enzyme activity is reduced to 2.17% of wild type activity

760615

DEAE-Sepharose column chromatography, phosphocellulose chromatography, and Ultrogel AcA-34 gel filtration

Rattus norvegicus

Q8CHM7

762299

Ni-NTA agarose column chromatography

Rattus norvegicus

Q8CHM7

761438

expressed in CHO cells and Saccharomyces cerevisiae strain CB80

Homo sapiens

Q9UJ83

760615

expressed in Saccharomyces cerevisiae strain CB80

Rattus norvegicus

Q8CHM7

761438

ExplorEnz (official portal for IUBMB Enzyme Nomenclature)

Expasy Enzyme Nomenclature Database

KEGG

MetaCyc

SABIO-RK

NCBI: PubMed, Protein, Nucleotide, Structure, Gene, OMIM

Enzyme Nomenclature (alternative site)

UniProt

PDB

PROSITE Database of protein families and domains

InterPro (database of protein families, domains and functional sites)