Names & Taxonomy
- Uniprot ID:
- Q9UJ83
- Entry Name:
- HACL1_HUMAN
- Status:
- reviewed
- Protein Names:
- 2-hydroxyacyl-CoA lyase 1 (EC 4.1.-.-) (2-hydroxyphytanoyl-CoA lyase) (2-HPCL) (Phytanoyl-CoA 2-hydroxylase 2)
- Gene Names:
- HACL1 HPCL HPCL2 PHYH2 HSPC279
- Gene Names Orf:
- HSPC279
- Gene Names Primary:
- HACL1
- Organism:
- Homo sapiens (Human)
Structure
- Length:
- 578
- Sequence:
- MPDSNFAERSEEQVSGAKVIAQALKTQDVEYIFGIVGIPVTEIAIAAQQLGIKYIGMRNEQAACYAASAIGYLTSRPGVCLVVSGPGLIHALGGMANANMNCWPLLVIGGSSERNQETMGAFQEFPQVEACRLYTKFSARPSSIEAIPFVIEKAVRSSIYGRPGACYVDIPADFVNLQVNVNSIKYMERCMSPPISMAETSAVCTAASVIRNAKQPLLIIGKGAAYAHAEESIKKLVEQYKLPFLPTPMGKGVVPDNHPYCVGAARSRALQFADVIVLFGARLNWILHFGLPPRYQPDVKFIQVDICAEELGNNVKPAVTLLGNIHAVTKQLLEELDKTPWQYPPESKWWKTLREKMKSNEAASKELASKKSLPMNYYTVFYHVQEQLPRDCFVVSEGANTMDIGRTVLQNYLPRHRLDAGTFGTMGVGLGFAIAAAVVAKDRSPGQWIICVEGDSAFGFSGMEVETICRYNLPIILLVVNNNGIYQGFDTDTWKEMLKFQDATAVVPPMCLLPNSHYEQVMTAFGGKGYFVQTPEELQKSLRQSLADTTKPSLINIMIEPQATRKAQDFHWLTRSNM
- Proteomes:
- UP000005640
Subcellular location
- Subcellular Location:
- Peroxisome.
Function
- Function:
- Catalyzes a carbon-carbon cleavage reaction; cleaves a 2-hydroxy-3-methylacyl-CoA into formyl-CoA and a 2-methyl-branched fatty aldehyde.
- Pathway:
- Lipid metabolism; fatty acid metabolism.
- Catalytic Activity:
- 2-hydroxyphytanoyl-CoA = pristanal + formyl-CoA.
- Cofactor:
- COFACTOR: Name=Mg(2+); Xref=ChEBI:CHEBI:18420; ; Note=Binds 1 Mg(2+) ion per subunit.;; COFACTOR: Name=thiamine diphosphate; Xref=ChEBI:CHEBI:58937; ; Note=Binds 1 thiamine pyrophosphate per subunit.;
- Gene Ontology Go:
- peroxisomal matrix
peroxisome
carbon-carbon lyase activity
cofactor binding
identical protein binding
magnesium ion binding
receptor binding
thiamine pyrophosphate binding
cellular lipid metabolic process
fatty acid alpha-oxidation
protein oligomerization
small molecule metabolic process - Gene Ontology Biological Process:
- cellular lipid metabolic process
fatty acid alpha-oxidation
protein oligomerization
small molecule metabolic process - Gene Ontology Molecular Function:
- carbon-carbon lyase activity
cofactor binding
identical protein binding
magnesium ion binding
receptor binding
thiamine pyrophosphate binding - Gene Ontology Cellular Component:
- peroxisomal matrix
peroxisome - Keywords:
- Alternative splicing
Complete proteome
Lyase
Magnesium
Metal-binding
Peroxisome
Phosphoprotein
Reference proteome
Thiamine pyrophosphate - Interacts With:
- Itself; Q8N7X4; Q96E35