LIPID MAPS

Gene/Proteome Database (LMPD)

LMPD ID

LMP002572

Gene ID

26061

Species

Homo sapiens (Human)

Gene Name

2-hydroxyacyl-CoA lyase 1

Gene Symbol

HACL1

Synonyms

2-HPCL; HPCL; HPCL2; PHYH2

Chromosome

Map Location

3p25.1

EC Number

4.1.-.-

Proteins

2-hydroxyacyl-CoA lyase 1 isoform a
Refseq ID	NP_036392
Protein GI	93004078
UniProt ID	Q9UJ83
mRNA ID	NM_012260
Length	578
RefSeq Status	VALIDATED
MPDSNFAERSEEQVSGAKVIAQALKTQDVEYIFGIVGIPVTEIAIAAQQLGIKYIGMRNEQAACYAASAIGYLTSRPGVCLVVSGPGLIHALGGMANANMNCWPLLVIGGSSERNQETMGAFQEFPQVEACRLYTKFSARPSSIEAIPFVIEKAVRSSIYGRPGACYVDIPADFVNLQVNVNSIKYMERCMSPPISMAETSAVCTAASVIRNAKQPLLIIGKGAAYAHAEESIKKLVEQYKLPFLPTPMGKGVVPDNHPYCVGAARSRALQFADVIVLFGARLNWILHFGLPPRYQPDVKFIQVDICAEELGNNVKPAVTLLGNIHAVTKQLLEELDKTPWQYPPESKWWKTLREKMKSNEAASKELASKKSLPMNYYTVFYHVQEQLPRDCFVVSEGANTMDIGRTVLQNYLPRHRLDAGTFGTMGVGLGFAIAAAVVAKDRSPGQWIICVEGDSAFGFSGMEVETICRYNLPIILLVVNNNGIYQGFDTDTWKEMLKFQDATAVVPPMCLLPNSHYEQVMTAFGGKGYFVQTPEELQKSLRQSLADTTKPSLINIMIEPQATRKAQDFHWLTRSNM

2-hydroxyacyl-CoA lyase 1 isoform b
Refseq ID	NP_001271342
Protein GI	548923731
UniProt ID	Q9UJ83
mRNA ID	NM_001284413
Length	551
RefSeq Status	VALIDATED
MPDSNFAERSEEQVSGAKVIAQALKTQDVEYIFGIVGIPVTEIAIAAQQLGIKYIGMRNEQAACYAASAIGYLTSRPLLVIGGSSERNQETMGAFQEFPQVEACRLYTKFSARPSSIEAIPFVIEKAVRSSIYGRPGACYVDIPADFVNLQVNVNSIKYMERCMSPPISMAETSAVCTAASVIRNAKQPLLIIGKGAAYAHAEESIKKLVEQYKLPFLPTPMGKGVVPDNHPYCVGAARSRALQFADVIVLFGARLNWILHFGLPPRYQPDVKFIQVDICAEELGNNVKPAVTLLGNIHAVTKQLLEELDKTPWQYPPESKWWKTLREKMKSNEAASKELASKKSLPMNYYTVFYHVQEQLPRDCFVVSEGANTMDIGRTVLQNYLPRHRLDAGTFGTMGVGLGFAIAAAVVAKDRSPGQWIICVEGDSAFGFSGMEVETICRYNLPIILLVVNNNGIYQGFDTDTWKEMLKFQDATAVVPPMCLLPNSHYEQVMTAFGGKGYFVQTPEELQKSLRQSLADTTKPSLINIMIEPQATRKAQDFHWLTRSNM

2-hydroxyacyl-CoA lyase 1 isoform c
Refseq ID	NP_001271344
Protein GI	548923731
UniProt ID	Q9UJ83
mRNA ID	NM_001284415
Length	551
RefSeq Status	VALIDATED
MPDSNFAERSEEQVSGAKVIAQALKTQDVEYIFGIVGIPVTEIAIAAQQLGIKYIGMRNEQAACYAASAIGYLTSRPLLVIGGSSERNQETMGAFQEFPQVEACRLYTKFSARPSSIEAIPFVIEKAVRSSIYGRPGACYVDIPADFVNLQVNVNSIKYMERCMSPPISMAETSAVCTAASVIRNAKQPLLIIGKGAAYAHAEESIKKLVEQYKLPFLPTPMGKGVVPDNHPYCVGAARSRALQFADVIVLFGARLNWILHFGLPPRYQPDVKFIQVDICAEELGNNVKPAVTLLGNIHAVTKQLLEELDKTPWQYPPESKWWKTLREKMKSNEAASKELASKKSLPMNYYTVFYHVQEQLPRDCFVVSEGANTMDIGRTVLQNYLPRHRLDAGTFGTMGVGLGFAIAAAVVAKDRSPGQWIICVEGDSAFGFSGMEVETICRYNLPIILLVVNNNGIYQGFDTDTWKEMLKFQDATAVVPPMCLLPNSHYEQVMTAFGGKGYFVQTPEELQKSLRQSLADTTKPSLINIMIEPQATRKAQDFHWLTRSNM

2-hydroxyacyl-CoA lyase 1 isoform d
Refseq ID	NP_001271345
Protein GI	548923675
UniProt ID	Q9UJ83
mRNA ID	NM_001284416
Length	496
RefSeq Status	VALIDATED
MPDSNFAERSEEQVSGAKVIAQALKTQDVEYIFGIVGIPVTEIAIAAQQLGIKYIGMRNEQAACYAASAIGYLTSRPGVCLVVSGPGLIHALGGMANANMNCWYMERCMSPPISMAETSAVCTAASVIRNAKQPLLIIGKGAAYAHAEESIKKLVEQYKLPFLPTPMGKGVVPDNHPYCVGAARSRALQFADVIVLFGARLNWILHFGLPPRYQPDVKFIQVDICAEELGNNVKPAVTLLGNIHAVTKQLLEELDKTPWQYPPESKWWKTLREKMKSNEAASKELASKKSLPMNYYTVFYHVQEQLPRDCFVVSEGANTMDIGRTVLQNYLPRHRLDAGTFGTMGVGLGFAIAAAVVAKDRSPGQWIICVEGDSAFGFSGMEVETICRYNLPIILLVVNNNGIYQGFDTDTWKEMLKFQDATAVVPPMCLLPNSHYEQVMTAFGGKGYFVQTPEELQKSLRQSLADTTKPSLINIMIEPQATRKAQDFHWLTRSNM

Gene Information

Entrez Gene ID

26061

Gene Name

2-hydroxyacyl-CoA lyase 1

Gene Symbol

HACL1

Species

Homo sapiens

Gene Ontology (GO Annotations)

GO ID	Source	Type	Description
GO:0005782	TAS:Reactome	C	peroxisomal matrix
GO:0005777	IDA:UniProtKB	C	peroxisome
GO:0016830	IDA:UniProtKB	F	carbon-carbon lyase activity
GO:0048037	IDA:UniProtKB	F	cofactor binding
GO:0042802	IPI:IntAct	F	identical protein binding
GO:0000287	IEA:InterPro	F	magnesium ion binding
GO:0005102	IPI:UniProtKB	F	receptor binding
GO:0030976	IDA:UniProtKB	F	thiamine pyrophosphate binding
GO:0044255	TAS:Reactome	P	cellular lipid metabolic process
GO:0001561	IDA:HGNC	P	fatty acid alpha-oxidation
GO:0051259	IDA:UniProtKB	P	protein oligomerization
GO:0044281	TAS:Reactome	P	small molecule metabolic process

KEGG Pathway Links

KEGG Pathway ID	Description
hsa04146	Peroxisome

BIOCYC Pathway Links

BIOCYC Pathway ID	Description
PWY66-387	fatty acid alpha-oxidation II
PWY66-388	fatty acid alpha-oxidation III

REACTOME Pathway Links

REACTOME Pathway ID	Description
REACT_17003	Alpha-oxidation of phytanate

Domain Information

InterPro Annotations

Accession	Description
IPR029035	DHS-like NAD/FAD-binding domain
IPR029061	Thiamin diphosphate-binding fold
IPR011766	Thiamine pyrophosphate enzyme, C-terminal TPP-binding
IPR012001	Thiamine pyrophosphate enzyme, N-terminal TPP-binding domain
IPR012000	Thiamine pyrophosphate enzyme, central domain

UniProt Annotations

Entry Information

Gene Name

2-hydroxyacyl-CoA lyase 1

Protein Entry

HACL1_HUMAN

UniProt ID

Q9UJ83

Species

Human

Comments

Comment Type	Description
Alternative Products	Event=Alternative splicing; Named isoforms=4; Name=1; IsoId=Q9UJ83-1; Sequence=Displayed; Name=2; IsoId=Q9UJ83-2; Sequence=VSP_054191; Note=No experimental confirmation available.; Name=4; IsoId=Q9UJ83-4; Sequence=VSP_054749, VSP_054750; Name=3; IsoId=Q9UJ83-3; Sequence=VSP_054192; Note=No experimental confirmation available.;
Catalytic Activity	2-hydroxyphytanoyl-CoA = pristanal + formyl- CoA.
Cofactor	Name=Mg(2+); Xref=ChEBI
Cofactor	Name=thiamine diphosphate; Xref=ChEBI
Function	Catalyzes a carbon-carbon cleavage reaction; cleaves a 2-hydroxy-3-methylacyl-CoA into formyl-CoA and a 2-methyl-branched fatty aldehyde.
Interaction	Self; NbExp=2; IntAct=EBI-746580, EBI-746580;
Pathway	Lipid metabolism; fatty acid metabolism.
Sequence Caution	Sequence=AAF28957.1; Type=Erroneous initiation; Note=Translation N-terminally shortened.; Evidence= ;
Similarity	Belongs to the TPP enzyme family.
Subcellular Location	Peroxisome.
Subunit	Homotetramer.
Tissue Specificity	Expressed in high levels in liver, also expressed in kidney, heart and skeletal muscle.

Identical and Related Proteins

Unique RefSeq proteins for LMP002572 (as displayed in Record Overview)

Protein GI	Database	Accession	Length	Protein Name
93004078	RefSeq	NP_036392	578	2-hydroxyacyl-CoA lyase 1 isoform a
548923731	RefSeq	NP_001271342	551	2-hydroxyacyl-CoA lyase 1 isoform b
548923731	RefSeq	NP_001271344	551	2-hydroxyacyl-CoA lyase 1 isoform c
548923675	RefSeq	NP_001271345	496	2-hydroxyacyl-CoA lyase 1 isoform d

Identical Sequences to LMP002572 proteins

Reference	Database	Accession	Length	Protein Name
GI:548923731	DBBJ	BAG63043.1	551	unnamed protein product [Homo sapiens]
GI:548923731	DBBJ	BAG63043.1	551	unnamed protein product [Homo sapiens]
GI:548923675	DBBJ	BAG63397.1	496	unnamed protein product [Homo sapiens]
GI:93004078	GenBank	AAM58747.1	578	Sequence 2 from patent US 6383737
GI:93004078	GenBank	EAW64251.1	578	phytanoyl-CoA 2-hydroxylase 2, isoform CRA_a [Homo sapiens]
GI:93004078	GenBank	ABM86484.1	578	2-hydroxyacyl-CoA lyase 1, partial [synthetic construct]
GI:93004078	GenBank	ABW03818.1	578	2-hydroxyacyl-CoA lyase 1 [synthetic construct]
GI:93004078	GenBank	AIC51100.1	578	HACL1, partial [synthetic construct]
GI:93004078	RefSeq	XP_003826249.1	578	PREDICTED: 2-hydroxyacyl-CoA lyase 1 [Pan paniscus]

Related Sequences to LMP002572 proteins

Reference	Database	Accession	Length	Protein Name
GI:93004078	GenBank	AAF28957.1	581	HSPC279, partial [Homo sapiens]
GI:548923731	GenBank	AAF28957.1	581	HSPC279, partial [Homo sapiens]
GI:548923731	GenBank	AAF28957.1	581	HSPC279, partial [Homo sapiens]
GI:548923731	GenBank	AAH01627.1	578	2-hydroxyacyl-CoA lyase 1 [Homo sapiens]
GI:548923731	GenBank	AAH01627.1	578	2-hydroxyacyl-CoA lyase 1 [Homo sapiens]
GI:548923731	GenBank	AAE62799.1	578	Sequence 2 from patent US 6200796
GI:548923731	GenBank	AAE62799.1	578	Sequence 2 from patent US 6200796
GI:548923731	GenBank	ABM86484.1	578	2-hydroxyacyl-CoA lyase 1, partial [synthetic construct]
GI:548923731	GenBank	ABM86484.1	578	2-hydroxyacyl-CoA lyase 1, partial [synthetic construct]
GI:548923731	GenBank	ABW03818.1	578	2-hydroxyacyl-CoA lyase 1 [synthetic construct]
GI:548923731	GenBank	ABW03818.1	578	2-hydroxyacyl-CoA lyase 1 [synthetic construct]
GI:93004078	GenBank	JAA11537.1	578	2-hydroxyacyl-CoA lyase 1 [Pan troglodytes]
GI:93004078	GenBank	JAA22922.1	578	2-hydroxyacyl-CoA lyase 1 [Pan troglodytes]
GI:93004078	GenBank	JAA35180.1	578	2-hydroxyacyl-CoA lyase 1 [Pan troglodytes]
GI:548923675	RefSeq	XP_003781766.1	499	PREDICTED: 2-hydroxyacyl-CoA lyase 1 isoform 2 [Otolemur garnettii]
GI:93004078	RefSeq	XP_004033733.1	578	PREDICTED: 2-hydroxyacyl-CoA lyase 1 isoform 1 [Gorilla gorilla gorilla]
GI:548923731	RefSeq	XP_004033734.1	551	PREDICTED: 2-hydroxyacyl-CoA lyase 1 isoform 2 [Gorilla gorilla gorilla]
GI:548923731	RefSeq	XP_004033734.1	551	PREDICTED: 2-hydroxyacyl-CoA lyase 1 isoform 2 [Gorilla gorilla gorilla]
GI:548923675	RefSeq	XP_004033735.1	496	PREDICTED: 2-hydroxyacyl-CoA lyase 1 isoform 3 [Gorilla gorilla gorilla]
GI:548923675	RefSeq	XP_004271988.1	499	PREDICTED: 2-hydroxyacyl-CoA lyase 1 isoform 5 [Orcinus orca]
GI:93004078	RefSeq	NP_001267049.1	578	2-hydroxyacyl-CoA lyase 1 [Pan troglodytes]
GI:548923675	RefSeq	XP_007470696.1	499	PREDICTED: 2-hydroxyacyl-CoA lyase 1 isoform X5 [Lipotes vexillifer]
GI:548923675	RefSeq	XP_007934928.1	499	PREDICTED: 2-hydroxyacyl-CoA lyase 1 isoform X4 [Orycteropus afer afer]
GI:548923675	RefSeq	XP_008151556.1	496	PREDICTED: 2-hydroxyacyl-CoA lyase 1 isoform X2 [Eptesicus fuscus]