HACL1

Functionⁱ

Catalyzes a carbon-carbon cleavage reaction; cleaves a 2-hydroxy-3-methylacyl-CoA into formyl-CoA and a 2-methyl-branched fatty aldehyde.

Catalytic activityⁱ

2-hydroxyphytanoyl-CoA = pristanal + formyl-CoA.

Cofactorⁱ

Protein has several cofactor binding sites:

Mg²⁺Note: Binds 1 Mg²⁺ ion per subunit.
thiamine diphosphateNote: Binds 1 thiamine pyrophosphate per subunit.

Pathwayⁱ: fatty acid metabolism

This protein is involved in the pathway fatty acid metabolism, which is part of Lipid metabolism.
View all proteins of this organism that are known to be involved in the pathway fatty acid metabolism and in Lipid metabolism.

Sites

Feature key	Position(s)	DescriptionActions	Length
Binding siteⁱ	60	Thiamine pyrophosphateBy similarity	1
Metal bindingⁱ	455	MagnesiumBy similarity	1
Metal bindingⁱ	482	MagnesiumBy similarity	1

GO - Molecular functionⁱ

carbon-carbon lyase activity
Source: UniProtKB
Inferred from direct assayⁱ
- 15644336
- 21708296
cofactor binding
Source: UniProtKB
Inferred from direct assayⁱ
- 15644336
identical protein binding
Source: IntAct
Inferred from physical interactionⁱ
- 16189514
- 25416956
magnesium ion binding Source: InterPro
signaling receptor binding
Source: UniProtKB
Inferred from physical interactionⁱ
- 20178365
thiamine pyrophosphate binding
Source: UniProtKB
Inferred from direct assayⁱ
- 21708296

View the complete GO annotation on QuickGO ...

GO - Biological processⁱ

fatty acid alpha-oxidation
Source: HGNC
Inferred from direct assayⁱ
- 10468558
protein complex oligomerization
Source: UniProtKB
Inferred from direct assayⁱ
- 21708296
protein targeting to peroxisome Source: Reactome

View the complete GO annotation on QuickGO ...

Keywordsⁱ

Molecular function	Lyase
Ligand	Magnesium, Metal-binding, Thiamine pyrophosphate

Enzyme and pathway databases

BioCycⁱ	MetaCyc:HS05516-MONOMER
Reactomeⁱ	R-HSA-389599 Alpha-oxidation of phytanate R-HSA-9033241 Peroxisomal protein import
UniPathwayⁱ	UPA00199

Chemistry databases

SwissLipidsⁱ	SLP:000001015

SwissLipidsⁱ

SLP:000001015

Names & Taxonomyⁱ

Protein namesⁱ	Recommended name: 2-hydroxyacyl-CoA lyase 1 (EC:4.1.-.-) Alternative name(s): 2-hydroxyphytanoyl-CoA lyase Short name: 2-HPCL Phytanoyl-CoA 2-hydroxylase 2
Gene namesⁱ	Name:HACL1 Synonyms:HPCL, HPCL2, PHYH2 ORF Names:HSPC279
Organismⁱ	Homo sapiens (Human)
Taxonomic identifierⁱ	9606 [NCBI]
Taxonomic lineageⁱ	cellular organisms › Eukaryota › Opisthokonta › Metazoa › Eumetazoa › Bilateria › Deuterostomia › Chordata › Craniata › Vertebrata › Gnathostomata › Teleostomi › Euteleostomi › Sarcopterygii › Dipnotetrapodomorpha › Tetrapoda › Amniota › Mammalia › Theria › Eutheria › Boreoeutheria › Euarchontoglires › Primates › Haplorrhini › Simiiformes › Catarrhini › Hominoidea › Hominidae › Homininae › Homo
Proteomesⁱ	UP000005640 Componentⁱ: Chromosome 3

Organism-specific databases

EuPathDBⁱ	HostDB:ENSG00000131373.14
Human Gene Nomenclature Database More...HGNCⁱ	HGNC:17856 HACL1
MIMⁱ	604300 gene
neXtProtⁱ	NX_Q9UJ83

Keywords - Cellular componentⁱ

Peroxisome

Pathology & Biotechⁱ

Organism-specific databases

DisGeNET More...DisGeNETⁱ	26061
Open Targets More...OpenTargetsⁱ	ENSG00000131373
PharmGKBⁱ	PA142671172

Polymorphism and mutation databases

BioMutaⁱ	HACL1
DMDMⁱ	20455027

PTM / Processingⁱ

Molecule processing

Feature key	Position(s)	DescriptionActions	Graphical view	Length
ChainⁱPRO_0000090816	1 – 578	2-hydroxyacyl-CoA lyase 1Add BLAST		578

Amino acid modifications

Feature key	Position(s)	DescriptionActions	Length
Modified residueⁱ	4	PhosphoserineCombined sources	1
Modified residueⁱ	351	N6-succinyllysineBy similarity	1
Modified residueⁱ	358	N6-succinyllysineBy similarity	1
Modified residueⁱ	365	N6-succinyllysineBy similarity	1

Keywords - PTMⁱ

Phosphoprotein

Proteomic databases

EPDⁱ	Q9UJ83
PaxDbⁱ	Q9UJ83
PeptideAtlas More...PeptideAtlasⁱ	Q9UJ83
PRIDEⁱ	Q9UJ83
ProteomicsDBⁱ	84601

PTM databases

iPTMnetⁱ	Q9UJ83
PhosphoSitePlusⁱ	Q9UJ83

Expressionⁱ

Tissue specificityⁱ

Expressed in high levels in liver, also expressed in kidney, heart and skeletal muscle.1 Publication

Gene expression databases

Bgeeⁱ	ENSG00000131373 Expressed in 190 organ(s), highest expression level in duodenum
CleanExⁱ	HS_HACL1
ExpressionAtlasⁱ	Q9UJ83 baseline and differential
Genevisibleⁱ	Q9UJ83 HS

Organism-specific databases

Human Protein Atlas More...HPAⁱ	HPA035496 HPA035497

HPAⁱ

HPA035496
HPA035497

Interactionⁱ

Subunit structureⁱ

Homotetramer.

Binary interactionsⁱ

With	Entry	#Exp.	IntAct
itself		3	EBI-746580,EBI-746580
MAGEB6	Q8N7X4	3	EBI-746580,EBI-6447163
ZMYND19	Q96E35	6	EBI-746580,EBI-746595

GO - Molecular functionⁱ

View the complete GO annotation on QuickGO ...

Protein-protein interaction databases

BioGridⁱ	117523, 13 interactors
IntActⁱ	Q9UJ83, 5 interactors
Molecular INTeraction database More...MINTⁱ	Q9UJ83
STRINGⁱ	9606.ENSP00000323811

Structureⁱ

3D structure databases

ProteinModelPortalⁱ	Q9UJ83
SMRⁱ	Q9UJ83
ModBaseⁱ	Search...
MobiDBⁱ	Search...

Family & Domainsⁱ

Region

Feature key	Position(s)	DescriptionActions	Graphical view	Length
Regionⁱ	401 – 486	Thiamine pyrophosphate bindingAdd BLAST		86

Motif

Feature key	Position(s)	DescriptionActions	Graphical view	Length
Motifⁱ	576 – 578	Microbody targeting signal		3

Sequence similaritiesⁱ

Belongs to the TPP enzyme family.Curated

Phylogenomic databases

eggNOGⁱ	KOG1185 Eukaryota COG0028 LUCA
Ensembl GeneTree More...GeneTreeⁱ	ENSGT00530000063412
HOGENOMⁱ	HOG000053808
HOVERGENⁱ	HBG027302
InParanoidⁱ	Q9UJ83
KEGG Orthology (KO) More...KOⁱ	K12261
OMAⁱ	QGMVRQW
Database of Orthologous Groups More...OrthoDBⁱ	EOG091G03OS
PhylomeDBⁱ	Q9UJ83
TreeFamⁱ	TF105690

Family and domain databases

InterProⁱ	View protein in InterPro IPR029035 DHS-like_NAD/FAD-binding_dom IPR029061 THDP-binding IPR012000 Thiamin_PyroP_enz_cen_dom IPR012001 Thiamin_PyroP_enz_TPP-bd_dom IPR011766 TPP_enzyme-bd_C
Pfam protein domain database More...Pfamⁱ	View protein in Pfam PF02775 TPP_enzyme_C, 1 hit PF00205 TPP_enzyme_M, 1 hit PF02776 TPP_enzyme_N, 1 hit
SUPFAMⁱ	SSF52467 SSF52467, 1 hit SSF52518 SSF52518, 2 hits

Sequences (4+)ⁱ

Sequence statusⁱ: Complete.

This entry describes 4 isoformsⁱ produced by alternative splicing. Align Add to basket

This entry has 4 described isoforms and 5 potential isoforms that are computationally mapped.Show all Align All

Isoform 1 (identifier: Q9UJ83-1) [UniParc]FASTA Add to basket

This isoform has been chosen as the 'canonical' sequence. All positional information in this entry refers to it. This is also the sequence that appears in the downloadable versions of the entry.

« Hide

        10         20         30         40         50
MPDSNFAERS EEQVSGAKVI AQALKTQDVE YIFGIVGIPV TEIAIAAQQL 
        60         70         80         90        100
GIKYIGMRNE QAACYAASAI GYLTSRPGVC LVVSGPGLIH ALGGMANANM 
       110        120        130        140        150
NCWPLLVIGG SSERNQETMG AFQEFPQVEA CRLYTKFSAR PSSIEAIPFV 
       160        170        180        190        200
IEKAVRSSIY GRPGACYVDI PADFVNLQVN VNSIKYMERC MSPPISMAET 
       210        220        230        240        250
SAVCTAASVI RNAKQPLLII GKGAAYAHAE ESIKKLVEQY KLPFLPTPMG 
       260        270        280        290        300
KGVVPDNHPY CVGAARSRAL QFADVIVLFG ARLNWILHFG LPPRYQPDVK 
       310        320        330        340        350
FIQVDICAEE LGNNVKPAVT LLGNIHAVTK QLLEELDKTP WQYPPESKWW 
       360        370        380        390        400
KTLREKMKSN EAASKELASK KSLPMNYYTV FYHVQEQLPR DCFVVSEGAN 
       410        420        430        440        450
TMDIGRTVLQ NYLPRHRLDA GTFGTMGVGL GFAIAAAVVA KDRSPGQWII 
       460        470        480        490        500
CVEGDSAFGF SGMEVETICR YNLPIILLVV NNNGIYQGFD TDTWKEMLKF 
       510        520        530        540        550
QDATAVVPPM CLLPNSHYEQ VMTAFGGKGY FVQTPEELQK SLRQSLADTT 
       560        570 
KPSLINIMIE PQATRKAQDF HWLTRSNM

Length:578

Mass (Da):63,729

Last modified:May 2, 2002 - v2

Checksum:ⁱCF4F4B1A97025140

GO

Isoform 2 (identifier: Q9UJ83-2) [UniParc]FASTA Add to basket

The sequence of this isoform differs from the canonical sequence as follows:
77-103: Missing.

« Hide

        10         20         30         40         50
MPDSNFAERS EEQVSGAKVI AQALKTQDVE YIFGIVGIPV TEIAIAAQQL 
        60         70         80         90        100
GIKYIGMRNE QAACYAASAI GYLTSRPLLV IGGSSERNQE TMGAFQEFPQ 
       110        120        130        140        150
VEACRLYTKF SARPSSIEAI PFVIEKAVRS SIYGRPGACY VDIPADFVNL 
       160        170        180        190        200
QVNVNSIKYM ERCMSPPISM AETSAVCTAA SVIRNAKQPL LIIGKGAAYA 
       210        220        230        240        250
HAEESIKKLV EQYKLPFLPT PMGKGVVPDN HPYCVGAARS RALQFADVIV 
       260        270        280        290        300
LFGARLNWIL HFGLPPRYQP DVKFIQVDIC AEELGNNVKP AVTLLGNIHA 
       310        320        330        340        350
VTKQLLEELD KTPWQYPPES KWWKTLREKM KSNEAASKEL ASKKSLPMNY 
       360        370        380        390        400
YTVFYHVQEQ LPRDCFVVSE GANTMDIGRT VLQNYLPRHR LDAGTFGTMG 
       410        420        430        440        450
VGLGFAIAAA VVAKDRSPGQ WIICVEGDSA FGFSGMEVET ICRYNLPIIL 
       460        470        480        490        500
LVVNNNGIYQ GFDTDTWKEM LKFQDATAVV PPMCLLPNSH YEQVMTAFGG 
       510        520        530        540        550
KGYFVQTPEE LQKSLRQSLA DTTKPSLINI MIEPQATRKA QDFHWLTRSN 

M

Note: No experimental confirmation available.

Show »

Length:551

Mass (Da):61,064

Checksum:ⁱE4CCA8D486BE14FB

GO

Isoform 4 (identifier: Q9UJ83-4) [UniParc]FASTA Add to basket

The sequence of this isoform differs from the canonical sequence as follows:
128-153: Missing.
332-365: Missing.

« Hide

        10         20         30         40         50
MPDSNFAERS EEQVSGAKVI AQALKTQDVE YIFGIVGIPV TEIAIAAQQL 
        60         70         80         90        100
GIKYIGMRNE QAACYAASAI GYLTSRPGVC LVVSGPGLIH ALGGMANANM 
       110        120        130        140        150
NCWPLLVIGG SSERNQETMG AFQEFPQAVR SSIYGRPGAC YVDIPADFVN 
       160        170        180        190        200
LQVNVNSIKY MERCMSPPIS MAETSAVCTA ASVIRNAKQP LLIIGKGAAY 
       210        220        230        240        250
AHAEESIKKL VEQYKLPFLP TPMGKGVVPD NHPYCVGAAR SRALQFADVI 
       260        270        280        290        300
VLFGARLNWI LHFGLPPRYQ PDVKFIQVDI CAEELGNNVK PAVTLLGNIH 
       310        320        330        340        350
AVTKQELASK KSLPMNYYTV FYHVQEQLPR DCFVVSEGAN TMDIGRTVLQ 
       360        370        380        390        400
NYLPRHRLDA GTFGTMGVGL GFAIAAAVVA KDRSPGQWII CVEGDSAFGF 
       410        420        430        440        450
SGMEVETICR YNLPIILLVV NNNGIYQGFD TDTWKEMLKF QDATAVVPPM 
       460        470        480        490        500
CLLPNSHYEQ VMTAFGGKGY FVQTPEELQK SLRQSLADTT KPSLINIMIE 
       510 
PQATRKAQDF HWLTRSNM

Show »

Length:518

Mass (Da):56,660

Checksum:ⁱC8ED2E77A6246C04

GO

Isoform 3 (identifier: Q9UJ83-3) [UniParc]FASTA Add to basket

The sequence of this isoform differs from the canonical sequence as follows:
104-185: Missing.

« Hide

        10         20         30         40         50
MPDSNFAERS EEQVSGAKVI AQALKTQDVE YIFGIVGIPV TEIAIAAQQL 
        60         70         80         90        100
GIKYIGMRNE QAACYAASAI GYLTSRPGVC LVVSGPGLIH ALGGMANANM 
       110        120        130        140        150
NCWYMERCMS PPISMAETSA VCTAASVIRN AKQPLLIIGK GAAYAHAEES 
       160        170        180        190        200
IKKLVEQYKL PFLPTPMGKG VVPDNHPYCV GAARSRALQF ADVIVLFGAR 
       210        220        230        240        250
LNWILHFGLP PRYQPDVKFI QVDICAEELG NNVKPAVTLL GNIHAVTKQL 
       260        270        280        290        300
LEELDKTPWQ YPPESKWWKT LREKMKSNEA ASKELASKKS LPMNYYTVFY 
       310        320        330        340        350
HVQEQLPRDC FVVSEGANTM DIGRTVLQNY LPRHRLDAGT FGTMGVGLGF 
       360        370        380        390        400
AIAAAVVAKD RSPGQWIICV EGDSAFGFSG MEVETICRYN LPIILLVVNN 
       410        420        430        440        450
NGIYQGFDTD TWKEMLKFQD ATAVVPPMCL LPNSHYEQVM TAFGGKGYFV 
       460        470        480        490 
QTPEELQKSL RQSLADTTKP SLINIMIEPQ ATRKAQDFHW LTRSNM

Note: No experimental confirmation available.

Show »

Length:496

Mass (Da):54,724

Checksum:ⁱBACFA0F55B8007A1

GO

Computationally mapped potential isoform sequencesⁱ

There are 5 potential isoforms mapped to this entry.BLAST Align Show all Add to basket

Entry	Entry name	Protein names	Gene names	Length	Annotation

B3KPX4	B3KPX4_HUMAN	2-hydroxyacyl-CoA lyase 1 2-hydroxyacyl-CoA lyase 1 (Phytanoyl-CoA 2-hydroxylase 2, isoform CRA_c) (cDNA FLJ32415 fis, clone SKMUS2000724, highly similar to 2-hydroxyphytanoyl-CoA lyase)	HACL1 PHYH2, hCG_1811148	337	Annotation score:
C9J306	C9J306_HUMAN	2-hydroxyacyl-CoA lyase 1 2-hydroxyacyl-CoA lyase 1	HACL1	244	Annotation score:
C9JJM7	C9JJM7_HUMAN	2-hydroxyacyl-CoA lyase 1 2-hydroxyacyl-CoA lyase 1	HACL1	157	Annotation score:
F8WCH9	F8WCH9_HUMAN	2-hydroxyacyl-CoA lyase 1 2-hydroxyacyl-CoA lyase 1	HACL1	137	Annotation score:
Q7Z773	Q7Z773_HUMAN	2-hydroxyacyl-CoA lyase 1 2-hydroxyacyl-CoA lyase 1 (HACL1 protein)	HACL1	119	Annotation score:

Sequence cautionⁱ

The sequence AAF28957 differs from that shown. Reason: Erroneous initiation. Translation N-terminally shortened.Curated

Experimental Info

Feature key	Position(s)	DescriptionActions	Graphical view	Length
Sequence conflictⁱ	447	Q → H in CAB60200 (PubMed:10468558).Curated		1
Sequence conflictⁱ	543	R → E in CAB60200 (PubMed:10468558).Curated		1

Alternative sequence

Feature key	Position(s)	DescriptionActions	Length
Alternative sequenceⁱVSP_054191	77 – 103	Missing in isoform 2. 1 PublicationAdd BLAST	27
Alternative sequenceⁱVSP_054192	104 – 185	Missing in isoform 3. 1 PublicationAdd BLAST	82
Alternative sequenceⁱVSP_054749	128 – 153	Missing in isoform 4. CuratedAdd BLAST	26
Alternative sequenceⁱVSP_054750	332 – 365	Missing in isoform 4. CuratedAdd BLAST	34

Sequence databases

Select the link destinations: EMBLⁱ GenBankⁱ DDBJⁱ Links Updated	AJ131753 mRNA Translation: CAB60200.1 AF161397 mRNA Translation: AAF28957.1 Different initiation. AK301546 mRNA Translation: BAG63043.1 AK301990 mRNA Translation: BAG63397.1 AC027129 Genomic DNA No translation available. BC001627 mRNA Translation: AAH01627.1
The Consensus CDS (CCDS) project More...CCDSⁱ	CCDS2627.1 [Q9UJ83-1] CCDS68360.1 [Q9UJ83-2] CCDS68361.1 [Q9UJ83-3] CCDS68362.1 [Q9UJ83-4]
NCBI Reference Sequences More...RefSeqⁱ	NP_001271342.1, NM_001284413.1 [Q9UJ83-2] NP_001271344.1, NM_001284415.1 [Q9UJ83-4] NP_001271345.1, NM_001284416.1 [Q9UJ83-3] NP_036392.2, NM_012260.3 [Q9UJ83-1]
UniGeneⁱ	Hs.63290

Genome annotation databases

Ensemblⁱ	ENST00000321169; ENSP00000323811; ENSG00000131373 [Q9UJ83-1] ENST00000451445; ENSP00000403656; ENSG00000131373 [Q9UJ83-3] ENST00000456194; ENSP00000390699; ENSG00000131373 [Q9UJ83-2] ENST00000457447; ENSP00000404883; ENSG00000131373 [Q9UJ83-4]
GeneIDⁱ	26061
KEGGⁱ	hsa:26061
UCSC genome browser More...UCSCⁱ	uc003caf.5 human [Q9UJ83-1]

Keywords - Coding sequence diversityⁱ

Alternative splicing

Similar proteinsⁱ

100% Identity
90% Identity
50% Identity

Protein	Similar proteins	Species	Length	Source
Q9UJ83	Uncharacterized protein	PANPA	578	UniRef100_Q9UJ83
Q9UJ83-2	Uncharacterized protein	PANPA	551	UniRef100_A0A2R9CPF1
Q9UJ83-4	Uncharacterized protein	PANPA	518	UniRef100_A0A2R9CEJ4
Q9UJ83-3	Uncharacterized protein	PANPA	496	UniRef100_A0A2R9CED8

Protein	Similar proteins		Species	Score	Length	Source
Q9UJ83	Uncharacterized protein		PANPA		578	UniRef90_Q9UJ83
2-hydroxyacyl-CoA lyase 1		GORGO		578
HACL1 isoform 2		PONAB		578
Uncharacterized protein		NOMLE		578
Uncharacterized protein		COLAP		578
+13
Q9UJ83-2	2-hydroxyacyl-CoA lyase 1		OTOGA		554	UniRef90_H0XA77
2-hydroxyacyl-CoA lyase 1 isoform X3		LIPVE		554
2-hydroxyacyl-CoA lyase 1		PANTR		551
2-hydroxyacyl-CoA lyase 1		SAIBB		551
Uncharacterized protein		MACMU		551
+18
Q9UJ83-4	2-hydroxyacyl-CoA lyase 1		PANTR		518	UniRef90_A0A2I3SUY8
2-hydroxyacyl-CoA lyase 1 isoform X4		LIPVE		521
cDNA FLJ58815, highly similar to 2-hydroxyphytanoyl-CoA lyase		HUMAN		518
2-hydroxyacyl-CoA lyase 1		SAIBB		518
HACL1 isoform 7		PONAB		518
+12
Q9UJ83-3	Uncharacterized protein		ICTTR		499	UniRef90_A0A287D8S0
2-hydroxyacyl-CoA lyase 1 isoform X5		LIPVE		499
2-hydroxyacyl-CoA lyase 1		PANTR		496
HACL1 isoform 4		PONAB		496
Uncharacterized protein		CERAT		496
+10

Protein	Similar proteins		Species	Score	Length	Source
Q9UJ83	Uncharacterized protein		PANPA		578	UniRef50_Q9UJ83
Uncharacterized protein		NOMLE		578
Uncharacterized protein		COLAP		578
HACL1 isoform 2		PONAB		578
2-hydroxyacyl-CoA lyase 1		GORGO		578
+100
Q9UJ83-2	2-hydroxyacyl-CoA lyase 1		MELGA		569	UniRef50_G1NEC4
Uncharacterized protein		XENLA		577
2-hydroxyacyl-CoA lyase 1		XENTR		590
2-hydroxyacyl-CoA lyase 1		FUNHE		594
2-hydroxyacyl-CoA lyase 1		Aurantiochytrium sp. FCC1311		581
+214
Q9UJ83-4	Uncharacterized protein		ICTTR		499	UniRef50_A0A287D8S0
2-hydroxyacyl-CoA lyase 1 isoform X5		LIPVE		499
2-hydroxyacyl-CoA lyase 1		PANTR		496
2-hydroxyacyl-CoA lyase 1		GORGO		496
HACL1 isoform 4		PONAB		496
+92
Q9UJ83-3	Uncharacterized protein		ICTTR		499	UniRef50_A0A287D8S0
2-hydroxyacyl-CoA lyase 1 isoform X5		LIPVE		499
2-hydroxyacyl-CoA lyase 1		PANTR		496
2-hydroxyacyl-CoA lyase 1		GORGO		496
HACL1 isoform 4		PONAB		496
+92

Cross-referencesⁱ

Sequence databases

Select the link destinations: EMBLⁱ GenBankⁱ DDBJⁱ Links Updated	AJ131753 mRNA Translation: CAB60200.1 AF161397 mRNA Translation: AAF28957.1 Different initiation. AK301546 mRNA Translation: BAG63043.1 AK301990 mRNA Translation: BAG63397.1 AC027129 Genomic DNA No translation available. BC001627 mRNA Translation: AAH01627.1
CCDSⁱ	CCDS2627.1 [Q9UJ83-1] CCDS68360.1 [Q9UJ83-2] CCDS68361.1 [Q9UJ83-3] CCDS68362.1 [Q9UJ83-4]
RefSeqⁱ	NP_001271342.1, NM_001284413.1 [Q9UJ83-2] NP_001271344.1, NM_001284415.1 [Q9UJ83-4] NP_001271345.1, NM_001284416.1 [Q9UJ83-3] NP_036392.2, NM_012260.3 [Q9UJ83-1]
UniGeneⁱ	Hs.63290

3D structure databases

ProteinModelPortalⁱ	Q9UJ83
SMRⁱ	Q9UJ83
ModBaseⁱ	Search...
MobiDBⁱ	Search...

Protein-protein interaction databases

BioGridⁱ	117523, 13 interactors
IntActⁱ	Q9UJ83, 5 interactors
MINTⁱ	Q9UJ83
STRINGⁱ	9606.ENSP00000323811

Chemistry databases

SwissLipidsⁱ	SLP:000001015

SwissLipidsⁱ

SLP:000001015

PTM databases

iPTMnetⁱ	Q9UJ83
PhosphoSitePlusⁱ	Q9UJ83

Polymorphism and mutation databases

BioMutaⁱ	HACL1
DMDMⁱ	20455027

Proteomic databases

EPDⁱ	Q9UJ83
PaxDbⁱ	Q9UJ83
PeptideAtlasⁱ	Q9UJ83
PRIDEⁱ	Q9UJ83
ProteomicsDBⁱ	84601

Protocols and materials databases

The DNASU plasmid repository More...DNASUⁱ	26061
Structural Biology Knowledgebase	Search...

Genome annotation databases

Ensemblⁱ	ENST00000321169; ENSP00000323811; ENSG00000131373 [Q9UJ83-1] ENST00000451445; ENSP00000403656; ENSG00000131373 [Q9UJ83-3] ENST00000456194; ENSP00000390699; ENSG00000131373 [Q9UJ83-2] ENST00000457447; ENSP00000404883; ENSG00000131373 [Q9UJ83-4]
GeneIDⁱ	26061
KEGGⁱ	hsa:26061
UCSCⁱ	uc003caf.5 human [Q9UJ83-1]

Organism-specific databases

CTDⁱ	26061
DisGeNETⁱ	26061
EuPathDBⁱ	HostDB:ENSG00000131373.14
GeneCardsⁱ	HACL1
HGNCⁱ	HGNC:17856 HACL1
HPAⁱ	HPA035496 HPA035497
MIMⁱ	604300 gene
neXtProtⁱ	NX_Q9UJ83
OpenTargetsⁱ	ENSG00000131373
PharmGKBⁱ	PA142671172
GenAtlas: human gene database More...GenAtlasⁱ	Search...

Phylogenomic databases

eggNOGⁱ	KOG1185 Eukaryota COG0028 LUCA
GeneTreeⁱ	ENSGT00530000063412
HOGENOMⁱ	HOG000053808
HOVERGENⁱ	HBG027302
InParanoidⁱ	Q9UJ83
KOⁱ	K12261
OMAⁱ	QGMVRQW
OrthoDBⁱ	EOG091G03OS
PhylomeDBⁱ	Q9UJ83
TreeFamⁱ	TF105690

Enzyme and pathway databases

UniPathwayⁱ	UPA00199
BioCycⁱ	MetaCyc:HS05516-MONOMER
Reactomeⁱ	R-HSA-389599 Alpha-oxidation of phytanate R-HSA-9033241 Peroxisomal protein import

Miscellaneous databases

ChiTaRSⁱ	HACL1 human
GenomeRNAiⁱ	26061
Protein Ontology More...PROⁱ	PR:Q9UJ83
SOURCEⁱ	Search...

Gene expression databases

Bgeeⁱ	ENSG00000131373 Expressed in 190 organ(s), highest expression level in duodenum
CleanExⁱ	HS_HACL1
ExpressionAtlasⁱ	Q9UJ83 baseline and differential
Genevisibleⁱ	Q9UJ83 HS

Family and domain databases

InterProⁱ	View protein in InterPro IPR029035 DHS-like_NAD/FAD-binding_dom IPR029061 THDP-binding IPR012000 Thiamin_PyroP_enz_cen_dom IPR012001 Thiamin_PyroP_enz_TPP-bd_dom IPR011766 TPP_enzyme-bd_C
Pfamⁱ	View protein in Pfam PF02775 TPP_enzyme_C, 1 hit PF00205 TPP_enzyme_M, 1 hit PF02776 TPP_enzyme_N, 1 hit
SUPFAMⁱ	SSF52467 SSF52467, 1 hit SSF52518 SSF52518, 2 hits
ProtoNetⁱ	Search...

Entry informationⁱ

Entry nameⁱ	HACL1_HUMAN
Accessionⁱ	Q9UJ83Primary (citable) accession number: Q9UJ83 Secondary accession number(s): B4DWI1 , B4DXI5, E9PEN4, Q9BV42, Q9P0A2
Entry historyⁱ	Integrated into UniProtKB/Swiss-Prot:	May 2, 2002
	Last sequence update:	May 2, 2002
	Last modified:	September 12, 2018
	This is version 157 of the entry and version 2 of the sequence. See complete history.
Entry statusⁱ	Reviewed (UniProtKB/Swiss-Prot)
Annotation program	Chordata Protein Annotation Program
Disclaimer	Any medical or genetic information present in this entry is provided for research, educational and informational purposes only. It is not in any way intended to be used as a substitute for professional medical advice, diagnosis, treatment or care.

Miscellaneousⁱ

Keywords - Technical termⁱ

Complete proteome, Reference proteome

Documents

Human chromosome 3
Human chromosome 3: entries, gene names and cross-references to MIM
SIMILARITY comments
Index of protein domains and families
PATHWAY comments
Index of metabolic and biosynthesis pathways
MIM cross-references
Online Mendelian Inheritance in Man (MIM) cross-references in UniProtKB/Swiss-Prot

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UniProtKB - Q9UJ83 (HACL1_HUMAN)

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2-hydroxyacyl-CoA lyase 1

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<p>This section provides any useful information about the protein, mostly biological knowledge.<p><a href='/help/function_section' target='_top'>More...</a></p>Functioni

<p>This subsection of the <a href="http://www.uniprot.org/help/function_section">'Function'</a> section describes the metabolic pathway(s) associated with a protein.<p><a href='/help/pathway' target='_top'>More...</a></p>Pathwayi: fatty acid metabolism

Sites

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GO - Biological processi

Enzyme and pathway databases

Chemistry databases

<p>This section provides information about the protein and gene name(s) and synonym(s) and about the organism that is the source of the protein sequence.<p><a href='/help/names_and_taxonomy_section' target='_top'>More...</a></p>Names & Taxonomyi

Organism-specific databases

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Keywords - Cellular componenti

<p>This section provides information on the disease(s) and phenotype(s) associated with a protein.<p><a href='/help/pathology_and_biotech_section' target='_top'>More...</a></p>Pathology & Biotechi

Organism-specific databases

Polymorphism and mutation databases

<p>This section describes post-translational modifications (PTMs) and/or processing events.<p><a href='/help/ptm_processing_section' target='_top'>More...</a></p>PTM / Processingi

Molecule processing

Amino acid modifications

Keywords - PTMi

Proteomic databases

PTM databases

<p>This section provides information on the expression of a gene at the mRNA or protein level in cells or in tissues of multicellular organisms.<p><a href='/help/expression_section' target='_top'>More...</a></p>Expressioni

Gene expression databases

Organism-specific databases

<p>This section provides information on the quaternary structure of a protein and on interaction(s) with other proteins or protein complexes.<p><a href='/help/interaction_section' target='_top'>More...</a></p>Interactioni

GO - Molecular functioni

Protein-protein interaction databases

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3D structure databases

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Phylogenomic databases

Family and domain databases

<p>This subsection of the ‘Sequence’ section reports difference(s) between the protein sequence shown in the UniProtKB entry and other available protein sequences derived from the same gene.<p><a href='/help/sequence_caution' target='_top'>More...</a></p>Sequence cautioni

Experimental Info

Alternative sequence

Sequence databases

Genome annotation databases

Keywords - Coding sequence diversityi

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Sequence databases

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Chemistry databases

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Polymorphism and mutation databases

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Genome annotation databases

Organism-specific databases

Phylogenomic databases

Enzyme and pathway databases

Miscellaneous databases

Gene expression databases

Family and domain databases

<p>This section provides general information on the entry.<p><a href='/help/entry_information_section' target='_top'>More...</a></p>Entry informationi

<p>This section contains any relevant information that doesn’t fit in any other defined sections<p><a href='/help/miscellaneous_section' target='_top'>More...</a></p>Miscellaneousi

Keywords - Technical termi

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Functionⁱ

Pathwayⁱ: fatty acid metabolism

GO - Molecular functionⁱ

GO - Biological processⁱ

Names & Taxonomyⁱ

Subcellular locationⁱ

Keywords - Cellular componentⁱ

Pathology & Biotechⁱ

PTM / Processingⁱ

Keywords - PTMⁱ

Expressionⁱ

Interactionⁱ

GO - Molecular functionⁱ

Structureⁱ

Family & Domainsⁱ

Sequence similaritiesⁱ

Sequence cautionⁱ

Keywords - Coding sequence diversityⁱ

Cross-referencesⁱ

Entry informationⁱ

Miscellaneousⁱ

Keywords - Technical termⁱ