UniProtKB - Q6Y1H2 (HACD2_HUMAN)
Protein
Very-long-chain (3R)-3-hydroxyacyl-CoA dehydratase 2
Gene
HACD2
Organism
Homo sapiens (Human)
Status
Functioni
Catalyzes the third of the very long-chain fatty acids (VLCFA) elongation four-step cycle (condensation, reduction, dehydration, and reduction). This endoplasmic reticulum-elongation process is characterized by the addition of two carbons to the lipid chain through each cycle. This enzyme catalyzes the dehydration of the 3-hydroxyacyl-CoA intermediate into trans-2,3-enoyl-CoA, within each cycle of elongation. Therefore, it participates in the production of various VLCFAs involved in multiple biological processes as precursors of membrane lipids and lipid mediators.1 Publication
Miscellaneous
Turns over rapidly through degradation by the proteasome system.1 Publication
Caution
Shares some similarity with tyrosine phosphatase proteins but it has probably no phosphatase activity.1 Publication
Catalytic activityi
- EC:4.2.1.1341 PublicationThis reaction proceeds in the forward1 Publication direction.
- This reaction proceeds in the forward1 Publication direction.
- This reaction proceeds in the forward1 Publication direction.
- This reaction proceeds in the forward1 Publication direction.
- This reaction proceeds in the forward1 Publication direction.
- This reaction proceeds in the forward1 Publication direction.
- This reaction proceeds in the forward1 Publication direction.
Kineticsi
- KM=121.7 µM for 3-hydroxypalmitoyl-CoA (at 37 degrees Celsius)1 Publication
: fatty acid biosynthesis Pathwayi
This protein is involved in the pathway fatty acid biosynthesis, which is part of Lipid metabolism.1 PublicationView all proteins of this organism that are known to be involved in the pathway fatty acid biosynthesis and in Lipid metabolism.
Sites
Feature key | Position(s) | DescriptionActions | Graphical view | Length |
---|---|---|---|---|
Active sitei | 176 | By similarity | 1 | |
Active sitei | 183 | By similarity | 1 |
GO - Molecular functioni
- 3-hydroxyacyl-CoA dehydratase activity Source: UniProtKB
- 3-hydroxy-arachidoyl-CoA dehydratase activity Source: UniProtKB-EC
- 3-hydroxy-behenoyl-CoA dehydratase activity Source: UniProtKB-EC
- 3-hydroxy-lignoceroyl-CoA dehydratase activity Source: UniProtKB-EC
- enzyme binding Source: UniProtKB
- very-long-chain 3-hydroxyacyl-CoA dehydratase activity Source: UniProtKB-EC
GO - Biological processi
- fatty acid elongation Source: UniProtKB
- long-chain fatty-acyl-CoA biosynthetic process Source: Reactome
- peptidyl-tyrosine dephosphorylation Source: GOC
- sphingolipid biosynthetic process Source: UniProtKB
- very long-chain fatty acid biosynthetic process Source: UniProtKB
Keywordsi
Molecular function | Lyase |
Biological process | Fatty acid biosynthesis, Fatty acid metabolism, Lipid biosynthesis, Lipid metabolism |
Enzyme and pathway databases
PathwayCommonsi | Q6Y1H2 |
Reactomei | R-HSA-75876, Synthesis of very long-chain fatty acyl-CoAs |
UniPathwayi | UPA00094 |
Chemistry databases
SwissLipidsi | SLP:000000438 |
Names & Taxonomyi
Protein namesi | Recommended name: Very-long-chain (3R)-3-hydroxyacyl-CoA dehydratase 2Curated (EC:4.2.1.1341 Publication)Alternative name(s): 3-hydroxyacyl-CoA dehydratase 21 Publication Short name: HACD21 Publication Protein-tyrosine phosphatase-like member BImported |
Gene namesi | |
Organismi | Homo sapiens (Human) |
Taxonomic identifieri | 9606 [NCBI] |
Taxonomic lineagei | Eukaryota › Metazoa › Chordata › Craniata › Vertebrata › Euteleostomi › Mammalia › Eutheria › Euarchontoglires › Primates › Haplorrhini › Catarrhini › Hominidae › Homo |
Proteomesi |
|
Organism-specific databases
HGNCi | HGNC:9640, HACD2 |
MIMi | 615939, gene |
neXtProti | NX_Q6Y1H2 |
VEuPathDBi | HostDB:ENSG00000206527.9 |
Subcellular locationi
Endoplasmic reticulum
- Endoplasmic reticulum membrane 2 Publications; Multi-pass membrane protein 2 Publications
Endoplasmic reticulum
- endoplasmic reticulum Source: UniProtKB
- endoplasmic reticulum membrane Source: Reactome
- integral component of endoplasmic reticulum membrane Source: GO_Central
Topology
Feature key | Position(s) | DescriptionActions | Graphical view | Length |
---|---|---|---|---|
Topological domaini | 2 – 41 | CytoplasmicSequence analysisAdd BLAST | 40 | |
Transmembranei | 42 – 60 | HelicalSequence analysisAdd BLAST | 19 | |
Topological domaini | 61 – 79 | LumenalSequence analysisAdd BLAST | 19 | |
Transmembranei | 80 – 97 | HelicalSequence analysisAdd BLAST | 18 | |
Topological domaini | 98 – 107 | CytoplasmicSequence analysis | 10 | |
Transmembranei | 108 – 125 | HelicalSequence analysisAdd BLAST | 18 | |
Topological domaini | 126 – 130 | LumenalSequence analysis | 5 | |
Transmembranei | 131 – 146 | HelicalSequence analysisAdd BLAST | 16 | |
Topological domaini | 147 – 169 | CytoplasmicSequence analysisAdd BLAST | 23 | |
Transmembranei | 170 – 187 | HelicalSequence analysisAdd BLAST | 18 | |
Topological domaini | 188 – 217 | LumenalSequence analysisAdd BLAST | 30 | |
Transmembranei | 218 – 235 | HelicalSequence analysisAdd BLAST | 18 | |
Topological domaini | 236 – 254 | CytoplasmicSequence analysisAdd BLAST | 19 |
Keywords - Cellular componenti
Endoplasmic reticulum, MembranePathology & Biotechi
Mutagenesis
Feature key | Position(s) | DescriptionActions | Graphical view | Length |
---|---|---|---|---|
Mutagenesisi | 101 | P → R: Does not restore any protein tyrosine phosphatase activity. 1 Publication | 1 |
Organism-specific databases
DisGeNETi | 201562 |
OpenTargetsi | ENSG00000206527 |
PharmGKBi | PA33983 |
Miscellaneous databases
Pharosi | Q6Y1H2, Tbio |
Genetic variation databases
BioMutai | HACD2 |
DMDMi | 74738322 |
PTM / Processingi
Molecule processing
Feature key | Position(s) | DescriptionActions | Graphical view | Length |
---|---|---|---|---|
Initiator methioninei | RemovedCombined sources | |||
ChainiPRO_0000349319 | 2 – 254 | Very-long-chain (3R)-3-hydroxyacyl-CoA dehydratase 2Add BLAST | 253 |
Amino acid modifications
Feature key | Position(s) | DescriptionActions | Graphical view | Length |
---|---|---|---|---|
Modified residuei | 2 | N-acetylalanineCombined sources1 Publication | 1 | |
Glycosylationi | 209 | N-linked (GlcNAc...) asparagineSequence analysis | 1 |
Keywords - PTMi
Acetylation, GlycoproteinProteomic databases
EPDi | Q6Y1H2 |
jPOSTi | Q6Y1H2 |
MassIVEi | Q6Y1H2 |
MaxQBi | Q6Y1H2 |
PaxDbi | Q6Y1H2 |
PeptideAtlasi | Q6Y1H2 |
PRIDEi | Q6Y1H2 |
ProteomicsDBi | 67831 |
TopDownProteomicsi | Q6Y1H2 |
PTM databases
GlyGeni | Q6Y1H2, 1 site |
iPTMneti | Q6Y1H2 |
PhosphoSitePlusi | Q6Y1H2 |
SwissPalmi | Q6Y1H2 |
Expressioni
Tissue specificityi
Highly expressed in testis, spleen, prostate, colon and heart, followed by moderate expression in thymus, ovary, small intestine, peripheral blood leukocytes, liver, skeletal muscle and pancreas. Weakly detected in kidney, placenta, brain and lung.1 Publication
Gene expression databases
Bgeei | ENSG00000206527, Expressed in adipose tissue and 250 other tissues |
ExpressionAtlasi | Q6Y1H2, baseline and differential |
Genevisiblei | Q6Y1H2, HS |
Organism-specific databases
HPAi | ENSG00000206527, Low tissue specificity |
Interactioni
Subunit structurei
May interact with enzymes of the ELO family (including ELOVL1); with those enzymes that mediate condensation, the first of the four steps of the reaction cycle responsible for fatty acids elongation, may be part of a larger fatty acids elongase complex (PubMed:18554506).
Interacts with BCAP31 (PubMed:15024066).
2 PublicationsBinary interactionsi
Hide detailsQ6Y1H2
GO - Molecular functioni
- enzyme binding Source: UniProtKB
Protein-protein interaction databases
BioGRIDi | 128393, 52 interactors |
IntActi | Q6Y1H2, 35 interactors |
STRINGi | 9606.ENSP00000373153 |
Miscellaneous databases
RNActi | Q6Y1H2, protein |
Family & Domainsi
Compositional bias
Feature key | Position(s) | DescriptionActions | Graphical view | Length |
---|---|---|---|---|
Compositional biasi | 12 – 18 | Poly-Gly | 7 |
Sequence similaritiesi
Belongs to the very long-chain fatty acids dehydratase HACD family.Curated
Keywords - Domaini
Transmembrane, Transmembrane helixPhylogenomic databases
eggNOGi | KOG3187, Eukaryota |
GeneTreei | ENSGT00530000062962 |
HOGENOMi | CLU_034302_2_0_1 |
InParanoidi | Q6Y1H2 |
OMAi | WAITRNY |
OrthoDBi | 1458293at2759 |
PhylomeDBi | Q6Y1H2 |
TreeFami | TF313326 |
Family and domain databases
InterProi | View protein in InterPro IPR007482, Tyr_Pase-like_PTPLA |
PANTHERi | PTHR11035, PTHR11035, 1 hit |
Pfami | View protein in Pfam PF04387, PTPLA, 1 hit |
(1+)i Sequence
Sequence statusi: Complete.
: The displayed sequence is further processed into a mature form. Sequence processingi
This entry has 1 described isoform and 2 potential isoforms that are computationally mapped.Show allAlign All
Q6Y1H2-1 [UniParc]FASTAAdd to basket
10 20 30 40 50
MAAVAATAAA KGNGGGGGRA GAGDASGTRK KKGPGPLATA YLVIYNVVMT
60 70 80 90 100
AGWLVIAVGL VRAYLAKGSY HSLYYSIEKP LKFFQTGALL EILHCAIGIV
110 120 130 140 150
PSSVVLTSFQ VMSRVFLIWA VTHSVKEVQS EDSVLLFVIA WTITEIIRYS
160 170 180 190 200
FYTFSLLNHL PYLIKWARYT LFIVLYPMGV SGELLTIYAA LPFVRQAGLY
210 220 230 240 250
SISLPNKYNF SFDYYAFLIL IMISYIPIFP QLYFHMIHQR RKILSHTEEH
KKFE
Computationally mapped potential isoform sequencesi
There are 2 potential isoforms mapped to this entry.BLASTAlignShow allAdd to basketC9JWG1 | C9JWG1_HUMAN | Very-long-chain (3R)-3-hydroxyacyl-... | HACD2 | 98 | Annotation score: | ||
H7C4K8 | H7C4K8_HUMAN | Very-long-chain (3R)-3-hydroxyacyl-... | HACD2 | 61 | Annotation score: |
Sequence databases
Select the link destinations: EMBLi GenBanki DDBJi Links Updated | AY191814 mRNA Translation: AAP20101.1 AK289983 mRNA Translation: BAF82672.1 AC020631 Genomic DNA No translation available. AC023165 Genomic DNA No translation available. AC025571 Genomic DNA No translation available. AC084039 Genomic DNA No translation available. BC049369 mRNA Translation: AAH49369.1 BC060839 mRNA Translation: AAH60839.1 |
CCDSi | CCDS46895.1 |
RefSeqi | NP_940684.1, NM_198402.4 |
Genome annotation databases
Ensembli | ENST00000383657; ENSP00000373153; ENSG00000206527 |
GeneIDi | 201562 |
KEGGi | hsa:201562 |
UCSCi | uc003egj.3, human |
Similar proteinsi
Cross-referencesi
Sequence databases
Select the link destinations: EMBLi GenBanki DDBJi Links Updated | AY191814 mRNA Translation: AAP20101.1 AK289983 mRNA Translation: BAF82672.1 AC020631 Genomic DNA No translation available. AC023165 Genomic DNA No translation available. AC025571 Genomic DNA No translation available. AC084039 Genomic DNA No translation available. BC049369 mRNA Translation: AAH49369.1 BC060839 mRNA Translation: AAH60839.1 |
CCDSi | CCDS46895.1 |
RefSeqi | NP_940684.1, NM_198402.4 |
3D structure databases
ModBasei | Search... |
SWISS-MODEL-Workspacei | Submit a new modelling project... |
Protein-protein interaction databases
BioGRIDi | 128393, 52 interactors |
IntActi | Q6Y1H2, 35 interactors |
STRINGi | 9606.ENSP00000373153 |
Chemistry databases
SwissLipidsi | SLP:000000438 |
PTM databases
GlyGeni | Q6Y1H2, 1 site |
iPTMneti | Q6Y1H2 |
PhosphoSitePlusi | Q6Y1H2 |
SwissPalmi | Q6Y1H2 |
Genetic variation databases
BioMutai | HACD2 |
DMDMi | 74738322 |
Proteomic databases
EPDi | Q6Y1H2 |
jPOSTi | Q6Y1H2 |
MassIVEi | Q6Y1H2 |
MaxQBi | Q6Y1H2 |
PaxDbi | Q6Y1H2 |
PeptideAtlasi | Q6Y1H2 |
PRIDEi | Q6Y1H2 |
ProteomicsDBi | 67831 |
TopDownProteomicsi | Q6Y1H2 |
Protocols and materials databases
Antibodypediai | 32955, 89 antibodies |
DNASUi | 201562 |
Genome annotation databases
Ensembli | ENST00000383657; ENSP00000373153; ENSG00000206527 |
GeneIDi | 201562 |
KEGGi | hsa:201562 |
UCSCi | uc003egj.3, human |
Organism-specific databases
CTDi | 201562 |
DisGeNETi | 201562 |
GeneCardsi | HACD2 |
HGNCi | HGNC:9640, HACD2 |
HPAi | ENSG00000206527, Low tissue specificity |
MIMi | 615939, gene |
neXtProti | NX_Q6Y1H2 |
OpenTargetsi | ENSG00000206527 |
PharmGKBi | PA33983 |
VEuPathDBi | HostDB:ENSG00000206527.9 |
GenAtlasi | Search... |
Phylogenomic databases
eggNOGi | KOG3187, Eukaryota |
GeneTreei | ENSGT00530000062962 |
HOGENOMi | CLU_034302_2_0_1 |
InParanoidi | Q6Y1H2 |
OMAi | WAITRNY |
OrthoDBi | 1458293at2759 |
PhylomeDBi | Q6Y1H2 |
TreeFami | TF313326 |
Enzyme and pathway databases
UniPathwayi | UPA00094 |
PathwayCommonsi | Q6Y1H2 |
Reactomei | R-HSA-75876, Synthesis of very long-chain fatty acyl-CoAs |
Miscellaneous databases
BioGRID-ORCSi | 201562, 28 hits in 995 CRISPR screens |
ChiTaRSi | HACD2, human |
GenomeRNAii | 201562 |
Pharosi | Q6Y1H2, Tbio |
PROi | PR:Q6Y1H2 |
RNActi | Q6Y1H2, protein |
SOURCEi | Search... |
Gene expression databases
Bgeei | ENSG00000206527, Expressed in adipose tissue and 250 other tissues |
ExpressionAtlasi | Q6Y1H2, baseline and differential |
Genevisiblei | Q6Y1H2, HS |
Family and domain databases
InterProi | View protein in InterPro IPR007482, Tyr_Pase-like_PTPLA |
PANTHERi | PTHR11035, PTHR11035, 1 hit |
Pfami | View protein in Pfam PF04387, PTPLA, 1 hit |
ProtoNeti | Search... |
MobiDBi | Search... |
Entry informationi
Entry namei | HACD2_HUMAN | |
Accessioni | Q6Y1H2Primary (citable) accession number: Q6Y1H2 | |
Entry historyi | Integrated into UniProtKB/Swiss-Prot: | September 2, 2008 |
Last sequence update: | July 5, 2004 | |
Last modified: | April 7, 2021 | |
This is version 146 of the entry and version 1 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
Reference proteomeDocuments
- Human chromosome 3
Human chromosome 3: entries, gene names and cross-references to MIM - MIM cross-references
Online Mendelian Inheritance in Man (MIM) cross-references in UniProtKB/Swiss-Prot - PATHWAY comments
Index of metabolic and biosynthesis pathways - SIMILARITY comments
Index of protein domains and families