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UniProtKB - Q08AH3 (ACS2A_HUMAN)
Protein
Acyl-coenzyme A synthetase ACSM2A, mitochondrial
Gene
ACSM2A
Organism
Homo sapiens (Human)
Status
Functioni
Catalyzes the activation of fatty acids by CoA to produce an acyl-CoA, the first step in fatty acid metabolism (By similarity).
Capable of activating medium-chain fatty acids (e.g. butyric (C4) to decanoic (C10) acids), and certain carboxylate-containing xenobiotics, e.g. benzoate (By similarity).
By similarityCatalytic activityi
- a medium chain fatty acid + ATP + CoA = a medium-chain fatty acyl-CoA + AMP + diphosphateBy similarityEC:6.2.1.2By similarityThis reaction proceeds in the forwardBy similarity direction.
- EC:6.2.1.25By similarityThis reaction proceeds in the forwardBy similarity direction.
- This reaction proceeds in the forwardBy similarity direction.
- This reaction proceeds in the forwardBy similarity direction.
- This reaction proceeds in the forwardBy similarity direction.
- This reaction proceeds in the forwardBy similarity direction.
Cofactori
Sites
Feature key | Position(s) | DescriptionActions | Graphical view | Length |
---|---|---|---|---|
Binding sitei | 139 | Coenzyme A1 Publication | 1 | |
Binding sitei | 364 | Substrate1 Publication | 1 | |
Binding sitei | 446 | ATP2 Publications | 1 | |
Binding sitei | 461 | ATP2 Publications | 1 | |
Binding sitei | 472 | Substrate1 Publication | 1 | |
Binding sitei | 501 | Coenzyme A1 Publication | 1 | |
Binding sitei | 532 | Coenzyme A1 Publication | 1 | |
Binding sitei | 557 | ATP2 Publications | 1 |
Regions
Feature key | Position(s) | DescriptionActions | Graphical view | Length |
---|---|---|---|---|
Nucleotide bindingi | 221 – 229 | ATP2 Publications | 9 | |
Nucleotide bindingi | 359 – 364 | ATP2 Publications | 6 |
GO - Molecular functioni
- ATP binding Source: UniProtKB-KW
- benzoate-CoA ligase activity Source: UniProtKB
- butyrate-CoA ligase activity Source: BHF-UCL
- decanoate-CoA ligase activity Source: RHEA
- fatty acid ligase activity Source: GO_Central
- fatty-acyl-CoA synthase activity Source: GO_Central
- metal ion binding Source: UniProtKB-KW
GO - Biological processi
- acyl-CoA metabolic process Source: GO_Central
- fatty acid biosynthetic process Source: GO_Central
- glucose homeostasis Source: BHF-UCL
- medium-chain fatty-acyl-CoA metabolic process Source: BHF-UCL
- triglyceride homeostasis Source: BHF-UCL
Keywordsi
Molecular function | Ligase |
Biological process | Fatty acid metabolism, Lipid metabolism |
Ligand | ATP-binding, Magnesium, Metal-binding, Nucleotide-binding |
Enzyme and pathway databases
PathwayCommonsi | Q08AH3 |
Reactomei | R-HSA-177128, Conjugation of salicylate with glycine |
SignaLinki | Q08AH3 |
Names & Taxonomyi
Protein namesi | |
Gene namesi | Name:ACSM2A Synonyms:ACSM2, MACS2 |
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:32017, ACSM2A |
MIMi | 614358, gene |
neXtProti | NX_Q08AH3 |
VEuPathDBi | HostDB:ENSG00000183747 |
Subcellular locationi
Mitochondrion
- Mitochondrion By similarity
Mitochondrion
- mitochondrial matrix Source: GO_Central
- mitochondrion Source: UniProtKB
Keywords - Cellular componenti
MitochondrionPathology & Biotechi
Organism-specific databases
DisGeNETi | 123876 |
OpenTargetsi | ENSG00000183747 |
PharmGKBi | PA162375402 |
Miscellaneous databases
Pharosi | Q08AH3, Tbio |
Genetic variation databases
BioMutai | ACSM2A |
DMDMi | 257050995 |
PTM / Processingi
Molecule processing
Feature key | Position(s) | DescriptionActions | Graphical view | Length |
---|---|---|---|---|
Transit peptidei | 1 – 46 | MitochondrionSequence analysisAdd BLAST | 46 | |
ChainiPRO_0000306093 | 47 – 577 | Acyl-coenzyme A synthetase ACSM2A, mitochondrialAdd BLAST | 531 |
Amino acid modifications
Feature key | Position(s) | DescriptionActions | Graphical view | Length |
---|---|---|---|---|
Modified residuei | 513 | PhosphoserineBy similarity | 1 |
Keywords - PTMi
PhosphoproteinProteomic databases
MassIVEi | Q08AH3 |
MaxQBi | Q08AH3 |
PaxDbi | Q08AH3 |
PeptideAtlasi | Q08AH3 |
PRIDEi | Q08AH3 |
ProteomicsDBi | 58672 |
PTM databases
iPTMneti | Q08AH3 |
PhosphoSitePlusi | Q08AH3 |
Expressioni
Gene expression databases
Bgeei | ENSG00000183747, Expressed in right lobe of liver and 91 other tissues |
ExpressionAtlasi | Q08AH3, baseline and differential |
Genevisiblei | Q08AH3, HS |
Organism-specific databases
HPAi | ENSG00000183747, Group enriched (kidney, liver) |
Interactioni
Subunit structurei
Monomer.
By similarityProtein-protein interaction databases
BioGRIDi | 125840, 2 interactors |
IntActi | Q08AH3, 1 interactor |
MINTi | Q08AH3 |
STRINGi | 9606.ENSP00000459451 |
Miscellaneous databases
RNActi | Q08AH3, protein |
Structurei
Secondary structure
Legend: HelixTurnBeta strandPDB Structure known for this area
Show more details3D structure databases
AlphaFoldDBi | Q08AH3 |
SMRi | Q08AH3 |
ModBasei | Search... |
PDBe-KBi | Search... |
Miscellaneous databases
EvolutionaryTracei | Q08AH3 |
Family & Domainsi
Region
Feature key | Position(s) | DescriptionActions | Graphical view | Length |
---|---|---|---|---|
Regioni | 469 – 471 | Coenzyme A binding1 Publication | 3 | |
Regioni | 540 – 542 | Coenzyme A binding1 Publication | 3 |
Sequence similaritiesi
Belongs to the ATP-dependent AMP-binding enzyme family.Curated
Keywords - Domaini
Transit peptidePhylogenomic databases
eggNOGi | KOG1175, Eukaryota |
GeneTreei | ENSGT00940000164294 |
InParanoidi | Q08AH3 |
OMAi | TPSDWAW |
OrthoDBi | 683933at2759 |
PhylomeDBi | Q08AH3 |
TreeFami | TF354264 |
Family and domain databases
Gene3Di | 3.30.300.30, 1 hit 3.40.50.12780, 1 hit |
InterProi | View protein in InterPro IPR025110, AMP-bd_C IPR045851, AMP-bd_C_sf IPR020845, AMP-binding_CS IPR000873, AMP-dep_Synth/Lig IPR042099, ANL_N_sf |
Pfami | View protein in Pfam PF00501, AMP-binding, 1 hit PF13193, AMP-binding_C, 1 hit |
PROSITEi | View protein in PROSITE PS00455, AMP_BINDING, 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 5 potential isoforms that are computationally mapped.Show allAlign All
Q08AH3-1 [UniParc]FASTAAdd to basket
10 20 30 40 50
MHWLRKVQGL CTLWGTQMSS RTLYINSRQL VSLQWGHQEV PAKFNFASDV
60 70 80 90 100
LDHWADMEKA GKRLPSPALW WVNGKGKELM WNFRELSENS QQAANVLSGA
110 120 130 140 150
CGLQRGDRVA VVLPRVPEWW LVILGCIRAG LIFMPGTIQM KSTDILYRLQ
160 170 180 190 200
MSKAKAIVAG DEVIQEVDTV ASECPSLRIK LLVSEKSCDG WLNFKKLLNE
210 220 230 240 250
ASTTHHCVET GSQEASAIYF TSGTSGLPKM AEHSYSSLGL KAKMDAGWTG
260 270 280 290 300
LQASDIMWTI SDTGWILNIL CSLMEPWALG ACTFVHLLPK FDPLVILKTL
310 320 330 340 350
SSYPIKSMMG APIVYRMLLQ QDLSSYKFPH LQNCVTVGES LLPETLENWR
360 370 380 390 400
AQTGLDIRES YGQTETGLTC MVSKTMKIKP GYMGTAASCY DVQIIDDKGN
410 420 430 440 450
VLPPGTEGDI GIRVKPIRPI GIFSGYVDNP DKTAANIRGD FWLLGDRGIK
460 470 480 490 500
DEDGYFQFMG RANDIINSSG YRIGPSEVEN ALMEHPAVVE TAVISSPDPV
510 520 530 540 550
RGEVVKAFVV LASQFLSHDP EQLTKELQQH VKSVTAPYKY PRKIEFVLNL
560 570
PKTVTGKIQR AKLRDKEWKM SGKARAQ
Computationally mapped potential isoform sequencesi
There are 5 potential isoforms mapped to this entry.BLASTAlignShow allAdd to basketF5GWL3 | F5GWL3_HUMAN | Acyl-coenzyme A synthetase ACSM2A, ... | ACSM2A | 498 | Annotation score: | ||
I3L339 | I3L339_HUMAN | Acyl-coenzyme A synthetase ACSM2A, ... | ACSM2A | 61 | Annotation score: | ||
E7EPS5 | E7EPS5_HUMAN | Acyl-coenzyme A synthetase ACSM2A, ... | ACSM2A | 208 | Annotation score: | ||
I3L2Y5 | I3L2Y5_HUMAN | Acyl-coenzyme A synthetase ACSM2A, ... | ACSM2A | 118 | Annotation score: | ||
I3L438 | I3L438_HUMAN | Acyl-coenzyme A synthetase ACSM2A, ... | ACSM2A | 105 | Annotation score: |
Sequence cautioni
The sequence AAC23497 differs from that shown. Reason: Erroneous gene model prediction.Curated
Experimental Info
Feature key | Position(s) | DescriptionActions | Graphical view | Length |
---|---|---|---|---|
Sequence conflicti | 463 | N → D in BAG53201 (PubMed:14702039).Curated | 1 | |
Sequence conflicti | 463 | N → D in AAI25177 (PubMed:15489334).Curated | 1 | |
Sequence conflicti | 463 | N → D in AAC23497 (PubMed:10493829).Curated | 1 |
Natural variant
Feature key | Position(s) | DescriptionActions | Graphical view | Length |
---|---|---|---|---|
Natural variantiVAR_058692 | 335 | V → L. Corresponds to variant dbSNP:rs4643305Ensembl. | 1 | |
Natural variantiVAR_058694 | 337 | V → G. Corresponds to variant dbSNP:rs4586421Ensembl. | 1 | |
Natural variantiVAR_035247 | 513 | S → L3 PublicationsCorresponds to variant dbSNP:rs1133607Ensembl. | 1 | |
Natural variantiVAR_035248 | 561 | A → T. Corresponds to variant dbSNP:rs1054977Ensembl. | 1 |
Sequence databases
Select the link destinations: EMBLi GenBanki DDBJi Links Updated | AK096039 mRNA Translation: BAG53201.1 AC137056 Genomic DNA No translation available. BC125176 mRNA Translation: AAI25177.1 AC003034 Genomic DNA Translation: AAC23497.1 Sequence problems. |
CCDSi | CCDS32401.1 |
RefSeqi | NP_001295101.1, NM_001308172.1 NP_001295883.1, NM_001308954.1 XP_016878412.1, XM_017022923.1 |
Genome annotation databases
Ensembli | ENST00000219054.10; ENSP00000219054.6; ENSG00000183747.12 ENST00000396104.2; ENSP00000379411.2; ENSG00000183747.12 ENST00000573854.6; ENSP00000459451.1; ENSG00000183747.12 ENST00000575690.5; ENSP00000460349.1; ENSG00000183747.12 |
GeneIDi | 123876 |
KEGGi | hsa:123876 |
MANE-Selecti | ENST00000573854.6; ENSP00000459451.1; NM_001308172.2; NP_001295101.1 |
UCSCi | uc002dhf.5, human |
Similar proteinsi
Cross-referencesi
Sequence databases
Select the link destinations: EMBLi GenBanki DDBJi Links Updated | AK096039 mRNA Translation: BAG53201.1 AC137056 Genomic DNA No translation available. BC125176 mRNA Translation: AAI25177.1 AC003034 Genomic DNA Translation: AAC23497.1 Sequence problems. |
CCDSi | CCDS32401.1 |
RefSeqi | NP_001295101.1, NM_001308172.1 NP_001295883.1, NM_001308954.1 XP_016878412.1, XM_017022923.1 |
3D structure databases
Select the link destinations: PDBei RCSB PDBi PDBji Links Updated | PDB entry | Method | Resolution (Å) | Chain | Positions | PDBsum |
2VZE | X-ray | 2.45 | A/B/C | 32-577 | [»] | |
2WD9 | X-ray | 2.60 | A/B/C | 32-576 | [»] | |
3B7W | X-ray | 2.00 | A | 32-577 | [»] | |
3C5E | X-ray | 1.60 | A | 32-577 | [»] | |
3DAY | X-ray | 1.95 | A | 32-577 | [»] | |
3EQ6 | X-ray | 2.40 | A/B | 32-577 | [»] | |
3GPC | X-ray | 1.90 | A/B | 32-577 | [»] | |
AlphaFoldDBi | Q08AH3 | |||||
SMRi | Q08AH3 | |||||
ModBasei | Search... | |||||
PDBe-KBi | Search... |
Protein-protein interaction databases
BioGRIDi | 125840, 2 interactors |
IntActi | Q08AH3, 1 interactor |
MINTi | Q08AH3 |
STRINGi | 9606.ENSP00000459451 |
PTM databases
iPTMneti | Q08AH3 |
PhosphoSitePlusi | Q08AH3 |
Genetic variation databases
BioMutai | ACSM2A |
DMDMi | 257050995 |
Proteomic databases
MassIVEi | Q08AH3 |
MaxQBi | Q08AH3 |
PaxDbi | Q08AH3 |
PeptideAtlasi | Q08AH3 |
PRIDEi | Q08AH3 |
ProteomicsDBi | 58672 |
Protocols and materials databases
Antibodypediai | 67877, 84 antibodies from 20 providers |
DNASUi | 123876 |
Genome annotation databases
Ensembli | ENST00000219054.10; ENSP00000219054.6; ENSG00000183747.12 ENST00000396104.2; ENSP00000379411.2; ENSG00000183747.12 ENST00000573854.6; ENSP00000459451.1; ENSG00000183747.12 ENST00000575690.5; ENSP00000460349.1; ENSG00000183747.12 |
GeneIDi | 123876 |
KEGGi | hsa:123876 |
MANE-Selecti | ENST00000573854.6; ENSP00000459451.1; NM_001308172.2; NP_001295101.1 |
UCSCi | uc002dhf.5, human |
Organism-specific databases
CTDi | 123876 |
DisGeNETi | 123876 |
GeneCardsi | ACSM2A |
HGNCi | HGNC:32017, ACSM2A |
HPAi | ENSG00000183747, Group enriched (kidney, liver) |
MIMi | 614358, gene |
neXtProti | NX_Q08AH3 |
OpenTargetsi | ENSG00000183747 |
PharmGKBi | PA162375402 |
VEuPathDBi | HostDB:ENSG00000183747 |
GenAtlasi | Search... |
Phylogenomic databases
eggNOGi | KOG1175, Eukaryota |
GeneTreei | ENSGT00940000164294 |
InParanoidi | Q08AH3 |
OMAi | TPSDWAW |
OrthoDBi | 683933at2759 |
PhylomeDBi | Q08AH3 |
TreeFami | TF354264 |
Enzyme and pathway databases
PathwayCommonsi | Q08AH3 |
Reactomei | R-HSA-177128, Conjugation of salicylate with glycine |
SignaLinki | Q08AH3 |
Miscellaneous databases
BioGRID-ORCSi | 123876, 7 hits in 1020 CRISPR screens |
ChiTaRSi | ACSM2A, human |
EvolutionaryTracei | Q08AH3 |
GenomeRNAii | 123876 |
Pharosi | Q08AH3, Tbio |
PROi | PR:Q08AH3 |
RNActi | Q08AH3, protein |
SOURCEi | Search... |
Gene expression databases
Bgeei | ENSG00000183747, Expressed in right lobe of liver and 91 other tissues |
ExpressionAtlasi | Q08AH3, baseline and differential |
Genevisiblei | Q08AH3, HS |
Family and domain databases
Gene3Di | 3.30.300.30, 1 hit 3.40.50.12780, 1 hit |
InterProi | View protein in InterPro IPR025110, AMP-bd_C IPR045851, AMP-bd_C_sf IPR020845, AMP-binding_CS IPR000873, AMP-dep_Synth/Lig IPR042099, ANL_N_sf |
Pfami | View protein in Pfam PF00501, AMP-binding, 1 hit PF13193, AMP-binding_C, 1 hit |
PROSITEi | View protein in PROSITE PS00455, AMP_BINDING, 1 hit |
MobiDBi | Search... |
Entry informationi
Entry namei | ACS2A_HUMAN | |
Accessioni | Q08AH3Primary (citable) accession number: Q08AH3 Secondary accession number(s): B3KTT9, O75202 | |
Entry historyi | Integrated into UniProtKB/Swiss-Prot: | October 2, 2007 |
Last sequence update: | September 1, 2009 | |
Last modified: | May 25, 2022 | |
This is version 115 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
3D-structure, Reference proteomeDocuments
- Human chromosome 16
Human chromosome 16: entries, gene names and cross-references to MIM - Human entries with genetic variants
List of human entries with genetic variants - Human variants curated from literature reports
Index of human variants curated from literature reports - MIM cross-references
Online Mendelian Inheritance in Man (MIM) cross-references in UniProtKB/Swiss-Prot - PDB cross-references
Index of Protein Data Bank (PDB) cross-references - SIMILARITY comments
Index of protein domains and families