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Protein

Acyl-coenzyme A synthetase ACSM2A, mitochondrial

Gene

ACSM2A

Organism
Homo sapiens (Human)
Status
Reviewed-Annotation score: Annotation score: 5 out of 5-Experimental evidence at protein leveli

Functioni

Has medium-chain fatty acid:CoA ligase activity with broad substrate specificity (in vitro). Acts on acids from C4 to C(11) and on the corresponding 3-hydroxy- and 2,3- or 3,4-unsaturated acids (in vitro) (By similarity).By similarity

Catalytic activityi

ATP + a carboxylate + CoA = AMP + diphosphate + an acyl-CoA.

Cofactori

Mg2+2 Publications, Mn2+2 Publications

Sites

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Binding sitei139Coenzyme A1 Publication1
Binding sitei364Substrate1 Publication1
Binding sitei446ATP2 Publications1
Binding sitei461ATP2 Publications1
Binding sitei472Substrate1 Publication1
Binding sitei501Coenzyme A1 Publication1
Binding sitei532Coenzyme A1 Publication1
Binding sitei557ATP2 Publications1

Regions

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Nucleotide bindingi221 – 229ATP2 Publications9
Nucleotide bindingi359 – 364ATP2 Publications6

GO - Molecular functioni

GO - Biological processi

  • 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
Complete GO annotation...

Keywords - Molecular functioni

Ligase

Keywords - Biological processi

Fatty acid metabolism, Lipid metabolism

Keywords - Ligandi

ATP-binding, Magnesium, Metal-binding, Nucleotide-binding

Enzyme and pathway databases

BioCyciZFISH:HS11921-MONOMER.
ReactomeiR-HSA-177128. Conjugation of salicylate with glycine.

Names & Taxonomyi

Protein namesi
Recommended name:
Acyl-coenzyme A synthetase ACSM2A, mitochondrial (EC:6.2.1.2)
Alternative name(s):
Acyl-CoA synthetase medium-chain family member 2A
Butyrate--CoA ligase 2A
Butyryl-coenzyme A synthetase 2A
Middle-chain acyl-CoA synthetase 2A
Gene namesi
Name:ACSM2A
Synonyms:ACSM2, MACS2
OrganismiHomo sapiens (Human)
Taxonomic identifieri9606 [NCBI]
Taxonomic lineageiEukaryotaMetazoaChordataCraniataVertebrataEuteleostomiMammaliaEutheriaEuarchontogliresPrimatesHaplorrhiniCatarrhiniHominidaeHomo
Proteomesi
  • UP000005640 Componenti: Chromosome 16

Organism-specific databases

HGNCiHGNC:32017. ACSM2A.

Subcellular locationi

GO - Cellular componenti

  • mitochondrial matrix Source: GO_Central
  • mitochondrion Source: BHF-UCL
Complete GO annotation...

Keywords - Cellular componenti

Mitochondrion

Pathology & Biotechi

Organism-specific databases

DisGeNETi123876.
OpenTargetsiENSG00000183747.
PharmGKBiPA162375402.

Polymorphism and mutation databases

BioMutaiACSM2A.
DMDMi257050995.

PTM / Processingi

Molecule processing

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Transit peptidei1 – 46MitochondrionSequence analysisAdd BLAST46
ChainiPRO_000030609347 – 577Acyl-coenzyme A synthetase ACSM2A, mitochondrialAdd BLAST531

Amino acid modifications

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Modified residuei141N6-acetyllysine; alternateBy similarity1
Modified residuei141N6-succinyllysine; alternateBy similarity1
Modified residuei327N6-acetyllysineBy similarity1
Modified residuei513PhosphoserineBy similarity1
Modified residuei525N6-acetyllysineBy similarity1
Modified residuei532N6-acetyllysine; alternateBy similarity1
Modified residuei532N6-succinyllysine; alternateBy similarity1
Modified residuei543N6-acetyllysineBy similarity1

Keywords - PTMi

Acetylation, Phosphoprotein

Proteomic databases

MaxQBiQ08AH3.
PaxDbiQ08AH3.
PeptideAtlasiQ08AH3.
PRIDEiQ08AH3.

PTM databases

iPTMnetiQ08AH3.
PhosphoSitePlusiQ08AH3.

Expressioni

Gene expression databases

BgeeiENSG00000183747.
ExpressionAtlasiQ08AH3. baseline and differential.
GenevisibleiQ08AH3. HS.

Organism-specific databases

HPAiHPA057699.

Interactioni

Subunit structurei

Monomer.By similarity

Protein-protein interaction databases

STRINGi9606.ENSP00000219054.

Structurei

Secondary structure

1577
Legend: HelixTurnBeta strandPDB Structure known for this area
Show more details
Feature keyPosition(s)DescriptionActionsGraphical viewLength
Turni34 – 36Combined sources3
Helixi46 – 49Combined sources4
Helixi51 – 59Combined sources9
Beta strandi67 – 72Combined sources6
Beta strandi74 – 76Combined sources3
Beta strandi78 – 82Combined sources5
Helixi83 – 98Combined sources16
Turni99 – 101Combined sources3
Beta strandi108 – 112Combined sources5
Helixi117 – 129Combined sources13
Beta strandi132 – 135Combined sources4
Helixi142 – 152Combined sources11
Beta strandi155 – 160Combined sources6
Turni161 – 163Combined sources3
Helixi164 – 170Combined sources7
Helixi171 – 173Combined sources3
Beta strandi179 – 186Combined sources8
Beta strandi191 – 193Combined sources3
Helixi194 – 200Combined sources7
Beta strandi214 – 220Combined sources7
Beta strandi224 – 227Combined sources4
Beta strandi230 – 234Combined sources5
Helixi235 – 245Combined sources11
Turni246 – 249Combined sources4
Beta strandi256 – 259Combined sources4
Helixi266 – 271Combined sources6
Helixi274 – 278Combined sources5
Beta strandi282 – 286Combined sources5
Helixi293 – 302Combined sources10
Beta strandi307 – 310Combined sources4
Helixi312 – 319Combined sources8
Turni323 – 325Combined sources3
Beta strandi333 – 339Combined sources7
Helixi343 – 353Combined sources11
Beta strandi358 – 363Combined sources6
Turni364 – 366Combined sources3
Beta strandi367 – 371Combined sources5
Beta strandi392 – 395Combined sources4
Beta strandi408 – 413Combined sources6
Beta strandi415 – 417Combined sources3
Helixi430 – 435Combined sources6
Beta strandi441 – 450Combined sources10
Beta strandi456 – 461Combined sources6
Helixi462 – 464Combined sources3
Beta strandi466 – 468Combined sources3
Beta strandi471 – 473Combined sources3
Helixi475 – 483Combined sources9
Beta strandi488 – 498Combined sources11
Turni499 – 501Combined sources3
Beta strandi502 – 511Combined sources10
Helixi513 – 515Combined sources3
Helixi520 – 534Combined sources15
Helixi537 – 539Combined sources3
Beta strandi542 – 548Combined sources7
Helixi560 – 567Combined sources8

3D structure databases

Select the link destinations:
PDBei
RCSB PDBi
PDBji
Links Updated
PDB entryMethodResolution (Å)ChainPositionsPDBsum
2VZEX-ray2.45A/B/C32-577[»]
2WD9X-ray2.60A/B/C32-576[»]
3B7WX-ray2.00A32-577[»]
3C5EX-ray1.60A32-577[»]
3DAYX-ray1.95A32-577[»]
3EQ6X-ray2.40A/B32-577[»]
3GPCX-ray1.90A/B32-577[»]
ProteinModelPortaliQ08AH3.
SMRiQ08AH3.
ModBaseiSearch...
MobiDBiSearch...

Miscellaneous databases

EvolutionaryTraceiQ08AH3.

Family & Domainsi

Region

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Regioni469 – 471Coenzyme A binding1 Publication3
Regioni540 – 542Coenzyme A binding1 Publication3

Sequence similaritiesi

Keywords - Domaini

Transit peptide

Phylogenomic databases

eggNOGiKOG1175. Eukaryota.
COG0365. LUCA.
GeneTreeiENSGT00760000119178.
HOGENOMiHOG000229982.
HOVERGENiHBG053031.
InParanoidiQ08AH3.
KOiK01896.
OMAiVIDHWAG.
OrthoDBiEOG091G03NW.
PhylomeDBiQ08AH3.
TreeFamiTF354264.

Family and domain databases

InterProiIPR025110. AMP-bd_C.
IPR020845. AMP-binding_CS.
IPR000873. AMP-dep_Synth/Lig.
[Graphical view]
PfamiPF00501. AMP-binding. 1 hit.
PF13193. AMP-binding_C. 1 hit.
[Graphical view]
PROSITEiPS00455. AMP_BINDING. 1 hit.
[Graphical view]

Sequencei

Sequence statusi: Complete.

Sequence processingi: The displayed sequence is further processed into a mature form.

Q08AH3-1 [UniParc]FASTAAdd to basket

« Hide

        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
Length:577
Mass (Da):64,223
Last modified:September 1, 2009 - v2
Checksum:i16167FC69562436F
GO

Sequence cautioni

The sequence AAC23497 differs from that shown. Reason: Erroneous gene model prediction.Curated

Experimental Info

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Sequence conflicti463N → D in BAG53201 (PubMed:14702039).Curated1
Sequence conflicti463N → D in AAI25177 (PubMed:15489334).Curated1
Sequence conflicti463N → D in AAC23497 (PubMed:10493829).Curated1

Natural variant

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Natural variantiVAR_058692335V → L.Corresponds to variant rs4643305dbSNPEnsembl.1
Natural variantiVAR_058693336T → A.Corresponds to variant rs5002299dbSNPEnsembl.1
Natural variantiVAR_058694337V → G.Corresponds to variant rs4586421dbSNPEnsembl.1
Natural variantiVAR_035247513S → L.3 PublicationsCorresponds to variant rs1133607dbSNPEnsembl.1
Natural variantiVAR_035248561A → T.Corresponds to variant rs1054977dbSNPEnsembl.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.
CCDSiCCDS32401.1.
RefSeqiNP_001295101.1. NM_001308172.1.
NP_001295883.1. NM_001308954.1.
XP_016878412.1. XM_017022923.1.
UniGeneiHs.298252.
Hs.656497.

Genome annotation databases

EnsembliENST00000219054; ENSP00000219054; ENSG00000183747.
ENST00000396104; ENSP00000379411; ENSG00000183747.
ENST00000573854; ENSP00000459451; ENSG00000183747.
ENST00000575690; ENSP00000460349; ENSG00000183747.
GeneIDi123876.
KEGGihsa:123876.
UCSCiuc002dhf.5. human.

Keywords - Coding sequence diversityi

Polymorphism

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.
CCDSiCCDS32401.1.
RefSeqiNP_001295101.1. NM_001308172.1.
NP_001295883.1. NM_001308954.1.
XP_016878412.1. XM_017022923.1.
UniGeneiHs.298252.
Hs.656497.

3D structure databases

Select the link destinations:
PDBei
RCSB PDBi
PDBji
Links Updated
PDB entryMethodResolution (Å)ChainPositionsPDBsum
2VZEX-ray2.45A/B/C32-577[»]
2WD9X-ray2.60A/B/C32-576[»]
3B7WX-ray2.00A32-577[»]
3C5EX-ray1.60A32-577[»]
3DAYX-ray1.95A32-577[»]
3EQ6X-ray2.40A/B32-577[»]
3GPCX-ray1.90A/B32-577[»]
ProteinModelPortaliQ08AH3.
SMRiQ08AH3.
ModBaseiSearch...
MobiDBiSearch...

Protein-protein interaction databases

STRINGi9606.ENSP00000219054.

PTM databases

iPTMnetiQ08AH3.
PhosphoSitePlusiQ08AH3.

Polymorphism and mutation databases

BioMutaiACSM2A.
DMDMi257050995.

Proteomic databases

MaxQBiQ08AH3.
PaxDbiQ08AH3.
PeptideAtlasiQ08AH3.
PRIDEiQ08AH3.

Protocols and materials databases

Structural Biology KnowledgebaseSearch...

Genome annotation databases

EnsembliENST00000219054; ENSP00000219054; ENSG00000183747.
ENST00000396104; ENSP00000379411; ENSG00000183747.
ENST00000573854; ENSP00000459451; ENSG00000183747.
ENST00000575690; ENSP00000460349; ENSG00000183747.
GeneIDi123876.
KEGGihsa:123876.
UCSCiuc002dhf.5. human.

Organism-specific databases

CTDi123876.
DisGeNETi123876.
GeneCardsiACSM2A.
H-InvDBHIX0012859.
HGNCiHGNC:32017. ACSM2A.
HPAiHPA057699.
MIMi614358. gene.
neXtProtiNX_Q08AH3.
OpenTargetsiENSG00000183747.
PharmGKBiPA162375402.
GenAtlasiSearch...

Phylogenomic databases

eggNOGiKOG1175. Eukaryota.
COG0365. LUCA.
GeneTreeiENSGT00760000119178.
HOGENOMiHOG000229982.
HOVERGENiHBG053031.
InParanoidiQ08AH3.
KOiK01896.
OMAiVIDHWAG.
OrthoDBiEOG091G03NW.
PhylomeDBiQ08AH3.
TreeFamiTF354264.

Enzyme and pathway databases

BioCyciZFISH:HS11921-MONOMER.
ReactomeiR-HSA-177128. Conjugation of salicylate with glycine.

Miscellaneous databases

EvolutionaryTraceiQ08AH3.
GenomeRNAii123876.
PROiQ08AH3.
SOURCEiSearch...

Gene expression databases

BgeeiENSG00000183747.
ExpressionAtlasiQ08AH3. baseline and differential.
GenevisibleiQ08AH3. HS.

Family and domain databases

InterProiIPR025110. AMP-bd_C.
IPR020845. AMP-binding_CS.
IPR000873. AMP-dep_Synth/Lig.
[Graphical view]
PfamiPF00501. AMP-binding. 1 hit.
PF13193. AMP-binding_C. 1 hit.
[Graphical view]
PROSITEiPS00455. AMP_BINDING. 1 hit.
[Graphical view]
ProtoNetiSearch...

Entry informationi

Entry nameiACS2A_HUMAN
AccessioniPrimary (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: November 30, 2016
This is version 86 of the entry and version 2 of the sequence. [Complete history]
Entry statusiReviewed (UniProtKB/Swiss-Prot)
Annotation programChordata Protein Annotation Program
DisclaimerAny 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, Complete proteome, Reference proteome

Documents

  1. Human chromosome 16
    Human chromosome 16: entries, gene names and cross-references to MIM
  2. Human entries with polymorphisms or disease mutations
    List of human entries with polymorphisms or disease mutations
  3. Human polymorphisms and disease mutations
    Index of human polymorphisms and disease mutations
  4. MIM cross-references
    Online Mendelian Inheritance in Man (MIM) cross-references in UniProtKB/Swiss-Prot
  5. PDB cross-references
    Index of Protein Data Bank (PDB) cross-references
  6. SIMILARITY comments
    Index of protein domains and families

Similar proteinsi

Links to similar proteins from the UniProt Reference Clusters (UniRef) at 100%, 90% and 50% sequence identity:
100%UniRef100 combines identical sequences and sub-fragments with 11 or more residues from any organism into one UniRef entry.
90%UniRef90 is built by clustering UniRef100 sequences that have at least 90% sequence identity to, and 80% overlap with, the longest sequence (a.k.a seed sequence).
50%UniRef50 is built by clustering UniRef90 seed sequences that have at least 50% sequence identity to, and 80% overlap with, the longest sequence in the cluster.