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Protein

Acyl-coenzyme A synthetase ACSM3, mitochondrial

Gene

ACSM3

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+By similarity

Sites

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Binding sitei461ATPBy similarity1
Binding sitei476ATPBy similarity1
Binding sitei572ATPBy similarity1

Regions

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Nucleotide bindingi235 – 243ATPBy similarity9
Nucleotide bindingi374 – 379ATPBy similarity6

GO - Molecular functioni

GO - Biological processi

  • acyl-CoA metabolic process Source: GO_Central
  • cholesterol homeostasis Source: BHF-UCL
  • fatty acid biosynthetic process Source: GO_Central
  • regulation of blood pressure Source: UniProtKB
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:HS00131-MONOMER.
ReactomeiR-HSA-77352. Beta oxidation of butanoyl-CoA to acetyl-CoA.

Chemistry databases

SwissLipidsiSLP:000001189.

Names & Taxonomyi

Protein namesi
Recommended name:
Acyl-coenzyme A synthetase ACSM3, mitochondrial (EC:6.2.1.2)
Alternative name(s):
Acyl-CoA synthetase medium-chain family member 3
Butyrate--CoA ligase 3
Butyryl-coenzyme A synthetase 3
Middle-chain acyl-CoA synthetase 3
Protein SA homolog
Gene namesi
Name:ACSM3
Synonyms:SAH
OrganismiHomo sapiens (Human)
Taxonomic identifieri9606 [NCBI]
Taxonomic lineageiEukaryotaMetazoaChordataCraniataVertebrataEuteleostomiMammaliaEutheriaEuarchontogliresPrimatesHaplorrhiniCatarrhiniHominidaeHomo
Proteomesi
  • UP000005640 Componenti: Chromosome 16

Organism-specific databases

HGNCiHGNC:10522. ACSM3.

Subcellular locationi

GO - Cellular componenti

Complete GO annotation...

Keywords - Cellular componenti

Mitochondrion

Pathology & Biotechi

Organism-specific databases

DisGeNETi6296.
MalaCardsiACSM3.
OpenTargetsiENSG00000005187.
PharmGKBiPA34930.

Polymorphism and mutation databases

BioMutaiACSM3.
DMDMi158706483.

PTM / Processingi

Molecule processing

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Transit peptidei1 – 27MitochondrionSequence analysisAdd BLAST27
ChainiPRO_000030609728 – 586Acyl-coenzyme A synthetase ACSM3, mitochondrialAdd BLAST559

Amino acid modifications

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Modified residuei73N6-succinyllysineBy similarity1
Modified residuei106N6-succinyllysineBy similarity1
Modified residuei157N6-acetyllysineBy similarity1

Keywords - PTMi

Acetylation

Proteomic databases

MaxQBiQ53FZ2.
PaxDbiQ53FZ2.
PeptideAtlasiQ53FZ2.
PRIDEiQ53FZ2.

PTM databases

iPTMnetiQ53FZ2.
PhosphoSitePlusiQ53FZ2.

Expressioni

Gene expression databases

BgeeiENSG00000005187.
CleanExiHS_ACSM3.
ExpressionAtlasiQ53FZ2. baseline and differential.
GenevisibleiQ53FZ2. HS.

Organism-specific databases

HPAiHPA041013.

Interactioni

Protein-protein interaction databases

BioGridi112203. 1 interactor.
IntActiQ53FZ2. 1 interactor.
STRINGi9606.ENSP00000289416.

Structurei

3D structure databases

ProteinModelPortaliQ53FZ2.
SMRiQ53FZ2.
ModBaseiSearch...
MobiDBiSearch...

Family & Domainsi

Sequence similaritiesi

Keywords - Domaini

Transit peptide

Phylogenomic databases

eggNOGiKOG1175. Eukaryota.
COG0365. LUCA.
GeneTreeiENSGT00760000119178.
HOGENOMiHOG000229982.
HOVERGENiHBG053031.
InParanoidiQ53FZ2.
KOiK01896.
OMAiAIPKAYV.
OrthoDBiEOG091G03NW.
PhylomeDBiQ53FZ2.
TreeFamiTF354287.

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]

Sequences (2)i

Sequence statusi: Complete.

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

This entry describes 2 isoformsi produced by alternative splicing. AlignAdd to basket

Isoform 1 (identifier: Q53FZ2-1) [UniParc]FASTAAdd 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
MLARVTRKML RHAKCFQRLA IFGSVRALHK DNRTATPQNF SNYESMKQDF
60 70 80 90 100
KLGIPEYFNF AKDVLDQWTD KEKAGKKPSN PAFWWINRNG EEMRWSFEEL
110 120 130 140 150
GSLSRKFANI LSEACSLQRG DRVILILPRV PEWWLANVAC LRTGTVLIPG
160 170 180 190 200
TTQLTQKDIL YRLQSSKANC IITNDVLAPA VDAVASKCEN LHSKLIVSEN
210 220 230 240 250
SREGWGNLKE LMKHASDSHT CVKTKHNEIM AIFFTSGTSG YPKMTAHTHS
260 270 280 290 300
SFGLGLSVNG RFWLDLTPSD VMWNTSDTGW AKSAWSSVFS PWIQGACVFT
310 320 330 340 350
HHLPRFEPTS ILQTLSKYPI TVFCSAPTVY RMLVQNDITS YKFKSLKHCV
360 370 380 390 400
SAGEPITPDV TEKWRNKTGL DIYEGYGQTE TVLICGNFKG MKIKPGSMGK
410 420 430 440 450
PSPAFDVKIV DVNGNVLPPG QEGDIGIQVL PNRPFGLFTH YVDNPSKTAS
460 470 480 490 500
TLRGNFYITG DRGYMDKDGY FWFVARADDV ILSSGYRIGP FEVENALNEH
510 520 530 540 550
PSVAESAVVS SPDPIRGEVV KAFVVLNPDY KSHDQEQLIK EIQEHVKKTT
560 570 580
APYKYPRKVE FIQELPKTIS GKTKRNELRK KEWKTI
Length:586
Mass (Da):66,153
Last modified:October 2, 2007 - v2
Checksum:i329967C751F11F1D
GO
Isoform 2 (identifier: Q53FZ2-2) [UniParc]FASTAAdd to basket

The sequence of this isoform differs from the canonical sequence as follows:
     409-438: IVDVNGNVLPPGQEGDIGIQVLPNRPFGLF → VCTSPSRRMFNNPICTLPTYRLPPYKLSLL
     439-586: Missing.

Show »
Length:438
Mass (Da):49,485
Checksum:iA94538369727118F
GO

Sequence cautioni

The sequence AAC31667 differs from that shown. Reason: Erroneous initiation.Curated
The sequence BAA03853 differs from that shown. Reason: Erroneous initiation.Curated
The sequence CAA56369 differs from that shown. Reason: Erroneous initiation.Curated

Experimental Info

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Sequence conflicti132E → R in BAA03853 (PubMed:7907320).Curated1
Sequence conflicti237G → A in BAA03853 (PubMed:7907320).Curated1
Sequence conflicti245T → S in BAA03853 (PubMed:7907320).Curated1
Sequence conflicti407V → D in BAD96859 (Ref. 4) Curated1
Sequence conflicti463G → A in BAA03853 (PubMed:7907320).Curated1
Sequence conflicti569I → V in BAA03853 (PubMed:7907320).Curated1

Natural variant

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Natural variantiVAR_035249100L → P.Corresponds to variant rs5713dbSNPEnsembl.1
Natural variantiVAR_048239270D → H.Corresponds to variant rs13306603dbSNPEnsembl.1
Natural variantiVAR_035250308P → T.Corresponds to variant rs7196188dbSNPEnsembl.1
Natural variantiVAR_035251367K → N.2 PublicationsCorresponds to variant rs5716dbSNPEnsembl.1

Alternative sequence

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Alternative sequenceiVSP_028395409 – 438IVDVN…PFGLF → VCTSPSRRMFNNPICTLPTY RLPPYKLSLL in isoform 2. 2 PublicationsAdd BLAST30
Alternative sequenceiVSP_028396439 – 586Missing in isoform 2. 2 PublicationsAdd BLAST148

Sequence databases

Select the link destinations:
EMBLi
GenBanki
DDBJi
Links Updated
D16350 mRNA. Translation: BAA03853.1. Different initiation.
X80062 mRNA. Translation: CAA56369.1. Different initiation.
AC004381 Genomic DNA. Translation: AAC31667.1. Different initiation.
AK223139 mRNA. Translation: BAD96859.1.
BC002790 mRNA. Translation: AAH02790.3.
CCDSiCCDS10589.1. [Q53FZ2-1]
CCDS45435.1. [Q53FZ2-2]
PIRiI54401.
S69913.
RefSeqiNP_005613.2. NM_005622.3. [Q53FZ2-1]
NP_973729.1. NM_202000.2. [Q53FZ2-2]
UniGeneiHs.706754.

Genome annotation databases

EnsembliENST00000289416; ENSP00000289416; ENSG00000005187. [Q53FZ2-1]
ENST00000440284; ENSP00000394565; ENSG00000005187. [Q53FZ2-2]
GeneIDi6296.
KEGGihsa:6296.
UCSCiuc002dhq.4. human. [Q53FZ2-1]

Keywords - Coding sequence diversityi

Alternative splicing, Polymorphism

Cross-referencesi

Sequence databases

Select the link destinations:
EMBLi
GenBanki
DDBJi
Links Updated
D16350 mRNA. Translation: BAA03853.1. Different initiation.
X80062 mRNA. Translation: CAA56369.1. Different initiation.
AC004381 Genomic DNA. Translation: AAC31667.1. Different initiation.
AK223139 mRNA. Translation: BAD96859.1.
BC002790 mRNA. Translation: AAH02790.3.
CCDSiCCDS10589.1. [Q53FZ2-1]
CCDS45435.1. [Q53FZ2-2]
PIRiI54401.
S69913.
RefSeqiNP_005613.2. NM_005622.3. [Q53FZ2-1]
NP_973729.1. NM_202000.2. [Q53FZ2-2]
UniGeneiHs.706754.

3D structure databases

ProteinModelPortaliQ53FZ2.
SMRiQ53FZ2.
ModBaseiSearch...
MobiDBiSearch...

Protein-protein interaction databases

BioGridi112203. 1 interactor.
IntActiQ53FZ2. 1 interactor.
STRINGi9606.ENSP00000289416.

Chemistry databases

SwissLipidsiSLP:000001189.

PTM databases

iPTMnetiQ53FZ2.
PhosphoSitePlusiQ53FZ2.

Polymorphism and mutation databases

BioMutaiACSM3.
DMDMi158706483.

Proteomic databases

MaxQBiQ53FZ2.
PaxDbiQ53FZ2.
PeptideAtlasiQ53FZ2.
PRIDEiQ53FZ2.

Protocols and materials databases

DNASUi6296.
Structural Biology KnowledgebaseSearch...

Genome annotation databases

EnsembliENST00000289416; ENSP00000289416; ENSG00000005187. [Q53FZ2-1]
ENST00000440284; ENSP00000394565; ENSG00000005187. [Q53FZ2-2]
GeneIDi6296.
KEGGihsa:6296.
UCSCiuc002dhq.4. human. [Q53FZ2-1]

Organism-specific databases

CTDi6296.
DisGeNETi6296.
GeneCardsiACSM3.
H-InvDBHIX0079728.
HIX0079834.
HGNCiHGNC:10522. ACSM3.
HPAiHPA041013.
MalaCardsiACSM3.
MIMi145505. gene.
neXtProtiNX_Q53FZ2.
OpenTargetsiENSG00000005187.
PharmGKBiPA34930.
GenAtlasiSearch...

Phylogenomic databases

eggNOGiKOG1175. Eukaryota.
COG0365. LUCA.
GeneTreeiENSGT00760000119178.
HOGENOMiHOG000229982.
HOVERGENiHBG053031.
InParanoidiQ53FZ2.
KOiK01896.
OMAiAIPKAYV.
OrthoDBiEOG091G03NW.
PhylomeDBiQ53FZ2.
TreeFamiTF354287.

Enzyme and pathway databases

BioCyciZFISH:HS00131-MONOMER.
ReactomeiR-HSA-77352. Beta oxidation of butanoyl-CoA to acetyl-CoA.

Miscellaneous databases

ChiTaRSiACSM3. human.
GeneWikiiACSM3.
GenomeRNAii6296.
PROiQ53FZ2.
SOURCEiSearch...

Gene expression databases

BgeeiENSG00000005187.
CleanExiHS_ACSM3.
ExpressionAtlasiQ53FZ2. baseline and differential.
GenevisibleiQ53FZ2. 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 nameiACSM3_HUMAN
AccessioniPrimary (citable) accession number: Q53FZ2
Secondary accession number(s): O60363
, Q13732, Q15425, Q7KYM6, Q9BUA2
Entry historyi
Integrated into UniProtKB/Swiss-Prot: October 2, 2007
Last sequence update: October 2, 2007
Last modified: November 30, 2016
This is version 104 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

Caution

It is uncertain whether Met-1 or Met-9 is the initiator.Curated

Keywords - Technical termi

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. 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.