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Protein

3-oxoacyl-[acyl-carrier-protein] synthase, mitochondrial

Gene

OXSM

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

Functioni

May play a role in the biosynthesis of lipoic acid as well as longer chain fatty acids required for optimal mitochondrial function.1 Publication

Catalytic activityi

Acyl-[acyl-carrier-protein] + malonyl-[acyl-carrier-protein] = 3-oxoacyl-[acyl-carrier-protein] + CO2 + [acyl-carrier-protein].

Enzyme regulationi

Inhibited by cerulenin.1 Publication

Kineticsi

The highest catalytic efficiency is observed for C10-[acyl-carrier-protein].
  1. KM=3.9 µM for C4-[acyl-carrier-protein]1 Publication
  2. KM=1.9 µM for C6-[acyl-carrier-protein]1 Publication
  3. KM=10.9 µM for C8-[acyl-carrier-protein]1 Publication
  4. KM=1.8 µM for C10-[acyl-carrier-protein]1 Publication
  5. KM=9.5 µM for C12-[acyl-carrier-protein]1 Publication
  6. KM=50.8 µM for C14-[acyl-carrier-protein]1 Publication
  1. Vmax=129 nmol/min/mg enzyme toward C4-[acyl-carrier-protein]1 Publication
  2. Vmax=241 nmol/min/mg enzyme toward C6-[acyl-carrier-protein]1 Publication
  3. Vmax=271 nmol/min/mg enzyme toward C8-[acyl-carrier-protein]1 Publication
  4. Vmax=364 nmol/min/mg enzyme toward C10-[acyl-carrier-protein]1 Publication
  5. Vmax=1045 nmol/min/mg enzyme toward C12-[acyl-carrier-protein]1 Publication
  6. Vmax=115 nmol/min/mg enzyme toward C14-[acyl-carrier-protein]1 Publication

Pathwayi: fatty acid biosynthesis

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

GO - Molecular functioni

  • 3-oxoacyl-[acyl-carrier-protein] synthase activity Source: HGNC

GO - Biological processi

  • acyl-CoA metabolic process Source: HGNC
  • medium-chain fatty acid biosynthetic process Source: HGNC
  • short-chain fatty acid biosynthetic process Source: HGNC

Keywordsi

Molecular functionAcyltransferase, Transferase
Biological processFatty acid biosynthesis, Fatty acid metabolism, Lipid biosynthesis, Lipid metabolism

Enzyme and pathway databases

BRENDAi2.3.1.41. 2681.
SABIO-RKiQ9NWU1.
UniPathwayiUPA00094.

Chemistry databases

SwissLipidsiSLP:000001258.

Names & Taxonomyi

Protein namesi
Recommended name:
3-oxoacyl-[acyl-carrier-protein] synthase, mitochondrial (EC:2.3.1.41)
Alternative name(s):
Beta-ketoacyl-ACP synthase
Gene namesi
Name:OXSM
OrganismiHomo sapiens (Human)
Taxonomic identifieri9606 [NCBI]
Taxonomic lineageiEukaryotaMetazoaChordataCraniataVertebrataEuteleostomiMammaliaEutheriaEuarchontogliresPrimatesHaplorrhiniCatarrhiniHominidaeHomo
Proteomesi
  • UP000005640 Componenti: Chromosome 3

Organism-specific databases

EuPathDBiHostDB:ENSG00000151093.7.
HGNCiHGNC:26063. OXSM.

Subcellular locationi

Extracellular region or secreted Cytosol Plasma membrane Cytoskeleton Lysosome Endosome Peroxisome ER Golgi apparatus Nucleus Mitochondrion Manual annotation Automatic computational assertionGraphics by Christian Stolte; Source: COMPARTMENTS

Keywords - Cellular componenti

Mitochondrion

Pathology & Biotechi

Organism-specific databases

OpenTargetsiENSG00000151093.
PharmGKBiPA142671214.

Polymorphism and mutation databases

BioMutaiOXSM.
DMDMi74753030.

PTM / Processingi

Molecule processing

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Transit peptidei1 – 27MitochondrionSequence analysisAdd BLAST27
ChainiPRO_000023266028 – 4593-oxoacyl-[acyl-carrier-protein] synthase, mitochondrialAdd BLAST432

Amino acid modifications

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Modified residuei109N6-acetyllysine; alternateBy similarity1
Modified residuei109N6-succinyllysine; alternateBy similarity1
Modified residuei113N6-succinyllysineBy similarity1
Modified residuei174N6-acetyllysine; alternateCombined sources1
Modified residuei174N6-succinyllysine; alternateBy similarity1

Keywords - PTMi

Acetylation

Proteomic databases

EPDiQ9NWU1.
MaxQBiQ9NWU1.
PaxDbiQ9NWU1.
PeptideAtlasiQ9NWU1.
PRIDEiQ9NWU1.

PTM databases

iPTMnetiQ9NWU1.
PhosphoSitePlusiQ9NWU1.
SwissPalmiQ9NWU1.

Expressioni

Tissue specificityi

Widely expressed. Higher expression in heart, skeletal muscle, liver and kidney which contain high levels of active mitochondria.1 Publication

Gene expression databases

BgeeiENSG00000151093.
CleanExiHS_OXSM.
ExpressionAtlasiQ9NWU1. baseline and differential.
GenevisibleiQ9NWU1. HS.

Organism-specific databases

HPAiHPA021293.
HPA021300.
HPA021337.

Interactioni

Protein-protein interaction databases

BioGridi120328. 10 interactors.
IntActiQ9NWU1. 1 interactor.
STRINGi9606.ENSP00000280701.

Structurei

Secondary structure

1459
Legend: HelixTurnBeta strandPDB Structure known for this area
Show more details
Feature keyPosition(s)DescriptionActionsGraphical viewLength
Beta strandi38 – 41Combined sources4
Beta strandi44 – 53Combined sources10
Beta strandi56 – 58Combined sources3
Helixi59 – 67Combined sources9
Beta strandi73 – 75Combined sources3
Helixi79 – 81Combined sources3
Beta strandi88 – 90Combined sources3
Beta strandi94 – 97Combined sources4
Helixi103 – 105Combined sources3
Helixi109 – 112Combined sources4
Helixi117 – 133Combined sources17
Helixi140 – 144Combined sources5
Beta strandi146 – 153Combined sources8
Helixi157 – 170Combined sources14
Helixi172 – 174Combined sources3
Helixi179 – 182Combined sources4
Helixi187 – 196Combined sources10
Helixi208 – 210Combined sources3
Helixi211 – 225Combined sources15
Beta strandi229 – 237Combined sources9
Helixi242 – 250Combined sources9
Turni260 – 262Combined sources3
Beta strandi279 – 287Combined sources9
Helixi288 – 293Combined sources6
Beta strandi300 – 309Combined sources10
Beta strandi314 – 316Combined sources3
Helixi322 – 335Combined sources14
Helixi339 – 341Combined sources3
Beta strandi344 – 346Combined sources3
Helixi353 – 367Combined sources15
Helixi368 – 372Combined sources5
Beta strandi374 – 377Combined sources4
Helixi380 – 383Combined sources4
Helixi387 – 389Combined sources3
Helixi390 – 404Combined sources15
Beta strandi426 – 428Combined sources3
Beta strandi435 – 437Combined sources3
Beta strandi439 – 446Combined sources8
Turni447 – 449Combined sources3
Beta strandi450 – 457Combined sources8

3D structure databases

Select the link destinations:
PDBei
RCSB PDBi
PDBji
Links Updated
PDB entryMethodResolution (Å)ChainPositionsPDBsum
2C9HX-ray1.80A39-459[»]
2IWYX-ray2.06A/B38-459[»]
2IWZX-ray1.65A/B38-459[»]
ProteinModelPortaliQ9NWU1.
SMRiQ9NWU1.
ModBaseiSearch...
MobiDBiSearch...

Miscellaneous databases

EvolutionaryTraceiQ9NWU1.

Family & Domainsi

Sequence similaritiesi

Belongs to the beta-ketoacyl-ACP synthases family.Curated

Keywords - Domaini

Transit peptide

Phylogenomic databases

eggNOGiKOG1394. Eukaryota.
COG0304. LUCA.
GeneTreeiENSGT00530000063309.
HOGENOMiHOG000060166.
HOVERGENiHBG082096.
InParanoidiQ9NWU1.
KOiK09458.
OMAiPFFVPKI.
OrthoDBiEOG091G071V.
PhylomeDBiQ9NWU1.

Family and domain databases

Gene3Di3.40.47.10. 2 hits.
InterProiView protein in InterPro
IPR017568. 3-oxoacyl-ACP_synth-2.
IPR018201. Ketoacyl_synth_AS.
IPR014031. Ketoacyl_synth_C.
IPR014030. Ketoacyl_synth_N.
IPR020841. PKS_Beta-ketoAc_synthase_dom.
IPR016039. Thiolase-like.
PfamiView protein in Pfam
PF00109. ketoacyl-synt. 1 hit.
PF02801. Ketoacyl-synt_C. 1 hit.
PIRSFiPIRSF000447. KAS_II. 1 hit.
SMARTiView protein in SMART
SM00825. PKS_KS. 1 hit.
SUPFAMiSSF53901. SSF53901. 2 hits.
TIGRFAMsiTIGR03150. fabF. 1 hit.
PROSITEiView protein in PROSITE
PS00606. B_KETOACYL_SYNTHASE. 1 hit.

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: Q9NWU1-1) [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
MSNCLQNFLK ITSTRLLCSR LCQQLRSKRK FFGTVPISRL HRRVVITGIG
60 70 80 90 100
LVTPLGVGTH LVWDRLIGGE SGIVSLVGEE YKSIPCSVAA YVPRGSDEGQ
110 120 130 140 150
FNEQNFVSKS DIKSMSSPTI MAIGAAELAM KDSGWHPQSE ADQVATGVAI
160 170 180 190 200
GMGMIPLEVV SETALNFQTK GYNKVSPFFV PKILVNMAAG QVSIRYKLKG
210 220 230 240 250
PNHAVSTACT TGAHAVGDSF RFIAHGDADV MVAGGTDSCI SPLSLAGFSR
260 270 280 290 300
ARALSTNSDP KLACRPFHPK RDGFVMGEGA AVLVLEEYEH AVQRRARIYA
310 320 330 340 350
EVLGYGLSGD AGHITAPDPE GEGALRCMAA ALKDAGVQPE EISYINAHAT
360 370 380 390 400
STPLGDAAEN KAIKHLFKDH AYALAVSSTK GATGHLLGAA GAVEAAFTTL
410 420 430 440 450
ACYYQKLPPT LNLDCSEPEF DLNYVPLKAQ EWKTEKRFIG LTNSFGFGGT

NATLCIAGL
Length:459
Mass (Da):48,843
Last modified:October 1, 2000 - v1
Checksum:iB2EF550CD354F18C
GO
Isoform 2 (identifier: Q9NWU1-2) [UniParc]FASTAAdd to basket

The sequence of this isoform differs from the canonical sequence as follows:
     229-311: Missing.

Show »
Length:376
Mass (Da):40,035
Checksum:iB9470D089DE7CF7D
GO

Natural variant

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Natural variantiVAR_036064106F → I in a breast cancer sample; somatic mutation. 1 Publication1

Alternative sequence

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Alternative sequenceiVSP_041671229 – 311Missing in isoform 2. 1 PublicationAdd BLAST83

Sequence databases

Select the link destinations:
EMBLi
GenBanki
DDBJi
Links Updated
AK000611 mRNA. Translation: BAA91286.1.
AK225260 mRNA. No translation available.
AC092798 Genomic DNA. No translation available.
BC008202 mRNA. Translation: AAH08202.1.
CCDSiCCDS2643.1. [Q9NWU1-1]
CCDS46780.1. [Q9NWU1-2]
RefSeqiNP_001138863.1. NM_001145391.1. [Q9NWU1-2]
NP_060367.1. NM_017897.2. [Q9NWU1-1]
XP_006713279.1. XM_006713216.3. [Q9NWU1-1]
XP_016862202.1. XM_017006713.1. [Q9NWU1-1]
UniGeneiHs.55781.

Genome annotation databases

EnsembliENST00000280701; ENSP00000280701; ENSG00000151093. [Q9NWU1-1]
ENST00000420173; ENSP00000411303; ENSG00000151093. [Q9NWU1-2]
GeneIDi54995.
KEGGihsa:54995.
UCSCiuc003cdn.4. human. [Q9NWU1-1]

Keywords - Coding sequence diversityi

Alternative splicing, Polymorphism

Similar proteinsi

Entry informationi

Entry nameiOXSM_HUMAN
AccessioniPrimary (citable) accession number: Q9NWU1
Entry historyiIntegrated into UniProtKB/Swiss-Prot: April 18, 2006
Last sequence update: October 1, 2000
Last modified: September 27, 2017
This is version 137 of the entry and version 1 of the sequence. See 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 3
    Human chromosome 3: 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. PATHWAY comments
    Index of metabolic and biosynthesis pathways
  6. PDB cross-references
    Index of Protein Data Bank (PDB) cross-references
  7. SIMILARITY comments
    Index of protein domains and families