Gene/Proteome Database (LMPD)

LMPD ID
LMP000458
Gene ID
Species
Homo sapiens (Human)
Gene Name
3-oxoacyl-ACP synthase, mitochondrial
Gene Symbol
Synonyms
FASN2D; KASI; KS
Alternate Names
3-oxoacyl-[acyl-carrier-protein] synthase, mitochondrial; beta-ketoacyl synthase; beta-ketoacyl-ACP synthase; 3-oxoacyl-acyl-carrier-protein synthase; 3-ketoacyl-acyl carrier protein synthase; type II mitochondrial beta-ketoacyl synthase; acyl-malonyl acyl carrier protein-condensing enzyme
Chromosome
3
Map Location
3p24.2
EC Number
2.3.1.41
Summary
This gene encodes a beta-ketoacyl synthetase. The encoded enzyme is required for elongation of fatty acid chains in the mitochondria. Alternatively spliced transcript variants have been described.[provided by RefSeq, Feb 2009]
Orthologs

Proteins

3-oxoacyl-[acyl-carrier-protein] synthase, mitochondrial isoform 1 precursor
Refseq ID NP_060367
Protein GI 8923559
UniProt ID Q9NWU1
mRNA ID NM_017897
Length 459
RefSeq Status REVIEWED
MSNCLQNFLKITSTRLLCSRLCQQLRSKRKFFGTVPISRLHRRVVITGIGLVTPLGVGTHLVWDRLIGGESGIVSLVGEEYKSIPCSVAAYVPRGSDEGQFNEQNFVSKSDIKSMSSPTIMAIGAAELAMKDSGWHPQSEADQVATGVAIGMGMIPLEVVSETALNFQTKGYNKVSPFFVPKILVNMAAGQVSIRYKLKGPNHAVSTACTTGAHAVGDSFRFIAHGDADVMVAGGTDSCISPLSLAGFSRARALSTNSDPKLACRPFHPKRDGFVMGEGAAVLVLEEYEHAVQRRARIYAEVLGYGLSGDAGHITAPDPEGEGALRCMAAALKDAGVQPEEISYINAHATSTPLGDAAENKAIKHLFKDHAYALAVSSTKGATGHLLGAAGAVEAAFTTLACYYQKLPPTLNLDCSEPEFDLNYVPLKAQEWKTEKRFIGLTNSFGFGGTNATLCIAGL
3-oxoacyl-[acyl-carrier-protein] synthase, mitochondrial isoform 2 precursor
Refseq ID NP_001138863
Protein GI 223972627
UniProt ID Q9NWU1
mRNA ID NM_001145391
Length 376
RefSeq Status REVIEWED
MSNCLQNFLKITSTRLLCSRLCQQLRSKRKFFGTVPISRLHRRVVITGIGLVTPLGVGTHLVWDRLIGGESGIVSLVGEEYKSIPCSVAAYVPRGSDEGQFNEQNFVSKSDIKSMSSPTIMAIGAAELAMKDSGWHPQSEADQVATGVAIGMGMIPLEVVSETALNFQTKGYNKVSPFFVPKILVNMAAGQVSIRYKLKGPNHAVSTACTTGAHAVGDSFRFIAHGDAGHITAPDPEGEGALRCMAAALKDAGVQPEEISYINAHATSTPLGDAAENKAIKHLFKDHAYALAVSSTKGATGHLLGAAGAVEAAFTTLACYYQKLPPTLNLDCSEPEFDLNYVPLKAQEWKTEKRFIGLTNSFGFGGTNATLCIAGL

Gene Information

Entrez Gene ID
Gene Name
3-oxoacyl-ACP synthase, mitochondrial
Gene Symbol
Species
Homo sapiens

Gene Ontology (GO Annotations)

GO ID Source Type Description
GO:0005739 IDA:LIFEdb C mitochondrion
GO:0004315 IDA:HGNC F 3-oxoacyl-[acyl-carrier-protein] synthase activity
GO:0006637 IDA:HGNC P acyl-CoA metabolic process
GO:0051792 IDA:HGNC P medium-chain fatty acid biosynthetic process
GO:0051790 IDA:HGNC P short-chain fatty acid biosynthetic process

KEGG Pathway Links

KEGG Pathway ID Description
hsa00780 Biotin metabolism
hsa00061 Fatty acid biosynthesis
hsa01212 Fatty acid metabolism

Domain Information

InterPro Annotations

Accession Description
IPR017568 3-oxoacyl-[acyl-carrier-protein] synthase 2
IPR018201 Beta-ketoacyl synthase, active site
IPR014031 Beta-ketoacyl synthase, C-terminal
IPR014030 Beta-ketoacyl synthase, N-terminal
IPR016039 Thiolase-like
IPR016038 Thiolase-like, subgroup

UniProt Annotations

Entry Information

Gene Name
3-oxoacyl-ACP synthase, mitochondrial
Protein Entry
OXSM_HUMAN
UniProt ID
Species
Human

Comments

Comment Type Description
Alternative Products Event=Alternative splicing; Named isoforms=2; Name=1; IsoId=Q9NWU1-1; Sequence=Displayed; Name=2; IsoId=Q9NWU1-2; Sequence=VSP_041671;
Biophysicochemical Properties Kinetic parameters: KM=3.9 uM for C4-[acyl-carrier-protein] ; KM=1.9 uM for C6-[acyl-carrier-protein] ; KM=10.9 uM for C8-[acyl-carrier-protein] ; KM=1.8 uM for C10-[acyl-carrier-protein] ; KM=9.5 uM for C12-[acyl-carrier-protein] ; KM=50.8 uM for C14-[acyl-carrier-protein] ; Vmax=129 nmol/min/mg enzyme toward C4-[acyl-carrier-protein] ; Vmax=241 nmol/min/mg enzyme toward C6-[acyl-carrier-protein] ; Vmax=271 nmol/min/mg enzyme toward C8-[acyl-carrier-protein] ; Vmax=364 nmol/min/mg enzyme toward C10-[acyl-carrier-protein] ; Vmax=1045 nmol/min/mg enzyme toward C12-[acyl-carrier-protein] ; Vmax=115 nmol/min/mg enzyme toward C14-[acyl-carrier-protein] ; Note=The highest catalytic efficiency is observed for C10-[acyl- carrier-protein].;
Catalytic Activity Acyl-[acyl-carrier-protein] + malonyl-[acyl- carrier-protein] = 3-oxoacyl-[acyl-carrier-protein] + CO(2) + [acyl-carrier-protein].
Enzyme Regulation Inhibited by cerulenin.
Function May play a role in the biosynthesis of lipoic acid as well as longer chain fatty acids required for optimal mitochondrial function.
Pathway Lipid metabolism; fatty acid biosynthesis.
Similarity Belongs to the beta-ketoacyl-ACP synthases family.
Subcellular Location Mitochondrion .
Tissue Specificity Widely expressed. Higher expression in heart, skeletal muscle, liver and kidney which contain high levels of active mitochondria.

Identical and Related Proteins

Unique RefSeq proteins for LMP000458 (as displayed in Record Overview)

Protein GI Database Accession Length Protein Name
8923559 RefSeq NP_060367 459 3-oxoacyl-[acyl-carrier-protein] synthase, mitochondrial isoform 1 precursor
223972627 RefSeq NP_001138863 376 3-oxoacyl-[acyl-carrier-protein] synthase, mitochondrial isoform 2 precursor

Identical Sequences to LMP000458 proteins

Reference Database Accession Length Protein Name
GI:8923559 EMBL CBN70680.1 459 unnamed protein product [Homo sapiens]
GI:8923559 EMBL CBN69072.1 459 unnamed protein product [Homo sapiens]
GI:8923559 GenBank ADZ15386.1 459 3-oxoacyl-ACP synthase, mitochondrial, partial [synthetic construct]
GI:8923559 GenBank AED35427.1 459 Sequence 60 from patent US 7879989
GI:8923559 GenBank AIC51694.1 459 OXSM, partial [synthetic construct]
GI:8923559 RefSeq XP_006713279.1 459 PREDICTED: 3-oxoacyl-[acyl-carrier-protein] synthase, mitochondrial isoform X1 [Homo sapiens]

Related Sequences to LMP000458 proteins

Reference Database Accession Length Protein Name
GI:8923559 GenBank JAA02415.1 459 3-oxoacyl-ACP synthase, mitochondrial [Pan troglodytes]
GI:8923559 RefSeq XP_001164714.1 459 PREDICTED: 3-oxoacyl-[acyl-carrier-protein] synthase, mitochondrial [Pan troglodytes]
GI:8923559 RefSeq XP_003826203.1 459 PREDICTED: 3-oxoacyl-[acyl-carrier-protein] synthase, mitochondrial [Pan paniscus]
GI:223972627 RefSeq XP_004634253.1 376 PREDICTED: 3-oxoacyl-[acyl-carrier-protein] synthase, mitochondrial isoform X2 [Octodon degus]
GI:223972627 RefSeq XP_004754399.1 377 PREDICTED: 3-oxoacyl-[acyl-carrier-protein] synthase, mitochondrial isoform X3 [Mustela putorius furo]
GI:223972627 RefSeq XP_003480211.2 377 PREDICTED: 3-oxoacyl-[acyl-carrier-protein] synthase, mitochondrial isoform X1 [Cavia porcellus]
GI:223972627 RefSeq XP_005545684.1 377 PREDICTED: 3-oxoacyl-[acyl-carrier-protein] synthase, mitochondrial isoform X2 [Macaca fascicularis]
GI:223972627 RefSeq XP_006735948.1 376 PREDICTED: 3-oxoacyl-[acyl-carrier-protein] synthase, mitochondrial isoform X2 [Leptonychotes weddellii]
GI:223972627 RefSeq XP_008151886.1 378 PREDICTED: 3-oxoacyl-[acyl-carrier-protein] synthase, mitochondrial isoform X2 [Eptesicus fuscus]
GI:8923559 RefSeq XP_008949876.1 459 PREDICTED: 3-oxoacyl-[acyl-carrier-protein] synthase, mitochondrial [Pan paniscus]
GI:8923559 RefSeq XP_008949877.1 459 PREDICTED: 3-oxoacyl-[acyl-carrier-protein] synthase, mitochondrial [Pan paniscus]
GI:8923559 RefSeq XP_009443311.1 459 PREDICTED: 3-oxoacyl-[acyl-carrier-protein] synthase, mitochondrial [Pan troglodytes]