Names & Taxonomy

Uniprot ID:
O15269
Entry Name:
SPTC1_HUMAN
Status:
reviewed
Protein Names:
Serine palmitoyltransferase 1 (EC 2.3.1.50) (Long chain base biosynthesis protein 1) (LCB 1) (Serine-palmitoyl-CoA transferase 1) (SPT 1) (SPT1)
Gene Names:
SPTLC1 LCB1
Gene Names Primary:
SPTLC1
Organism:
Homo sapiens (Human)

Structure

Length:
473
Sequence:
MATATEQWVLVEMVQALYEAPAYHLILEGILILWIIRLLFSKTYKLQERSDLTVKEKEELIEEWQPEPLVPPVPKDHPALNYNIVSGPPSHKTVVNGKECINFASFNFLGLLDNPRVKAAALASLKKYGVGTCGPRGFYGTFDVHLDLEDRLAKFMKTEEAIIYSYGFATIASAIPAYSKRGDIVFVDRAACFAIQKGLQASRSDIKLFKHNDMADLERLLKEQEIEDQKNPRKARVTRRFIVVEGLYMNTGTICPLPELVKLKYKYKARIFLEESLSFGVLGEHGRGVTEHYGINIDDIDLISANMENALASIGGFCCGRSFVIDHQRLSGQGYCFSASLPPLLAAAAIEALNIMEENPGIFAVLKEKCGQIHKALQGISGLKVVGESLSPAFHLQLEESTGSREQDVRLLQEIVDQCMNRSIALTQARYLEKEEKCLPPPSIRVVVTVEQTEEELERAASTIKEVAQAVLL
Proteomes:
UP000005640

Subcellular location

Subcellular Location:
Endoplasmic reticulum membrane

Function

Function:
Serine palmitoyltransferase (SPT). The heterodimer formed with SPTLC2 or SPTLC3 constitutes the catalytic core. The composition of the serine palmitoyltransferase (SPT) complex determines the substrate preference. The SPTLC1-SPTLC2-SPTSSA complex shows a strong preference for C16-CoA substrate, while the SPTLC1-SPTLC3-SPTSSA isozyme uses both C14-CoA and C16-CoA as substrates, with a slight preference for C14-CoA. The SPTLC1-SPTLC2-SPTSSB complex shows a strong preference for C18-CoA substrate, while the SPTLC1-SPTLC3-SPTSSB isozyme displays an ability to use a broader range of acyl-CoAs, without apparent preference.
Pathway:
Lipid metabolism; sphingolipid metabolism.
Catalytic Activity:
Palmitoyl-CoA + L-serine = CoA + 3-dehydro-D-sphinganine + CO(2).
Cofactor:
COFACTOR: Name=pyridoxal 5'-phosphate; Xref=ChEBI:CHEBI:597326;
Kinetics:
BIOPHYSICOCHEMICAL PROPERTIES: Kinetic parameters: KM=0.75 mM for serine
Cross Reference Drug Bank:
DB00133
Gene Ontology Go:
endoplasmic reticulum membrane
integral component of membrane
serine C-palmitoyltransferase complex
SPOTS complex
pyridoxal phosphate binding
serine C-palmitoyltransferase activity
ceramide biosynthetic process
positive regulation of lipophagy
small molecule metabolic process
sphinganine biosynthetic process
sphingolipid biosynthetic process
sphingolipid metabolic process
sphingomyelin biosynthetic process
sphingosine biosynthetic process
Gene Ontology Biological Process:
ceramide biosynthetic process
positive regulation of lipophagy
small molecule metabolic process
sphinganine biosynthetic process
sphingolipid biosynthetic process
sphingolipid metabolic process
sphingomyelin biosynthetic process
sphingosine biosynthetic process
Gene Ontology Molecular Function:
pyridoxal phosphate binding
serine C-palmitoyltransferase activity
Gene Ontology Cellular Component:
endoplasmic reticulum membrane
integral component of membrane
serine C-palmitoyltransferase complex
SPOTS complex
Keywords:
Acyltransferase
Alternative splicing
Complete proteome
Disease mutation
Endoplasmic reticulum
Lipid metabolism
Membrane
Neurodegeneration
Neuropathy
Phosphoprotein
Polymorphism
Pyridoxal phosphate
Reference proteome
Sphingolipid metabolism
Transferase
Transmembrane
Transmembrane helix

Publication

PubMed ID:
9363775 11242114 14702039 15164053 15489334 17023427 19416851 20504773 20182505 21269460 23629659 25944712 15037712 17060578 16959974 19651702 19132419 21618344 22302274 23454272 24247255