Center for Bioinformatics
Oxidoreductases | Transferases | Hydrolases | Lyases | Isomerases | Ligases

Basic Information

Enzyme Number

2.3.1.50

Official Name

serine C-palmitoyltransferase

Name from literature

serine palmitoyltransferase

Pathway from literature

SM biosynthesis

Pathway from KEGG

Lipid Metabolism; Sphingolipid metabolism; map00600

Organisms

Human (9606)

Genome localization

9q22.2[10558 ], 14q24.3-q31[9517 ],

Comments

A pyridoxal-phosphate protein.

Rate-limiting Description

"Further investigation using a labeled substrate for serine palmitoyltransferase, the rate-limiting enzyme in the pathway, resulted in an accumulation of label in ceramide upon etoposide treatment." (10722759)

"In data obtained from intact cell radiolabeling studies, it was observed that the first and rate-limiting enzyme in de novo synthesis, serine palmitoyltransferase, is activated and controls the production of ceramide through this pathway." (10818445)

"Furthermore, the activity of serine palmitoyltransferase (SPT), the rate-limiting enzyme in sphingolipid synthesis, was increased in nicotinamide-treated cells." (10971324)

Regulatory Information

Regulatory type

Detail

transcriptional level;

"Although SPT is a housekeeping enzyme, its activity is regulated transcriptionally and post-transcriptionally, and its up-regulation is suggested to play a role in apoptosis induced by certain types of stress." (12782147)

post translational modification;

"Although SPT is a housekeeping enzyme, its activity is regulated transcriptionally and post-transcriptionally, and its up-regulation is suggested to play a role in apoptosis induced by certain types of stress." (12782147)

key enzyme;

"More specifically we demonstrated that ceramide accumulation induced by resveratrol can be traced to the activation of serine palmitoyltransferase (SPT), the key enzyme of de novo ceramide biosynthetic pathway" (14563682#9363775)

phosphorylation;

"Global, in vivo, and site-specific phosphorylation dynamics in signaling networks." (17081983)

phosphorylation;

O15269

phosphorylation;

O15270

phosphorylation;

O15270:from_uniprot:384_Phosphoserine

phosphorylation;

O15270:from_uniprot:387_Phosphotyrosine

Gene ontology

Gene ontology

GO:0030170 (F) pyridoxal phosphate binding [Q9NUV7, O15269, O15270 ];
GO:0016021 (C) integral to membrane [Q9NUV7, O15269, O15270 ];
GO:0009058 (P) biosynthetic process [Q9NUV7, O15269, O15270 ];
GO:0004758 (F) serine C-palmitoyltransferase activity [Q9NUV7, O15269, O15270 ];
GO:0016769 (F) transferase activity, transferring nitrogen... [Q9NUV7, O15269, O15270 ];
GO:0006665 (P) sphingolipid metabolic process [O15269 ];
GO:0005515 (F) protein binding [O15269 ];
GO:0005789 (C) endoplasmic reticulum membrane [Q9NUV7, O15269, O15270 ];

Tissue expression

Tissue From HPRD

Epidermis [05755 ];

Tissue specificity

Expressed in most tissues, except peripheral blood cells and bone marrow, with highest levels in heart, kidney, liver, uterus and skin [Q9NUV7 ];

Widely expressed [O15270 ];

Widely expressed. Not detected in small intestine [O15269 ];

Subcellular localization

Localization

endoplasmic reticulum [Q9NUV7, O15269, O15270 ];

Disease relevance

Disease

Defects in SPTLC1 are a cause of hereditary sensory neuropathy type 1 (HSN1) [MIM:162400]. HSN1 is an autosomal dominant progressive degeneration of dorsal root ganglia and motor neurons with onset in the second or third decades. Initial symptoms are sensory loss in the feet followed by distal muscle wasting and weakness. Loss of pain sensation leads to chronic skin ulcers and distal amputations. HSN1 is the most common hereditary disorder of peripheral sensory neurons [O15269 ];

Links

SwissProt

O15269; O15270; Q9NUV7

Entrez Gene

10558; 9517

HPRD

05754; 05755; 12765



  Copyright 2009, Center for Bioinformatics 
  Last Modified: 2009-03-24  
  Design by Zhao Min