Details for: SPTLC1

Gene ID: 10558

Symbol: SPTLC1

Ensembl ID: ENSG00000090054

Description: serine palmitoyltransferase long chain base subunit 1

Associated with

Other Information

Genular Protein ID: 1362049628

Symbol: SPTC1_HUMAN

Name: Serine palmitoyltransferase 1

UniProtKB Accession Codes:

Database IDs:

Citations:

PubMed ID: 9363775

Title: Human and murine serine-palmitoyl-CoA transferase. Cloning, expression and characterization of the key enzyme in sphingolipid synthesis.

PubMed ID: 9363775

DOI: 10.1111/j.1432-1033.1997.00239.x

PubMed ID: 11242114

Title: Mutations in SPTLC1, encoding serine palmitoyltransferase, long chain base subunit-1, cause hereditary sensory neuropathy type I.

PubMed ID: 11242114

DOI: 10.1038/85879

PubMed ID: 14702039

Title: Complete sequencing and characterization of 21,243 full-length human cDNAs.

PubMed ID: 14702039

DOI: 10.1038/ng1285

PubMed ID: 15164053

Title: DNA sequence and analysis of human chromosome 9.

PubMed ID: 15164053

DOI: 10.1038/nature02465

PubMed ID: 15489334

Title: The status, quality, and expansion of the NIH full-length cDNA project: the Mammalian Gene Collection (MGC).

PubMed ID: 15489334

DOI: 10.1101/gr.2596504

PubMed ID: 17023427

Title: Cloning and initial characterization of a new subunit for mammalian serine-palmitoyltransferase.

PubMed ID: 17023427

DOI: 10.1074/jbc.m608066200

PubMed ID: 19648650

Title: The SPTLC3 subunit of serine palmitoyltransferase generates short chain sphingoid bases.

PubMed ID: 19648650

DOI: 10.1074/jbc.m109.023192

PubMed ID: 19416851

Title: Identification of small subunits of mammalian serine palmitoyltransferase that confer distinct acyl-CoA substrate specificities.

PubMed ID: 19416851

DOI: 10.1073/pnas.0811269106

PubMed ID: 20504773

Title: A disease-causing mutation in the active site of serine palmitoyltransferase causes catalytic promiscuity.

PubMed ID: 20504773

DOI: 10.1074/jbc.m110.122259

PubMed ID: 20182505

Title: Orm family proteins mediate sphingolipid homeostasis.

PubMed ID: 20182505

DOI: 10.1038/nature08787

PubMed ID: 21269460

Title: Initial characterization of the human central proteome.

PubMed ID: 21269460

DOI: 10.1186/1752-0509-5-17

PubMed ID: 21994399

Title: MicroRNA-137/181c regulates serine palmitoyltransferase and in turn amyloid beta, novel targets in sporadic Alzheimer's disease.

PubMed ID: 21994399

DOI: 10.1523/jneurosci.3883-11.2011

PubMed ID: 23629659

Title: Phosphorylation of serine palmitoyltransferase long chain-1 (SPTLC1) on tyrosine 164 inhibits its activity and promotes cell survival.

PubMed ID: 23629659

DOI: 10.1074/jbc.m112.409185

PubMed ID: 25944712

Title: N-terminome analysis of the human mitochondrial proteome.

PubMed ID: 25944712

DOI: 10.1002/pmic.201400617

PubMed ID: 30700557

Title: The ORMDL/Orm-serine palmitoyltransferase (SPT) complex is directly regulated by ceramide: Reconstitution of SPT regulation in isolated membranes.

PubMed ID: 30700557

DOI: 10.1074/jbc.ra118.007291

PubMed ID: 33558761

Title: Structural insights into the regulation of human serine palmitoyltransferase complexes.

PubMed ID: 33558761

DOI: 10.1038/s41594-020-00551-9

PubMed ID: 33558762

Title: Structural insights into the assembly and substrate selectivity of human SPT-ORMDL3 complex.

PubMed ID: 33558762

DOI: 10.1038/s41594-020-00553-7

PubMed ID: 37308477

Title: Ceramide sensing by human SPT-ORMDL complex for establishing sphingolipid homeostasis.

PubMed ID: 37308477

DOI: 10.1038/s41467-023-39274-y

PubMed ID: 15037712

Title: SPTLC1 mutation in twin sisters with hereditary sensory neuropathy type I.

PubMed ID: 15037712

DOI: 10.1212/01.wnl.0000115388.10828.5c

PubMed ID: 17060578

Title: Charcot-Marie-Tooth disease due to a de novo mutation of the RAB7 gene.

PubMed ID: 17060578

DOI: 10.1212/01.wnl.0000240068.21499.f5

PubMed ID: 16959974

Title: The consensus coding sequences of human breast and colorectal cancers.

PubMed ID: 16959974

DOI: 10.1126/science.1133427

PubMed ID: 19651702

Title: Genes for hereditary sensory and autonomic neuropathies: a genotype-phenotype correlation.

PubMed ID: 19651702

DOI: 10.1093/brain/awp198

PubMed ID: 19132419

Title: A systematic comparison of all mutations in hereditary sensory neuropathy type I (HSAN I) reveals that the G387A mutation is not disease associated.

PubMed ID: 19132419

DOI: 10.1007/s10048-008-0168-7

PubMed ID: 21618344

Title: Characterization of two mutations in the SPTLC1 subunit of serine palmitoyltransferase associated with hereditary sensory and autonomic neuropathy type I.

PubMed ID: 21618344

DOI: 10.1002/humu.21481

PubMed ID: 22302274

Title: Frequency of mutations in the genes associated with hereditary sensory and autonomic neuropathy in a UK cohort.

PubMed ID: 22302274

DOI: 10.1007/s00415-011-6397-y

PubMed ID: 23454272

Title: Mutations at Ser331 in the HSN type I gene SPTLC1 are associated with a distinct syndromic phenotype.

PubMed ID: 23454272

DOI: 10.1016/j.ejmg.2013.02.002

PubMed ID: 24247255

Title: Early-onset severe hereditary sensory and autonomic neuropathy type 1 with S331F SPTLC1 mutation.

PubMed ID: 24247255

DOI: 10.3892/mmr.2013.1808

PubMed ID: 30420926

Title: V144D Mutation of SPTLC1 Can Present with Both Painful and Painless Phenotypes in Hereditary Sensory and Autonomic Neuropathies Type I.

PubMed ID: 30420926

DOI: 10.1155/2018/1898151

PubMed ID: 34459874

Title: Association of Variants in the SPTLC1 Gene With Juvenile Amyotrophic Lateral Sclerosis.

PubMed ID: 34459874

DOI: 10.1001/jamaneurol.2021.2598

PubMed ID: 34059824

Title: Childhood amyotrophic lateral sclerosis caused by excess sphingolipid synthesis.

PubMed ID: 34059824

DOI: 10.1038/s41591-021-01346-1

PubMed ID: 36204986

Title: A de novo c.113 T > C: p.L38R mutation of SPTLC1: case report of a girl with sporadic juvenile amyotrophic lateral sclerosis.

PubMed ID: 36204986

DOI: 10.1080/21678421.2022.2096409

Sequence Information:

  • Length: 473
  • Mass: 52744
  • Checksum: BA9E056A869D2EA2
  • Sequence:
  • MATATEQWVL VEMVQALYEA PAYHLILEGI LILWIIRLLF SKTYKLQERS DLTVKEKEEL 
    IEEWQPEPLV PPVPKDHPAL NYNIVSGPPS HKTVVNGKEC INFASFNFLG LLDNPRVKAA 
    ALASLKKYGV GTCGPRGFYG TFDVHLDLED RLAKFMKTEE AIIYSYGFAT IASAIPAYSK 
    RGDIVFVDRA ACFAIQKGLQ ASRSDIKLFK HNDMADLERL LKEQEIEDQK NPRKARVTRR 
    FIVVEGLYMN TGTICPLPEL VKLKYKYKAR IFLEESLSFG VLGEHGRGVT EHYGINIDDI 
    DLISANMENA LASIGGFCCG RSFVIDHQRL SGQGYCFSAS LPPLLAAAAI EALNIMEENP 
    GIFAVLKEKC GQIHKALQGI SGLKVVGESL SPAFHLQLEE STGSREQDVR LLQEIVDQCM 
    NRSIALTQAR YLEKEEKCLP PPSIRVVVTV EQTEEELERA ASTIKEVAQA VLL

Genular Protein ID: 2236438418

Symbol: Q6NUL7_HUMAN

Name: N/A

UniProtKB Accession Codes:

Database IDs:

Citations:

PubMed ID: 15489334

Title: The status, quality, and expansion of the NIH full-length cDNA project: the Mammalian Gene Collection (MGC).

PubMed ID: 15489334

DOI: 10.1101/gr.2596504

Sequence Information:

  • Length: 513
  • Mass: 57396
  • Checksum: F8870EF1B008FEC3
  • Sequence:
  • MATATEQWVL VEMVQALYEA PAYHLILEGI LILWIIRLLF SKTYKLQERS DLTVKEKEEL 
    IEEWQPEPLV PPVPKDHPAL NYNIVSGPPS HKTVVNGKEC INFASFNFLG LLDNPRVKAA 
    ALASLKKYGV GTCGPRGFYG TFDVHLDLED RLAKFMKTEE AIIYSYGFAT IASAIPAYSK 
    RGDIVFVDRA ACFAIQKGLQ ASRSDIKLFK HNDMADLERL LKEQEIEDQK NPRKARVTRR 
    FIVVEGLYMN TGTICPLPEL VKLKYKYKAR IFLEESLSFG VLGEHGRGVT EHYGINIDDI 
    DLISANMENA LASIGGFCCG RSFVIDHQRL SGQGYCFSAS LPPLLAAAAI EALNIMEENP 
    GIFAVLKEKC GQIHKALQGI SGLKVVGESL SPAFHLQLEE STGSREQDVR LLQEIVDQCM 
    NRSIALTRAR YLEKEEKCLP PPRGRTGESC VHHQGGSPGR PALGRVPGPW PPATQHAERT 
    QDSRWPWSGL KESKNMWIFD RIVTKWCQYG PIV

Database document:

This is a preview of the gene's schema. Only a few entries are kept for 'singleCellExpressions,' 'mRNAExpressions,' and other large data arrays for visualization purposes. You can zoom in with the mouse wheel for a closer view, and the text will adjust automatically if necessary. For the full schema, download it here.