SEPSECS (Sep (O-phosphoserine) tRNA:Sec (selenocysteine) tRNA synthase)

2016-10-01  

Identity

HGNC
LOCATION
4p15.2
LOCUSID
ALIAS
LP,PCH2D,SLA,SLA/LP
FUSION GENES

Other Information

Locus ID:

NCBI: 51091
MIM: 613009
HGNC: 30605
Ensembl: ENSG00000109618

Variants:

dbSNP: 51091
ClinVar: 51091
TCGA: ENSG00000109618
COSMIC: SEPSECS

RNA/Proteins

Gene IDTranscript IDUniprot
ENSG00000109618ENST00000358971J3KP25
ENSG00000109618ENST00000382103Q9HD40
ENSG00000109618ENST00000503150H0Y9D2
ENSG00000109618ENST00000513285D6R969
ENSG00000109618ENST00000514585Q9HD40

Expression (GTEx)

0
5
10
15

Pathways

PathwaySourceExternal ID
Selenocompound metabolismKEGGko00450
Aminoacyl-tRNA biosynthesisKEGGko00970
Selenocompound metabolismKEGGhsa00450
Aminoacyl-tRNA biosynthesisKEGGhsa00970
MetabolismREACTOMER-HSA-1430728
Metabolism of amino acids and derivativesREACTOMER-HSA-71291
Selenoamino acid metabolismREACTOMER-HSA-2408522
Selenocysteine synthesisREACTOMER-HSA-2408557

References

Pubmed IDYearTitleCitations
377618922023Integrative Analysis Unveils the Correlation of Aminoacyl-tRNA Biosynthesis Metabolites with the Methylation of the SEPSECS Gene in Huntington's Disease Brain Tissue.1
377618922023Integrative Analysis Unveils the Correlation of Aminoacyl-tRNA Biosynthesis Metabolites with the Methylation of the SEPSECS Gene in Huntington's Disease Brain Tissue.1
350915082022Biallelic SEPSECS variants in two siblings with pontocerebellar hypoplasia type 2D underscore the relevance of splice-disrupting synonymous variants in disease.0
350915082022Biallelic SEPSECS variants in two siblings with pontocerebellar hypoplasia type 2D underscore the relevance of splice-disrupting synonymous variants in disease.0
348847332021Human Genetic Disorders Resulting in Systemic Selenoprotein Deficiency.14
348847332021Human Genetic Disorders Resulting in Systemic Selenoprotein Deficiency.14
281338632017Genetics and genotype-phenotype correlations in early onset epileptic encephalopathy with burst suppression.47
281338632017Genetics and genotype-phenotype correlations in early onset epileptic encephalopathy with burst suppression.47
268884822016Milder progressive cerebellar atrophy caused by biallelic SEPSECS mutations.10
275763442016Structural basis for early-onset neurological disorders caused by mutations in human selenocysteine synthase.11
268884822016Milder progressive cerebellar atrophy caused by biallelic SEPSECS mutations.10
275763442016Structural basis for early-onset neurological disorders caused by mutations in human selenocysteine synthase.11
261157352015Selenoprotein biosynthesis defect causes progressive encephalopathy with elevated lactate.23
261157352015Selenoprotein biosynthesis defect causes progressive encephalopathy with elevated lactate.23
251908122014Structural asymmetry of the terminal catalytic complex in selenocysteine synthesis.5

Citation

Dessen P

SEPSECS (Sep (O-phosphoserine) tRNA:Sec (selenocysteine) tRNA synthase)

Atlas Genet Cytogenet Oncol Haematol. 2016-10-01

Online version: http://atlasgeneticsoncology.org/gene/56393/