LIAS (lipoic acid synthetase)

2014-11-01  

Identity

HGNC
LOCATION
4p14
LOCUSID
ALIAS
HGCLAS,HUSSY-01,LAS,LIP1,LS,PDHLD
FUSION GENES

Other Information

Locus ID:

NCBI: 11019
MIM: 607031
HGNC: 16429
Ensembl: ENSG00000121897

Variants:

dbSNP: 11019
ClinVar: 11019
TCGA: ENSG00000121897
COSMIC: LIAS

RNA/Proteins

Gene IDTranscript IDUniprot
ENSG00000121897ENST00000261434A0A1X7SBR7
ENSG00000121897ENST00000340169O43766
ENSG00000121897ENST00000381846O43766
ENSG00000121897ENST00000424936Q6P5Q6
ENSG00000121897ENST00000509519B4E0L7
ENSG00000121897ENST00000513731D6RCP8
ENSG00000121897ENST00000638422A0A1W2PNQ5
ENSG00000121897ENST00000638430A0A1W2PQ02
ENSG00000121897ENST00000638451A0A1W2PQE9
ENSG00000121897ENST00000638816A0A1W2PR81
ENSG00000121897ENST00000638837A0A1W2PR40
ENSG00000121897ENST00000639422A0A1W2PQS9
ENSG00000121897ENST00000640349A0A1W2PPM2
ENSG00000121897ENST00000640489A0A1W2PRD2
ENSG00000121897ENST00000640672A0A1W2PRE7
ENSG00000121897ENST00000640689A0A1W2PQ87
ENSG00000121897ENST00000640888O43766
ENSG00000121897ENST00000640888A0A024R9W0

Expression (GTEx)

0
1
2
3
4
5
6
7
8

Pathways

PathwaySourceExternal ID
Lipoic acid metabolismKEGGko00785
Lipoic acid metabolismKEGGhsa00785
Metabolic pathwaysKEGGhsa01100
MetabolismREACTOMER-HSA-1430728
Metabolism of amino acids and derivativesREACTOMER-HSA-71291
Glyoxylate metabolism and glycine degradationREACTOMER-HSA-389661

Protein levels (Protein atlas)

Not detected
Low
Medium
High

References

Pubmed IDYearTitleCitations
374536612023FDX1 regulates cellular protein lipoylation through direct binding to LIAS.12
374536612023FDX1 regulates cellular protein lipoylation through direct binding to LIAS.12
335624932021Characterization and Reconstitution of Human Lipoyl Synthase (LIAS) Supports ISCA2 and ISCU as Primary Cluster Donors and an Ordered Mechanism of Cluster Assembly.11
335624932021Characterization and Reconstitution of Human Lipoyl Synthase (LIAS) Supports ISCA2 and ISCU as Primary Cluster Donors and an Ordered Mechanism of Cluster Assembly.11
277178432017Homology modeling of Homo sapiens lipoic acid synthase: Substrate docking and insights on its binding mode.5
277178432017Homology modeling of Homo sapiens lipoic acid synthase: Substrate docking and insights on its binding mode.5
279237732016Mitochondrial Protein Lipoylation and the 2-Oxoglutarate Dehydrogenase Complex Controls HIF1α Stability in Aerobic Conditions.83
279237732016Mitochondrial Protein Lipoylation and the 2-Oxoglutarate Dehydrogenase Complex Controls HIF1α Stability in Aerobic Conditions.83
261081462015Novel compound heterozygous LIAS mutations cause glycine encephalopathy.6
261081462015Novel compound heterozygous LIAS mutations cause glycine encephalopathy.6
243342902014Variant non ketotic hyperglycinemia is caused by mutations in LIAS, BOLA3 and the novel gene GLRX5.84
243342902014Variant non ketotic hyperglycinemia is caused by mutations in LIAS, BOLA3 and the novel gene GLRX5.84
221526802011Lipoic acid synthetase deficiency causes neonatal-onset epilepsy, defective mitochondrial energy metabolism, and glycine elevation.45
221526802011Lipoic acid synthetase deficiency causes neonatal-onset epilepsy, defective mitochondrial energy metabolism, and glycine elevation.45
208776242010Genetic variants in nuclear-encoded mitochondrial genes influence AIDS progression.20

Citation

Dessen P

LIAS (lipoic acid synthetase)

Atlas Genet Cytogenet Oncol Haematol. 2014-11-01

Online version: http://atlasgeneticsoncology.org/gene/65259/lias-(lipoic-acid-synthetase)