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FLASH GENE
Symbol LIAS contributors: mct - updated : 20-09-2017
HGNC name lipoic acid synthetase
HGNC id 16429
DNA
TYPE functioning gene
STRUCTURE 18.61 kb     11 Exon(s)
10 Kb 5' upstream gene genomic sequence study
MAPPING cloned Y linked N status provisional
RNA
TRANSCRIPTS type messenger
identificationnb exonstypebpproduct
ProteinkDaAAspecific expressionYearPubmed
11 - 1743 - 372 - 2001 11389890
10 - 1631 - 322 - 2001 11389890
10 - 1635 - 329 - 2001 11389890
4 - 702 - 142 - 2001 11389890
3 - 610 - 100 - 2001 11389890
EXPRESSION
Type
   expressed in (based on citations)
organ(s)
cell lineage
cell lines
fluid/secretion
at STAGE
PROTEIN
PHYSICAL PROPERTIES
STRUCTURE
motifs/domains
iron sulfur protein
conjugated MetalloP
HOMOLOGY
interspecies ortholog to murine Lias
homolog to yeast LIP-5
Homologene
FAMILY biotin and lipoic acid synthetase family
CATEGORY enzyme
SUBCELLULAR LOCALIZATION     intracellular
intracellular,cytoplasm,organelle,mitochondria,matrix
basic FUNCTION
  • involved in the synthesis of alpha-(+)-lipoic acid
  • may be involved in the sulfur insertion chemistry in lipoate biosynthesis
  • involved in the endogenous synthesis of lipoic acid, a potent mitochondrial antioxidant
  • in mitochondria, lipoic acid synthase produces alpha-lipoic acid, an antioxidant and an essential cofactor in alpha-ketoacid dehydrogenase complexes, which participate in glucose oxidation and ATP generation
  • lipoic acid is synthesized within the mitochondria by the enzyme, Lipoic acid synthase (LIAS)
  • LIPT2 and LIAS are involved in the lipoylation of GCS H protein
  • CELLULAR PROCESS
    PHYSIOLOGICAL PROCESS
    PATHWAY
    metabolism cofactor
    signaling
    lipoic acid biosynthesis
    a component
    INTERACTION
    DNA
    RNA
    small molecule
    protein
    cell & other
    REGULATION
    ASSOCIATED DISORDERS
    corresponding disease(s) LIASD
    Other morbid association(s)
    TypeGene ModificationChromosome rearrangementProtein expressionProtein Function
    constitutional       loss of function
    results in an overall disturbance in the antioxidant defense network, leading to increased inflammation, insulin resistance, and mitochondrial dysfunction
    Susceptibility
    Variant & Polymorphism
    Candidate gene
    Marker
    Therapy target
    ANIMAL & CELL MODELS
  • mitochondria in proximal tubular cells were particularly sensitive to damage in diabetic mice with reduced lipoic acid production