Gene
LIAS
lipoic acid synthetase
- HGNC 16429(opens in a new tab)
- Ensembl gene ENSG00000121897(opens in a new tab)
- NCBI Gene 11019(opens in a new tab)
Summary
- Cytogenetic location
- 4p14
- Coordinates
- Chromosome 4: 39,459,056–39,479,506 (GRCh38)
- Exons
- 11 exons across 20.5 kb
- Protein
- 372 amino acids
- Inheritance
- Autosomal recessive
- Also known as
- LAS
- MANE Select transcript
- ENST00000640888.2NM_006859.4
- Locus type
- gene with protein product
- Very high
- Confident
- Low
- Very low
Reference genomeGRCh38hg38
Positions on gene and variant pages follow this choice, in this browser.
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Pyruvate Dehydrogenase & Pyruvate Carboxylase Deficiency
- Pyruvate Dehydrogenase DeficiencyAutosomal recessive
Caused by variants in PDHA1 (X-linked, often new in the child) or in PDHB, DLAT, PDHX, DLD, PDP1 or LIAS, which can cause lactic acidosis, developmental delay, seizures and movement problems.
- 4 GenCC submitters rate it definitive or strong
What knowing changes
Knowing allows a ketogenic diet, avoidance of high-carbohydrate loads, and thiamine for responsive forms.
Inheritance
- Autosomal recessive
- It takes a changed copy from each parent. With one, you are a carrier and usually unaffected; when both parents are carriers, each child has a one-in-four chance of inheriting two.
How LIAS is laid out
- Coding sequence
- Untranslated region
- Intron
- Direction of transcription
- Known disease-causing sites
- Stretches short reads find hard
Exons of the gene's MANE Select transcript (release v1.5), from NCBI and EMBL-EBI, on the GRCh38 reference genome.
Chromosome bands from UCSC's GRCh38 ideogram (2022-10-29).
Nearby genes from Ensembl release 116, on GRCh38.
Exons make up 3.6 kb of the gene's 20.5 kb; 1,119 of those bases code for the protein.
How a genome test reads LIAS
26
positions in LIAS where ClinVar classes a change as pathogenic or likely pathogenic
None of them falls in a region short reads find hard.
A whole genome covers all 20.5 kb of LIAS, introns included, not only the 3.6 kb of exons.
Traits linked to LIAS
The Human Phenotype Ontology lists these as features of the rare conditions LIAS can cause. Each trait's page lists the other genes linked to it.
External links
Gene records
Transcript
Protein
Where this comes from
- HGNC: the gene's symbol, name, other symbols and location.
- MANE Select v1.5, from NCBI and EMBL-EBI: the transcript, its exons and coding sequence.
- GenCC and ClinGen, released 2026-10-16: which conditions the gene is linked to, and how strongly.
- The ACMG secondary findings list, SF v3.2.
- ClinVar and the GIAB stratifications: known disease-causing sites, and the regions short reads find hard.
- The Human Phenotype Ontology, release 2026-09-01: the traits linked to the gene. Find out more at hpo.jax.org.
- Data release 2026.10.16-c6eea6bf.
Cite this page
Dante Labs. (n.d.). LIAS gene: lipoic acid synthetase. Retrieved 17 October 2026, from https://dantelabs.com/genes/lias
Dante Labs. LIAS gene: lipoic acid synthetase [Internet]. [cited 17 October 2026]. Available from: https://dantelabs.com/genes/lias
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