Gene
ADAMTS3
ADAM metallopeptidase with thrombospondin type 1 motif 3
- HGNC 219↗(opens in a new tab)
- Ensembl gene ENSG00000156140↗(opens in a new tab)
- NCBI Gene 9508↗(opens in a new tab)
Summary
- Cytogenetic location
- 4q13.3
- Coordinates
- Chromosome 4: 72,280,969–72,569,221 (GRCh38)
- Exons
- 22 exons across 288.3 kb
- Protein
- 1,205 amino acids
- Inheritance
- Autosomal recessive
- Also known as
- KIAA0366, ADAMTS-4
- MANE Select transcript
- ENST00000286657.10NM_014243.3
- 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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Primary Lymphoedema & Lymphatic Malformations
- Hennekam Syndrome Type 3Autosomal recessive
A condition caused by changes in ADAMTS3 that combines lymphoedema and swollen lymph vessels in the gut and other organs.
- 2 GenCC submitters rate it definitive or strong
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 ADAMTS3 is laid out
- Coding sequence
- Untranslated region
- Intron
- Direction of transcription
- 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 6.2 kb of the gene's 288.3 kb; 3,618 of those bases code for the protein.
Traits linked to ADAMTS3
The Human Phenotype Ontology lists these as features of the rare conditions ADAMTS3 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.
- 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.). ADAMTS3 gene: ADAM metallopeptidase with thrombospondin type 1 motif 3. Retrieved 16 October 2026, from https://dantelabs.com/genes/adamts3
Dante Labs. ADAMTS3 gene: ADAM metallopeptidase with thrombospondin type 1 motif 3 [Internet]. [cited 16 October 2026]. Available from: https://dantelabs.com/genes/adamts3
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