ADAMTSL2

Chr 9AR

ADAMTS like 2

Also known as: ADAMTSL-2, GPHYSD1

The protein is a secreted glycoprotein that binds to the cell surface and extracellular matrix and interacts with latent transforming growth factor beta binding protein 1. Mutations cause geleophysic dysplasia 1, a skeletal dysplasia with characteristic facial features, short stature, and cardiac abnormalities that typically presents in early childhood. Inheritance is autosomal recessive.

OMIMResearchSummary from RefSeq, OMIM
LOFmechanismARLOEUF 0.731 OMIM phenotype
Clinical SummaryADAMTSL2
Population Constraint (gnomAD)
Low constraint (pLI 0.00) — loss-of-function variants are relatively tolerated in the population.

Population Genetics & Constraint

gnomAD v4 — loss-of-function & missense intolerance

Tolerant — LoF & missense variants common in population
LoF Constraint
0.73LOEUF
pLI 0.002
Z-score 2.43
OE 0.41 (0.240.73)
Tolerant

Typical tolerance to LoF variation

Missense Constraint
1.25Z-score
OE missense 0.77 (0.690.87)
185 obs / 239.3 exp
Tolerant

Mild missense constraint

Observed / Expected Ratios
LoF OE0.41 (0.240.73)
00.351.4
Missense OE0.77 (0.690.87)
00.61.4
Synonymous OE1.22
01.21.6
LoF obs/exp: 8 / 19.6Missense obs/exp: 185 / 239.3Syn Z: -1.83
Curated Mechanism (G2P)Gene2Phenotype (DDG2P) ↗
definitiveADAMTSL2-related geleophysic dysplasiaLOFAR

Predictions shown for reference only — model trained on dominant genes, not applicable to AR conditions.

DN
0.6937th %ile
GOF
0.5954th %ile
LOF
0.3454th %ile

The Badonyi & Marsh prediction model was trained exclusively on dominant disease genes. Predictions are not reliable for genes with autosomal recessive inheritance and are shown at reduced opacity for reference only.

Predictions from Badonyi M, Marsh JA. PLoS ONE. 2024;19(8):e0307312.

ClinVar Variant Classifications

0 submitted variants in ClinVar

Protein Context — Lollipop Plot

ADAMTSL2 · protein map & ClinVar variants

Showing all ClinVar variants across the protein. Search a specific variant to highlight its position.

3D Protein StructureAlphaFold

Clinical Trials

Active and recruiting trials from ClinicalTrials.gov

No active trials found for this gene.

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Clinical Literature
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