ADAMTS15

Chr 11AR

ADAM metallopeptidase with thrombospondin type 1 motif 15

Also known as: DA12

The ADAMTS15 protein is a metalloprotease that cleaves the proteoglycan versican in the pericellular matrix surrounding myoblasts, facilitating myoblast contact and fusion required for skeletal muscle development and regeneration. Mutations cause distal arthrogryposis type 12, a condition affecting joint mobility primarily in the hands and feet, inherited in an autosomal recessive pattern. The gene is highly constrained against loss-of-function variants (pLI near 1.0), indicating intolerance to protein-truncating mutations.

Summary from RefSeq, OMIM, UniProt
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Primary Disease Associations & Inheritance

Arthrogryposis, distal, type 12MIM #620545
AR
0
Active trials
2
Pubs (1 yr)
24
P/LP submissions
9%
P/LP missense
0.72
LOEUF
DN
Mechanism· predicted
Clinical SummaryADAMTS15
Population Constraint (gnomAD)
Low constraint (pLI 0.00) — loss-of-function variants are relatively tolerated in the population.
📋
ClinVar Variants
23 unique Pathogenic / Likely Pathogenic· 69 VUS of 100 total submissions

Population Genetics & Constraint

gnomAD v4 — loss-of-function & missense intolerance

Tolerant — LoF & missense variants common in population
LoF Constraint
0.72LOEUF
pLI 0.000
Z-score 2.80
OE 0.47 (0.310.72)
Tolerant

Typical tolerance to LoF variation

Missense Constraint
0.51Z-score
OE missense 0.94 (0.881.01)
553 obs / 587.7 exp
Tolerant

Mild missense constraint

Observed / Expected Ratios
LoF OE0.47 (0.310.72)
00.351.4
Missense OE0.94 (0.881.01)
00.61.4
Synonymous OE1.05
01.21.6
LoF obs/exp: 15 / 32.1Missense obs/exp: 553 / 587.7Syn Z: -0.68
Curated Mechanism (G2P)Gene2Phenotype (DDG2P) ↗
moderateADAMTS15-related distal arthrogryposisOTHERAR

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

DN
0.7133th %ile
GOF
0.6151th %ile
LOF
0.3066th %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

100 submitted variants in ClinVar

Classification Summary

Pathogenic18
Likely Pathogenic5
VUS69
Likely Benign4
Benign4
18
Pathogenic
5
Likely Pathogenic
69
VUS
4
Likely Benign
4
Benign

Curated Variants Distribution

Classified variants from ClinVar · 5 ACMG categories

ClassificationLoFMissense + InframeNon-codingSynonymousTotal
Pathogenic
1
1
16
0
18
Likely Pathogenic
0
1
4
0
5
VUS
0
68
1
0
69
Likely Benign
0
3
0
1
4
Benign
0
1
0
3
4
Total174214100

LoF = frameshift, stop gained/lost, canonical splice · Counts from ClinVar esearch · Updated hourly

View in ClinVar →

Protein Context — Lollipop Plot

ADAMTS15 · protein map & ClinVar variants

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

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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