ADAMTS9

Chr 3

ADAM metallopeptidase with thrombospondin type 1 motif 9

This gene encodes a member of the ADAMTS (a disintegrin and metalloproteinase with thrombospondin motifs) protein family. Members of the family share several distinct protein modules, including a propeptide region, a metalloproteinase domain, a disintegrin-like domain, and a thrombospondin type 1 (TS) motif. Individual members of this family differ in the number of C-terminal TS motifs, and some have unique C-terminal domains. Members of the ADAMTS family have been implicated in the cleavage of proteoglycans, the control of organ shape during development, and the inhibition of angiogenesis. This gene is localized to chromosome 3p14.3-p14.2, an area known to be lost in hereditary renal tumors. Alternative splicing results in multiple transcript variants encoding different isoforms that may undergo similar proteolytic processing. [provided by RefSeq, Jan 2016]

ResearchGenerating clinical summary…
DNmechanismLOEUF 0.39
Clinical SummaryADAMTS9
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Gene-Disease Validity (ClinGen)
ciliopathy · ARLimited

Limited evidence — not for standalone diagnostic reporting

Population Constraint (gnomAD)
Constrained for loss-of-function variants (OE-LoF 0.29) despite low pLI — interpret in context.
Some data sources returned errors (1)

omim: Error: OMIM fetch failed: 429

Population Genetics & Constraint

gnomAD v4 — loss-of-function & missense intolerance

Moderate LoF intolerance
LoF Constraint?
0.39LOEUF
pLI 0.000
Z-score 7.13
OE 0.29 (0.220.39)
Moderately constrained

More LoF-intolerant than ~75% of genes

Missense Constraint?
0.95Z-score
OE missense 0.92 (0.870.97)
1030 obs / 1119.9 exp
Tolerant

Mild missense constraint

Observed / Expected Ratios?
LoF OE?0.29 (0.220.39)
00.351.4
Missense OE?0.92 (0.870.97)
00.61.4
Synonymous OE?1.19
01.21.6
LoF obs/exp: 35 / 118.9Missense obs/exp: 1030 / 1119.9Syn Z: -3.09
Curated Mechanism (G2P)Gene2Phenotype (DDG2P) ↗
strongADAMTS9-related nephronophthisis related ciliopathyOTHERAR

This gene — mechanism propensity

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

DN
0.6841th %ile
GOF
0.5366th %ile
LOF
0.3552th %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

ADAMTS9 · 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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