ADAM28

Chr 8

ADAM metallopeptidase domain 28

Also known as: ADAM 28, MDC-L, MDCL, eMDC II, eMDCII

The protein is a lymphocyte-expressed metalloprotease and disintegrin that mediates cell adhesion and proteolytic processing of surface proteins including FASL and CD40L, and may function in sperm maturation. ADAM28 mutations cause autosomal recessive cone-rod dystrophy, a retinal degeneration disorder affecting central vision. The gene shows low constraint to loss-of-function variation, consistent with recessive inheritance where one functional copy is insufficient.

Summary from RefSeq, UniProt
Research Assistant →
0
Active trials
12
Pubs (1 yr)
82
P/LP submissions
0%
P/LP missense
1.16
LOEUF
Multiple*
Mechanism· predicted
Clinical SummaryADAM28
Population Constraint (gnomAD)
Low constraint (pLI 0.00) — loss-of-function variants are relatively tolerated in the population.
📋
ClinVar Variants
82 unique Pathogenic / Likely Pathogenic· 114 VUS of 244 total submissions

Population Genetics & Constraint

gnomAD v4 — loss-of-function & missense intolerance

Tolerant — LoF & missense variants common in population
LoF Constraint
1.16LOEUF
pLI 0.000
Z-score 0.68
OE 0.89 (0.691.16)
Tolerant

Highly tolerant — LoF variants common in population

Missense Constraint
-0.92Z-score
OE missense 1.13 (1.041.22)
467 obs / 414.5 exp
Tolerant

Tolerant to missense variation

Observed / Expected Ratios
LoF OE0.89 (0.691.16)
00.351.4
Missense OE1.13 (1.041.22)
00.61.4
Synonymous OE1.05
01.21.6
LoF obs/exp: 39 / 43.8Missense obs/exp: 467 / 414.5Syn Z: -0.43
DN
0.7036th %ile
GOF
0.6540th %ile
LOF
0.2774th %ile

This gene has evidence for multiple mechanisms of pathogenicity (dominant-negative and gain-of-function). Both the Badonyi & Marsh prediction and the broader genomic evidence point to dominant-negative as the predominant mechanism. Different variants in this gene may act through different mechanisms — interpret in context of the specific variant.

DNprediction above median
GOFprediction above median

Note: In-silico variant effect predictors (SIFT, PolyPhen, REVEL, CADD) may underestimate pathogenicity of missense variants in genes with GOF or DN mechanisms. Consider functional evidence and clinical context.

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

ClinVar Variant Classifications

244 submitted variants in ClinVar

Classification Summary

Pathogenic78
Likely Pathogenic4
VUS114
Likely Benign12
Benign1
78
Pathogenic
4
Likely Pathogenic
114
VUS
12
Likely Benign
1
Benign

Curated Variants Distribution

Classified variants from ClinVar · 5 ACMG categories

ClassificationLoFMissense + InframeNon-codingSynonymousTotal
Pathogenic
0
0
78
0
78
Likely Pathogenic
0
0
4
0
4
VUS
0
109
5
0
114
Likely Benign
0
8
1
3
12
Benign
0
1
0
0
1
Total0118883209

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

View in ClinVar →

Protein Context — Lollipop Plot

ADAM28 · 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.

Search ClinicalTrials.gov →
Clinical Literature
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Recent Gene-Specific Literature
Gene in title · MEDLINE · newest first
Europe PMC