ADAM23

Chr 2

ADAM metallopeptidase domain 23

Also known as: MDC-3, MDC3

ADAM23 encodes a non-catalytic membrane-anchored metalloprotease involved in cell-cell and cell-matrix interactions during neurogenesis and muscle development. Mutations cause autosomal recessive intellectual disability with seizures and brain malformations. The phenotype involves the central nervous system with early childhood onset of developmental delays and epilepsy.

OMIMResearchSummary from RefSeq, UniProt
LOEUF 0.28
Clinical SummaryADAM23
Population Constraint (gnomAD)
Highly constrained gene — heterozygous loss-of-function variants are very rare in the population (pLI 1.00). One damaged copy is likely sufficient to cause disease.

Population Genetics & Constraint

gnomAD v4 — loss-of-function & missense intolerance

LoF intolerant — likely haploinsufficient
LoF Constraint
0.28LOEUF
pLI 0.997
Z-score 5.69
OE 0.15 (0.090.28)
Highly constrained

Highly LoF-intolerant (top ~10% of genes)

Missense Constraint
1.90Z-score
OE missense 0.75 (0.680.82)
329 obs / 441.3 exp
Tolerant

Mild missense constraint

Observed / Expected Ratios
LoF OE0.15 (0.090.28)
00.351.4
Missense OE0.75 (0.680.82)
00.61.4
Synonymous OE0.75
01.21.6
LoF obs/exp: 8 / 52.4Missense obs/exp: 329 / 441.3Syn Z: 2.45

ClinVar Variant Classifications

0 submitted variants in ClinVar

Protein Context — Lollipop Plot

ADAM23 · 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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