SMAD7

Chr 18

SMAD family member 7

Also known as: CRCS3, MADH7, MADH8

SMAD7 encodes a nuclear protein that antagonizes TGF-beta signaling by binding to TGF-beta receptors and preventing SMAD2 access, while also recruiting SMURF2 to promote receptor degradation. Mutations cause susceptibility to colorectal cancer type 3 with autosomal inheritance. The gene shows moderate constraint against loss-of-function variants (LOEUF 0.507), suggesting some intolerance to complete protein loss.

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

{Colorectal cancer, susceptibility to, 3}MIM #612229
UniProtColorectal cancer 3
1
Active trials
156
Pubs (1 yr)
41
P/LP submissions
0%
P/LP missense
0.51
LOEUF
LOF
Mechanism· predicted
Clinical SummarySMAD7
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Gene-Disease Validity (ClinGen)
congenital heart disease · ADLimited

Limited evidence — not for standalone diagnostic reporting

Population Constraint (gnomAD)
Moderately constrained gene (pLI 0.55) — some intolerance to loss-of-function variants.
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ClinVar Variants
39 unique Pathogenic / Likely Pathogenic· 64 VUS of 124 total submissions
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Clinical Trials
1 active or recruiting trial — potential therapeutic options may be available
📖
GeneReview available — SMAD7
Authoritative clinical overview · Recommended first read
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Population Genetics & Constraint

gnomAD v4 — loss-of-function & missense intolerance

Moderate LoF intolerance
LoF Constraint
0.51LOEUF
pLI 0.549
Z-score 2.91
OE 0.20 (0.090.51)
Moderately constrained

More LoF-intolerant than ~75% of genes

Missense Constraint
1.65Z-score
OE missense 0.69 (0.600.79)
154 obs / 223.5 exp
Tolerant

Mild missense constraint

Observed / Expected Ratios
LoF OE0.20 (0.090.51)
00.351.4
Missense OE0.69 (0.600.79)
00.61.4
Synonymous OE1.24
01.21.6
LoF obs/exp: 3 / 15.3Missense obs/exp: 154 / 223.5Syn Z: -1.97
DN
0.3097th %ile
GOF
0.2895th %ile
LOF
0.74top 10%

The highest-scoring mechanism for this gene is loss-of-function (haploinsufficiency).

LOFprediction above median

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

ClinVar Variant Classifications

124 submitted variants in ClinVar

Classification Summary

Pathogenic38
Likely Pathogenic1
VUS64
Likely Benign14
Benign5
38
Pathogenic
1
Likely Pathogenic
64
VUS
14
Likely Benign
5
Benign

Curated Variants Distribution

Classified variants from ClinVar · 5 ACMG categories

ClassificationLoFMissense + InframeNon-codingSynonymousTotal
Pathogenic
0
0
38
0
38
Likely Pathogenic
0
0
1
0
1
VUS
0
56
7
1
64
Likely Benign
0
5
4
5
14
Benign
0
1
0
4
5
Total0625010122

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

View in ClinVar →

Protein Context — Lollipop Plot

SMAD7 · protein map & ClinVar variants

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

Clinical Literature
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