MUTYH

Chr 1

mutY DNA glycosylase

Also known as: MYH

This gene encodes a DNA glycosylase involved in oxidative DNA damage repair. The enzyme excises adenine bases from the DNA backbone at sites where adenine is inappropriately paired with guanine, cytosine, or 8-oxo-7,8-dihydroguanine, a major oxidatively damaged DNA lesion. The protein is localized to the nucleus and mitochondria. This gene product is thought to play a role in signaling apoptosis by the introduction of single-strand breaks following oxidative damage. Mutations in this gene result in heritable predisposition to colorectal cancer, termed MUTYH-associated polyposis (MAP). Multiple transcript variants encoding different isoforms have been found for this gene. [provided by RefSeq, Apr 2017]

GeneReviewsResearchGenerating clinical summary…

Primary Disease Associations & Inheritance

UniProtFamilial adenomatous polyposis 2
UniProtGastric cancer

Clinical highlights

Gene-disease validity (ClinGen)
familial ovarian cancer · ARDisputedevidence questions this relationship6 gene-disease associations curated in total
Interpreting a novel variant
Loss of function is the curated mechanism (Gene2Phenotype), though the gene is not strongly LoF-constrained in the population — weigh truncating variants against that tolerance.Curated gene-level mechanism — a prior for triage, not a per-variant call.
3
Active trials
144
Pubs (1 yr)
P/LP submissions
P/LP missense
1.19
LOEUF
LOF
Mechanism· G2P
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GeneReview available — MUTYH
Authoritative clinical overview · Recommended first read
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Some data sources returned errors (1)

omim: Error: OMIM fetch failed: 429

Population Genetics & Constraint

gnomAD v4 — loss-of-function & missense intolerance

Tolerant — LoF & missense variants common in population
LoF Constraint?
1.19LOEUF
pLI 0.000
Z-score 0.66
OE 0.88 (0.661.19)
Tolerant

Highly tolerant — LoF variants common in population

Missense Constraint?
-0.21Z-score
OE missense 1.03 (0.941.13)
322 obs / 311.7 exp
Tolerant

Tolerant to missense variation

Observed / Expected Ratios?
LoF OE?0.88 (0.661.19)
00.351.4
Missense OE?1.03 (0.941.13)
00.61.4
Synonymous OE?1.01
01.21.6
LoF obs/exp: 30 / 34.2Missense obs/exp: 322 / 311.7Syn Z: -0.13

ClinVar Variant Classifications

0 submitted variants in ClinVar

Protein Context — Lollipop Plot

MUTYH · protein map & ClinVar variants

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