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MDDGA1

Chr 9AR

protein O-mannosyltransferase 1

Also known as: LGMD2K, LGMDR11, MDDGA1, MDDGB1, MDDGC1, RT

The protein encoded by MDDGA1 is an O-mannosyltransferase that functions in the endoplasmic reticulum membrane and requires interaction with POMT2 for enzymatic activity. Mutations cause muscular dystrophy-dystroglycanopathy with congenital brain and eye anomalies (including Walker-Warburg syndrome), affecting muscle, brain, and ocular development. This condition follows autosomal recessive inheritance.

OMIMResearchSummary from RefSeq, OMIM
AR1 OMIM phenotype
Some data sources returned errors (2)

ensembl: Error: Ensembl fetch failed: 400 for https://rest.ensembl.org/lookup/symbol/homo_sapiens/MDDGA1?content-type=application/json&expand=1

gnomad: Error: Gene not found

Population Genetics & Constraint

Constraint data not available from gnomAD.

ClinVar Variant Classifications

0 submitted variants in ClinVar

Protein Context — Lollipop Plot

MDDGA1 · 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

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Clinical Literature
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Recent Gene-Specific Literature
Gene in title · MEDLINE · newest first
Europe PMC

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