MDDGA1

Chr 9AR

protein O-mannosyltransferase 1

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

The protein encoded by this gene is an O-mannosyltransferase that requires interaction with the product of the POMT2 gene for enzymatic function. The encoded protein is found in the membrane of the endoplasmic reticulum. Defects in this gene are a cause of Walker-Warburg syndrome (WWS) and limb-girdle muscular dystrophy type 2K (LGMD2K). Several transcript variants encoding different isoforms have been found for this gene.[provided by RefSeq, Oct 2008]

Primary Disease Associations & Inheritance

Muscular dystrophy-dystroglycanopathy (congenital with brain and eye anomalies), type A, 1MIM #236670
AR
0
ClinVar variants
0
Pathogenic / LP
pLI score
0
Active trials
Clinical SummaryMDDGA1
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.

OMIM — Genotype-Phenotype Relationships

1 OMIM entry

Muscular dystrophy-dystroglycanopathy (congenital with brain and eye anomalies), type A, 1

MIM #236670

Molecular basis of disorder known

Autosomal recessive
Clinical Literature
Landmark / reviewRecent case evidence
Key Publications
Landmark & review papers · by relevance
PubMed
Syndromic Hydrocephalus.
Varagur K et al.·Neurosurg Clin N Am
2022Review
Dandy-Walker Malformation.
Society for Maternal-Fetal Medicine (SMFM) et al.·Am J Obstet Gynecol
2020Review
Walker-Warburg syndrome.
Vajsar J et al.·Orphanet J Rare Dis
2006Review
Walker-Warburg syndrome.
Suthar R et al.·Neurol India
2018Case report
Fukutin and alpha-dystroglycanopathies.
Toda T et al.·Acta Myol
2005Review
Walker-Warburg syndrome.
Agrawal S·BMJ Case Rep
2011Case report
Walker-Warburg Syndrome.
Khalaf SS et al.·J AAPOS
2006Case report
[Αlpha-dystroglycanopathy].
Toda T·Rinsho Shinkeigaku
2013
Top 10 resultsSearch PubMed ↗
Recent Gene-Specific Literature
Gene in title · MEDLINE · newest first
Europe PMC

No open access results found

Clinical Trials

Active and recruiting trials from ClinicalTrials.gov

No active trials found for this gene.

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