GMPPB

Chr 3

GDP-mannose pyrophosphorylase B

Also known as: LGMDR19, MDDGA14, MDDGB14, MDDGC14

This gene is thought to encode a GDP-mannose pyrophosphorylase. The encoded protein catalyzes the conversion of mannose-1-phosphate and GTP to GDP-mannose, a reaction involved in the production of N-linked oligosaccharides. Alternatively spliced transcript variants encoding distinct isoforms have been described. [provided by RefSeq, Jan 2009]

GeneReviewsResearchGenerating clinical summary…

Primary Disease Associations & Inheritance

UniProtMuscular dystrophy-dystroglycanopathy congenital with brain and eye anomalies A14
UniProtMuscular dystrophy-dystroglycanopathy congenital with impaired intellectual development B14
UniProtMuscular dystrophy-dystroglycanopathy limb-girdle C14

Clinical highlights

Gene-disease validity (ClinGen)
myopathy caused by variation in GMPPB · ARDefinitivesufficient evidence for diagnostic panels
1
Active trials
24
Pubs (1 yr)
P/LP submissions
P/LP missense
1.09
LOEUF
Mechanism
📖
GeneReview available — GMPPB
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?
1.09LOEUF
pLI 0.000
Z-score 1.28
OE 0.67 (0.431.09)
Tolerant

Highly tolerant — LoF variants common in population

Missense Constraint?
1.02Z-score
OE missense 0.82 (0.730.92)
205 obs / 250.4 exp
Tolerant

Mild missense constraint

Observed / Expected Ratios?
LoF OE?0.67 (0.431.09)
00.351.4
Missense OE?0.82 (0.730.92)
00.61.4
Synonymous OE?1.06
01.21.6
LoF obs/exp: 12 / 17.8Missense obs/exp: 205 / 250.4Syn Z: -0.45

ClinVar Variant Classifications

0 submitted variants in ClinVar

Protein Context — Lollipop Plot

GMPPB · protein map & ClinVar variants

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