VMA12

Chr 17AR

vacuolar ATPase assembly factor VMA12

Also known as: C17orf32, CDG2P, TMEM199, VPH2

The protein encoded by this gene has been observed to localize to the endoplasmic reticulum (ER)-Golgi intermediate compartment (ERGIC) and coat protein complex I (COPI) in some human cells. The encoded protein shares some homology with the yeast protein Vma12. Defects in this gene are a cause of congenital disorder of glycosylation, type IIp. [provided by RefSeq, Mar 2016]

Primary Disease Associations & Inheritance

Congenital disorder of glycosylation, type IIpMIM #616829
AR
0
Active trials
12
Pathogenic / LP
84
ClinVar variants
3
Pubs (1 yr)
Missense Z
LOEUF
Clinical SummaryVMA12
📋
ClinVar Variants
12 Pathogenic / Likely Pathogenic· 39 VUS of 84 total submissions
Some data sources returned errors (1)

gnomad: Error: Gene not found

Population Genetics & Constraint

Constraint data not available from gnomAD.

ClinVar Variant Classifications

84 submitted variants in ClinVar

Classification Summary

Pathogenic8
Likely Pathogenic4
VUS39
Likely Benign23
Benign9
Conflicting1
8
Pathogenic
4
Likely Pathogenic
39
VUS
23
Likely Benign
9
Benign
1
Conflicting

Curated Variants Distribution

Classified variants from ClinVar · 5 ACMG categories

ClassificationLoFMissense + InframeNon-codingSynonymousTotal
Pathogenic
3
1
4
0
8
Likely Pathogenic
1
2
1
0
4
VUS
0
36
3
0
39
Likely Benign
0
2
12
9
23
Benign
0
4
5
0
9
Conflicting
1
Total44525984

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

View in ClinVar →

Protein Context — Lollipop Plot

VMA12 · protein map & ClinVar variants

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

Gene2Phenotype Curations

VMA12-related congenital disorder of glycosylation

strong
ARLoss Of FunctionAbsent Gene Product
Dev. Disorders
G2P ↗

Gene2Phenotype curations · DECIPHER consortium patient cohort (public variants) · deciphergenomics.org

OMIM — Genotype-Phenotype Relationships

1 OMIM entry

Congenital disorder of glycosylation, type IIp

MIM #616829

Molecular basis of disorder known

Autosomal recessive

Clinical Trials

Active and recruiting trials from ClinicalTrials.gov

No active trials found for this gene.

Search ClinicalTrials.gov →
Clinical Literature
Landmark / reviewRecent case evidence
Key Publications
Landmark & review papers · by relevance
PubMed
Top 1 results · since 2015Search PubMed ↗