The protein is a C-mannosyltransferase that adds mannose sugars to tryptophan residues on target proteins in the endoplasmic reticulum membrane. Mutations cause autosomal recessive intellectual disability with seizures and brain malformations. The gene shows no intolerance to loss-of-function variants based on population data.

Summary from RefSeq, UniProt
Research Assistant →
0
Active trials
1
Pubs (1 yr)
39
P/LP submissions
0%
P/LP missense
1.07
LOEUF
Mechanism
Clinical SummaryDPY19L4
Population Constraint (gnomAD)
Low constraint (pLI 0.00) — loss-of-function variants are relatively tolerated in the population.
📋
ClinVar Variants
39 unique Pathogenic / Likely Pathogenic· 77 VUS of 169 total submissions

Population Genetics & Constraint

gnomAD v4 — loss-of-function & missense intolerance

Tolerant — LoF & missense variants common in population
LoF Constraint
1.07LOEUF
pLI 0.000
Z-score 1.15
OE 0.82 (0.631.07)
Tolerant

Highly tolerant — LoF variants common in population

Missense Constraint
0.57Z-score
OE missense 0.92 (0.841.00)
337 obs / 367.9 exp
Tolerant

Mild missense constraint

Observed / Expected Ratios
LoF OE0.82 (0.631.07)
00.351.4
Missense OE0.92 (0.841.00)
00.61.4
Synonymous OE0.81
01.21.6
LoF obs/exp: 37 / 45.3Missense obs/exp: 337 / 367.9Syn Z: 1.65

ClinVar Variant Classifications

169 submitted variants in ClinVar

Classification Summary

Pathogenic37
Likely Pathogenic2
VUS77
Likely Benign4
Benign1
37
Pathogenic
2
Likely Pathogenic
77
VUS
4
Likely Benign
1
Benign

Curated Variants Distribution

Classified variants from ClinVar · 5 ACMG categories

ClassificationLoFMissense + InframeNon-codingSynonymousTotal
Pathogenic
0
0
37
0
37
Likely Pathogenic
0
0
2
0
2
VUS
0
76
1
0
77
Likely Benign
0
2
1
1
4
Benign
0
0
1
0
1
Total078421121

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

View in ClinVar →

Protein Context — Lollipop Plot

DPY19L4 · protein map & ClinVar variants

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

Clinical Trials

Active and recruiting trials from ClinicalTrials.gov

No active trials found for this gene.

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

No open access results found