DPY30

Chr 2

dpy-30 histone methyltransferase complex regulatory subunit

Also known as: Cps25, HDPY-30, Saf19

The protein is a core subunit of SET1/MLL histone methyltransferase complexes that methylates histone H3 at lysine 4, an epigenetic mark for transcriptional activation, and regulates cell cycle progression and hematopoietic cell differentiation. Mutations cause intellectual disability with seizures and variable dysmorphic features, following an autosomal dominant inheritance pattern. The gene shows relatively low constraint to loss-of-function variation.

OMIMResearchSummary from RefSeq, UniProt
LOEUF 0.98
Clinical SummaryDPY30
Population Constraint (gnomAD)
Constrained for loss-of-function variants (OE-LoF 0.31) despite low pLI — interpret in context.
📋
ClinVar Variants
28 unique Pathogenic / Likely Pathogenic· 13 VUS of 44 total submissions

Population Genetics & Constraint

gnomAD v4 — loss-of-function & missense intolerance

Tolerant — LoF & missense variants common in population
LoF Constraint
0.98LOEUF
pLI 0.177
Z-score 1.62
OE 0.31 (0.130.98)
Tolerant

Typical tolerance to LoF variation

Missense Constraint
1.47Z-score
OE missense 0.42 (0.300.60)
21 obs / 50.2 exp
Tolerant

Mild missense constraint

Observed / Expected Ratios
LoF OE0.31 (0.130.98)
00.351.4
Missense OE0.42 (0.300.60)
00.61.4
Synonymous OE1.42
01.21.6
LoF obs/exp: 2 / 6.4Missense obs/exp: 21 / 50.2Syn Z: -1.35

ClinVar Variant Classifications

44 submitted variants in ClinVar

Classification Summary

Pathogenic26
Likely Pathogenic2
VUS13
Benign1
26
Pathogenic
2
Likely Pathogenic
13
VUS
1
Benign

Curated Variants Distribution

Classified variants from ClinVar · 5 ACMG categories

ClassificationLoFMissense + InframeNon-codingSynonymousTotal
Pathogenic
0
0
26
0
26
Likely Pathogenic
0
0
2
0
2
VUS
0
5
8
0
13
Likely Benign
0
0
0
0
0
Benign
0
0
1
0
1
Total0537042

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

View in ClinVar →

Protein Context — Lollipop Plot

DPY30 · 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

No active trials found for this gene.

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Clinical Literature
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