SSU72

Chr 1

SSU72 homolog, RNA polymerase II CTD phosphatase

Also known as: HSPC182, PNAS-120

SSU72 encodes a protein phosphatase that dephosphorylates the C-terminal domain of RNA polymerase II and plays a role in mRNA processing and polyadenylation. Mutations cause autosomal recessive neurodevelopmental disorder with early-onset seizures, developmental delay, and intellectual disability. The gene shows evidence of constraint against loss-of-function variants (LOEUF 0.51), suggesting intolerance to complete protein loss.

Summary from RefSeq, UniProt
Research Assistant →
0
Active trials
6
Pubs (1 yr)
139
P/LP submissions
P/LP missense
0.51
LOEUF
Mechanism
Clinical SummarySSU72
Population Constraint (gnomAD)
Moderately constrained gene (pLI 0.78) — some intolerance to loss-of-function variants.
📋
ClinVar Variants
132 unique Pathogenic / Likely Pathogenic· 31 VUS of 171 total submissions

Population Genetics & Constraint

gnomAD v4 — loss-of-function & missense intolerance

Moderate LoF intolerance
LoF Constraint
0.51LOEUF
pLI 0.779
Z-score 2.53
OE 0.11 (0.040.51)
Moderately constrained

More LoF-intolerant than ~75% of genes

Missense Constraint
2.56Z-score
OE missense 0.32 (0.240.42)
36 obs / 112.3 exp
Mild constraint

Moderately missense-constrained (top ~2.5%)

Observed / Expected Ratios
LoF OE0.11 (0.040.51)
00.351.4
Missense OE0.32 (0.240.42)
00.61.4
Synonymous OE1.08
01.21.6
LoF obs/exp: 1 / 9.3Missense obs/exp: 36 / 112.3Syn Z: -0.45

ClinVar Variant Classifications

171 submitted variants in ClinVar

Classification Summary

Pathogenic125
Likely Pathogenic7
VUS31
Likely Benign1
125
Pathogenic
7
Likely Pathogenic
31
VUS
1
Likely Benign

Curated Variants Distribution

Classified variants from ClinVar · 5 ACMG categories· variant type breakdown unavailable

ClassificationLoFMissense + InframeNon-codingSynonymousTotal
Pathogenic
125
Likely Pathogenic
7
VUS
31
Likely Benign
1
Benign
0
Total164

Counts from ClinVar esearch · Updated hourly

View in ClinVar →

Protein Context — Lollipop Plot

SSU72 · 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.

Search ClinicalTrials.gov →
Clinical Literature
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Key Publications
Landmark & review papers · by relevance
PubMed
Top 5 results · since 2015Search PubMed ↗