RGS22

Chr 8

regulator of G protein signaling 22

Also known as: CT145, PRTD-NY2

The RGS22 protein inhibits G-protein signaling by increasing the GTPase activity of G protein alpha subunits, driving them into their inactive GDP-bound form. Mutations in RGS22 cause autosomal recessive developmental and epileptic encephalopathy with onset in infancy, characterized by severe intellectual disability, refractory seizures, and developmental regression. The gene shows minimal constraint against loss-of-function variants in population databases.

Summary from RefSeq, UniProt
Research Assistant →
0
Active trials
1
Pubs (1 yr)
36
P/LP submissions
0%
P/LP missense
0.96
LOEUF
Mechanism
Clinical SummaryRGS22
Population Constraint (gnomAD)
Low constraint (pLI 0.00) — loss-of-function variants are relatively tolerated in the population.
📋
ClinVar Variants
36 unique Pathogenic / Likely Pathogenic· 148 VUS of 214 total submissions

Population Genetics & Constraint

gnomAD v4 — loss-of-function & missense intolerance

Tolerant — LoF & missense variants common in population
LoF Constraint
0.96LOEUF
pLI 0.000
Z-score 1.86
OE 0.76 (0.620.96)
Tolerant

Typical tolerance to LoF variation

Missense Constraint
0.41Z-score
OE missense 0.95 (0.891.02)
580 obs / 608.3 exp
Tolerant

Mild missense constraint

Observed / Expected Ratios
LoF OE0.76 (0.620.96)
00.351.4
Missense OE0.95 (0.891.02)
00.61.4
Synonymous OE0.91
01.21.6
LoF obs/exp: 56 / 73.2Missense obs/exp: 580 / 608.3Syn Z: 1.01

ClinVar Variant Classifications

214 submitted variants in ClinVar

Classification Summary

Pathogenic35
Likely Pathogenic1
VUS148
Likely Benign12
Benign4
35
Pathogenic
1
Likely Pathogenic
148
VUS
12
Likely Benign
4
Benign

Curated Variants Distribution

Classified variants from ClinVar · 5 ACMG categories

ClassificationLoFMissense + InframeNon-codingSynonymousTotal
Pathogenic
0
0
35
0
35
Likely Pathogenic
0
0
1
0
1
VUS
0
140
8
0
148
Likely Benign
0
10
0
2
12
Benign
1
0
0
3
4
Total1150445200

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

View in ClinVar →

Protein Context — Lollipop Plot

RGS22 · 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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