REP15

Chr 12

RAB15 effector protein

Also known as: RAB15EP

The protein encoded by REP15 acts as an effector for active Rab GTPases and regulates transferrin receptor recycling from endocytic compartments to the cell surface, controlling cellular processes including proliferation and migration. Mutations in REP15 cause autosomal recessive developmental and epileptic encephalopathy with movement abnormalities, typically presenting in infancy. The gene is not highly constrained against loss-of-function variants.

Summary from RefSeq, UniProt
Research Assistant →
0
Active trials
0
Pubs (1 yr)
33
P/LP submissions
0%
P/LP missense
1.78
LOEUF
Mechanism
Clinical SummaryREP15
Population Constraint (gnomAD)
Low constraint (pLI 0.00) — loss-of-function variants are relatively tolerated in the population.
📋
ClinVar Variants
33 unique Pathogenic / Likely Pathogenic· 27 VUS of 63 total submissions

Population Genetics & Constraint

gnomAD v4 — loss-of-function & missense intolerance

Tolerant — LoF & missense variants common in population
LoF Constraint
1.78LOEUF
pLI 0.000
Z-score -0.09
OE 1.04 (0.581.78)
Tolerant

Highly tolerant — LoF variants common in population

Missense Constraint
0.44Z-score
OE missense 0.89 (0.761.04)
106 obs / 119.5 exp
Tolerant

Mild missense constraint

Observed / Expected Ratios
LoF OE1.04 (0.581.78)
00.351.4
Missense OE0.89 (0.761.04)
00.61.4
Synonymous OE1.05
01.21.6
LoF obs/exp: 7 / 6.8Missense obs/exp: 106 / 119.5Syn Z: -0.28

ClinVar Variant Classifications

63 submitted variants in ClinVar

Classification Summary

Pathogenic31
Likely Pathogenic2
VUS27
Likely Benign3
31
Pathogenic
2
Likely Pathogenic
27
VUS
3
Likely Benign

Curated Variants Distribution

Classified variants from ClinVar · 5 ACMG categories

ClassificationLoFMissense + InframeNon-codingSynonymousTotal
Pathogenic
0
0
31
0
31
Likely Pathogenic
0
0
2
0
2
VUS
0
25
2
0
27
Likely Benign
0
3
0
0
3
Benign
0
0
0
0
0
Total02835063

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

View in ClinVar →

Protein Context — Lollipop Plot

REP15 · 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.

Search ClinicalTrials.gov →
Clinical Literature
Open Research Assistant →
Recent Gene-Specific Literature
Gene in title · MEDLINE · newest first
Europe PMC