LEPROT

Chr 1

leptin receptor overlapping transcript

Also known as: OB-RGRP, OBRGRP, VPS55

LEPROT encodes a protein that negatively regulates cell surface expression of leptin and growth hormone receptors, particularly decreasing leptin response and contributing to hepatic growth hormone resistance during nutrient scarcity. Mutations cause autosomal recessive obesity and short stature, affecting metabolic and growth pathways. The gene shows moderate tolerance to loss-of-function variants, consistent with recessive inheritance patterns.

Summary from RefSeq, UniProt
Research Assistant →
0
Active trials
3
Pubs (1 yr)
0
P/LP submissions
P/LP missense
0.87
LOEUF
Multiple*
Mechanism· predicted
Clinical SummaryLEPROT
Population Constraint (gnomAD)
Constrained for loss-of-function variants (OE-LoF 0.18) despite low pLI — interpret in context.

Population Genetics & Constraint

gnomAD v4 — loss-of-function & missense intolerance

Tolerant — LoF & missense variants common in population
LoF Constraint
0.87LOEUF
pLI 0.438
Z-score 1.76
OE 0.18 (0.070.87)
Tolerant

Typical tolerance to LoF variation

Missense Constraint
0.71Z-score
OE missense 0.77 (0.610.96)
55 obs / 71.9 exp
Tolerant

Mild missense constraint

Observed / Expected Ratios
LoF OE0.18 (0.070.87)
00.351.4
Missense OE0.77 (0.610.96)
00.61.4
Synonymous OE0.87
01.21.6
LoF obs/exp: 1 / 5.4Missense obs/exp: 55 / 71.9Syn Z: 0.51
DN
0.6358th %ile
GOF
0.80top 10%
LOF
0.3261th %ile

This gene has evidence for multiple mechanisms of pathogenicity (gain-of-function and dominant-negative). Both the Badonyi & Marsh prediction and the broader genomic evidence point to gain-of-function as the predominant mechanism. Different variants in this gene may act through different mechanisms — interpret in context of the specific variant.

GOFprediction above median
DNprediction above median

Note: In-silico variant effect predictors (SIFT, PolyPhen, REVEL, CADD) may underestimate pathogenicity of missense variants in genes with GOF or DN mechanisms. Consider functional evidence and clinical context.

Predictions from Badonyi M, Marsh JA. PLoS ONE. 2024;19(8):e0307312.

ClinVar Variant Classifications

0 submitted variants in ClinVar

Protein Context — Lollipop Plot

LEPROT · 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
Open Research Assistant →
Key Publications
Landmark & review papers · by relevance
PubMed
Top 1 results · since 2015Search PubMed ↗