DLEU1

Chr 13

deleted in lymphocytic leukemia 1

Also known as: BCMS, BCMS1, DLB1, LEU1, LINC00021, NCRNA00021, XTP6

DLEU1 encodes a protein that may act as a tumor suppressor. Mutations in this gene have not been definitively associated with specific pediatric neurological disorders in the current literature. The gene shows relatively low constraint to loss-of-function variation, suggesting it may be more tolerant to protein-truncating mutations than highly constrained developmental genes.

Summary from UniProt
Research Assistant →
0
Active trials
9
Pubs (1 yr)
60
P/LP submissions
P/LP missense
1.48
LOEUF
Multiple*
Mechanism· predicted
Clinical SummaryDLEU1
Population Constraint (gnomAD)
Constrained for loss-of-function variants (OE-LoF 0.00) despite low pLI — interpret in context.
📋
ClinVar Variants
60 unique Pathogenic / Likely Pathogenic· 5 VUS of 68 total submissions

Population Genetics & Constraint

gnomAD v4 — loss-of-function & missense intolerance

Tolerant — LoF & missense variants common in population
LoF Constraint
1.48LOEUF
pLI 0.470
Z-score 1.20
OE 0.00 (0.001.48)
Tolerant

Highly tolerant — LoF variants common in population

Missense Constraint
0.68Z-score
OE missense 0.70 (0.520.95)
28 obs / 40.2 exp
Tolerant

Mild missense constraint

Observed / Expected Ratios
LoF OE0.00 (0.001.48)
00.351.4
Missense OE0.70 (0.520.95)
00.61.4
Synonymous OE0.47
01.21.6
LoF obs/exp: 0 / 1.7Missense obs/exp: 28 / 40.2Syn Z: 1.62
DN
0.6647th %ile
GOF
0.7028th %ile
LOF
0.3841th %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

68 submitted variants in ClinVar

Classification Summary

Pathogenic58
Likely Pathogenic2
VUS5
58
Pathogenic
2
Likely Pathogenic
5
VUS

Curated Variants Distribution

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

ClassificationLoFMissense + InframeNon-codingSynonymousTotal
Pathogenic
58
Likely Pathogenic
2
VUS
5
Likely Benign
0
Benign
0
Total65

Counts from ClinVar esearch · Updated hourly

View in ClinVar →

Protein Context — Lollipop Plot

DLEU1 · 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 →