EPB41L1

Chr 20AD

erythrocyte membrane protein band 4.1 like 1

Also known as: 4.1N, MRD11

The encoded protein stabilizes dopamine receptors at neuronal membranes and maintains neuronal membrane stability through interactions with the cytoskeleton and membrane channels. Mutations cause autosomal recessive mental retardation with neuropsychiatric features including intellectual disability, autism spectrum disorder, and behavioral abnormalities. This gene is highly constrained against loss-of-function variants, indicating that complete protein loss is likely incompatible with normal development.

OMIMResearchSummary from RefSeq, UniProt
ADLOEUF 0.341 OMIM phenotype
Clinical SummaryEPB41L1
🧬
Gene-Disease Validity (ClinGen)
complex neurodevelopmental disorder · ADLimited

Limited evidence — not for standalone diagnostic reporting

Population Constraint (gnomAD)
Moderately constrained gene (pLI 0.88) — some intolerance to loss-of-function variants.

Population Genetics & Constraint

gnomAD v4 — loss-of-function & missense intolerance

LoF intolerant — likely haploinsufficient
LoF Constraint
0.34LOEUF
pLI 0.877
Z-score 4.87
OE 0.19 (0.110.34)
Highly constrained

Highly LoF-intolerant (top ~10% of genes)

Missense Constraint
1.94Z-score
OE missense 0.76 (0.700.83)
394 obs / 518.2 exp
Tolerant

Mild missense constraint

Observed / Expected Ratios
LoF OE0.19 (0.110.34)
00.351.4
Missense OE0.76 (0.700.83)
00.61.4
Synonymous OE0.85
01.21.6
LoF obs/exp: 8 / 42.1Missense obs/exp: 394 / 518.2Syn Z: 1.73

ClinVar Variant Classifications

0 submitted variants in ClinVar

Protein Context — Lollipop Plot

EPB41L1 · 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 →