LLGL1

Chr 17

LLGL scribble cell polarity complex component 1

Also known as: DLG4, HUGL, HUGL-1, HUGL1, LLGL, Lgl1, Mgl1

The protein maintains cell polarity and epithelial integrity as part of the cortical cytoskeleton, regulates mitotic spindle orientation in neuroepithelial cells, and is involved in axonogenesis through regulation of vesicular trafficking. Mutations cause autosomal recessive congenital hydrocephalus with severe developmental delays and brain malformations. This gene is highly intolerant to loss-of-function variants in the general population.

OMIMResearchSummary from RefSeq, UniProt
LOEUF 0.53
Clinical SummaryLLGL1
Population Constraint (gnomAD)
Low constraint (pLI 0.00) — loss-of-function variants are relatively tolerated in the population.

Population Genetics & Constraint

gnomAD v4 — loss-of-function & missense intolerance

Moderate LoF intolerance
LoF Constraint
0.53LOEUF
pLI 0.000
Z-score 4.16
OE 0.35 (0.240.53)
Moderately constrained

More LoF-intolerant than ~75% of genes

Missense Constraint
1.45Z-score
OE missense 0.84 (0.790.90)
575 obs / 681.9 exp
Tolerant

Mild missense constraint

Observed / Expected Ratios
LoF OE0.35 (0.240.53)
00.351.4
Missense OE0.84 (0.790.90)
00.61.4
Synonymous OE1.16
01.21.6
LoF obs/exp: 17 / 48.1Missense obs/exp: 575 / 681.9Syn Z: -2.16

ClinVar Variant Classifications

0 submitted variants in ClinVar

Protein Context — Lollipop Plot

LLGL1 · 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.

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Clinical Literature
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