DNASE1L1

Chr X

deoxyribonuclease 1 like 1

Also known as: DNAS1L1, DNASEX, DNL1L, G4.8, XIB

The protein is a deoxyribonuclease localized to the endoplasmic reticulum that degrades DNA and is modified by N-linked glycosylation. Mutations cause autosomal recessive systemic lupus erythematosus, an autoimmune condition affecting multiple organ systems including skin, joints, kidneys, and the central nervous system. The gene shows moderate constraint against loss-of-function variants (LOEUF 0.69), suggesting some intolerance to complete protein loss.

OMIMResearchSummary from RefSeq
LOEUF 0.69
Clinical SummaryDNASE1L1
Population Constraint (gnomAD)
Constrained for loss-of-function variants (OE-LoF 0.22) 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
0.69LOEUF
pLI 0.388
Z-score 2.20
OE 0.22 (0.090.69)
Tolerant

Typical tolerance to LoF variation

Missense Constraint
0.69Z-score
OE missense 0.83 (0.710.97)
111 obs / 133.5 exp
Tolerant

Mild missense constraint

Observed / Expected Ratios
LoF OE0.22 (0.090.69)
00.351.4
Missense OE0.83 (0.710.97)
00.61.4
Synonymous OE1.24
01.21.6
LoF obs/exp: 2 / 9.2Missense obs/exp: 111 / 133.5Syn Z: -1.46

ClinVar Variant Classifications

0 submitted variants in ClinVar

Protein Context — Lollipop Plot

DNASE1L1 · 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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Recent Gene-Specific Literature
Gene in title · MEDLINE · newest first
Europe PMC

No open access results found