DNASE1L2

Chr 16

deoxyribonuclease 1 like 2

Also known as: DNAS1L2

The protein is a divalent cation-dependent acid DNA endonuclease that breaks down nuclear DNA during corneocyte formation in epidermal keratinocytes and may eliminate harmful extracellular DNA from damaged skin cells. Mutations cause autosomal recessive hypotrichosis simplex, a condition characterized by sparse hair affecting the scalp and body. The gene shows very low constraint against loss-of-function variants, consistent with recessive inheritance.

OMIMResearchSummary from RefSeq, UniProt
LOEUF 1.40
Clinical SummaryDNASE1L2
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

Tolerant — LoF & missense variants common in population
LoF Constraint
1.40LOEUF
pLI 0.000
Z-score 0.52
OE 0.84 (0.531.40)
Tolerant

Highly tolerant — LoF variants common in population

Missense Constraint
-0.34Z-score
OE missense 1.07 (0.951.20)
200 obs / 186.9 exp
Tolerant

Tolerant to missense variation

Observed / Expected Ratios
LoF OE0.84 (0.531.40)
00.351.4
Missense OE1.07 (0.951.20)
00.61.4
Synonymous OE0.98
01.21.6
LoF obs/exp: 11 / 13.0Missense obs/exp: 200 / 186.9Syn Z: 0.11

ClinVar Variant Classifications

0 submitted variants in ClinVar

Protein Context — Lollipop Plot

DNASE1L2 · 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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