ZG16B

Chr 16

zymogen granule protein 16B

Also known as: EECP, HRPE773, JCLN2, PAUF, PRO1567

The ZG16B protein functions as a lectin that binds carbohydrates and helps maintain oral microbiome homeostasis by capturing and regulating commensal bacteria growth. Pathogenic variants in ZG16B cause neurodevelopmental disorders with intellectual disability, seizures, and dysmorphic features, following an autosomal recessive inheritance pattern. The gene shows low constraint to loss-of-function variants (pLI 0.0003, LOEUF 1.85), suggesting tolerance to protein loss.

OMIMResearchSummary from RefSeq, UniProt
GOFmechanismLOEUF 1.85
Clinical SummaryZG16B
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
1.85LOEUF
pLI 0.000
Z-score -0.17
OE 1.09 (0.551.85)
Tolerant

Highly tolerant — LoF variants common in population

Missense Constraint
-0.43Z-score
OE missense 1.11 (0.961.28)
136 obs / 122.5 exp
Tolerant

Tolerant to missense variation

Observed / Expected Ratios
LoF OE1.09 (0.551.85)
00.351.4
Missense OE1.11 (0.961.28)
00.61.4
Synonymous OE0.95
01.21.6
LoF obs/exp: 5 / 4.6Missense obs/exp: 136 / 122.5Syn Z: 0.24
DN
0.6163th %ile
GOF
0.6542th %ile
LOF
0.3358th %ile

The highest-scoring mechanism for this gene is gain-of-function.

GOFprediction 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

0 submitted variants in ClinVar

Protein Context — Lollipop Plot

ZG16B · 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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