CNPY3

Chr 6AR

canopy FGF signaling regulator 3

Also known as: CAG4A, DEE60, EIEE60, ERDA5, PRAT4A, TNRC5

The protein functions as a co-chaperone for HSP90B1 and is required for proper folding and endoplasmic reticulum export of most toll-like receptors, controlling both innate and adaptive immune responses. Biallelic mutations cause developmental and epileptic encephalopathy 60, inherited in an autosomal recessive pattern. This represents a neuroinflammatory condition affecting both developmental and epileptic manifestations in the pediatric population.

OMIMResearchSummary from RefSeq, OMIM, UniProt
LOFmechanismARLOEUF 0.571 OMIM phenotype
Clinical SummaryCNPY3
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

Moderate LoF intolerance
LoF Constraint
0.57LOEUF
pLI 0.398
Z-score 2.68
OE 0.22 (0.100.57)
Moderately constrained

More LoF-intolerant than ~75% of genes

Missense Constraint
0.42Z-score
OE missense 0.90 (0.791.04)
136 obs / 150.5 exp
Tolerant

Mild missense constraint

Observed / Expected Ratios
LoF OE0.22 (0.100.57)
00.351.4
Missense OE0.90 (0.791.04)
00.61.4
Synonymous OE0.81
01.21.6
LoF obs/exp: 3 / 13.7Missense obs/exp: 136 / 150.5Syn Z: 1.18

ClinVar Variant Classifications

0 submitted variants in ClinVar

Protein Context — Lollipop Plot

CNPY3 · 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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