ENKUR

Chr 10

enkurin, TRPC channel interacting protein

Also known as: C10orf63, CFAP106

This gene encodes an adapter protein that localizes calcium-sensitive signaling machinery to calcium channels and functions as a microtubule inner protein required for motile cilia beating. Mutations cause primary ciliary dyskinesia with autosomal recessive inheritance, affecting respiratory function and fertility. The gene is not highly constrained against loss-of-function variants.

Summary from RefSeq, UniProt
Research Assistant →
0
Active trials
3
Pubs (1 yr)
10
P/LP submissions
0%
P/LP missense
1.20
LOEUF
DN
Mechanism· predicted
Clinical SummaryENKUR
Population Constraint (gnomAD)
Low constraint (pLI 0.00) — loss-of-function variants are relatively tolerated in the population.
📋
ClinVar Variants
10 unique Pathogenic / Likely Pathogenic· 157 VUS of 186 total submissions

Population Genetics & Constraint

gnomAD v4 — loss-of-function & missense intolerance

Tolerant — LoF & missense variants common in population
LoF Constraint
1.20LOEUF
pLI 0.000
Z-score 1.08
OE 0.67 (0.391.20)
Tolerant

Highly tolerant — LoF variants common in population

Missense Constraint
-0.02Z-score
OE missense 1.01 (0.881.16)
140 obs / 139.3 exp
Tolerant

Tolerant to missense variation

Observed / Expected Ratios
LoF OE0.67 (0.391.20)
00.351.4
Missense OE1.01 (0.881.16)
00.61.4
Synonymous OE1.08
01.21.6
LoF obs/exp: 8 / 12.0Missense obs/exp: 140 / 139.3Syn Z: -0.42
DN
0.78top 25%
GOF
0.3292th %ile
LOF
0.3067th %ile

The highest-scoring mechanism for this gene is dominant-negative.

DNprediction 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

186 submitted variants in ClinVar

Classification Summary

Pathogenic9
Likely Pathogenic1
VUS157
Likely Benign11
Benign2
9
Pathogenic
1
Likely Pathogenic
157
VUS
11
Likely Benign
2
Benign

Curated Variants Distribution

Classified variants from ClinVar · 5 ACMG categories

ClassificationLoFMissense + InframeNon-codingSynonymousTotal
Pathogenic
0
0
9
0
9
Likely Pathogenic
0
0
1
0
1
VUS
1
152
4
0
157
Likely Benign
0
9
0
2
11
Benign
0
0
2
0
2
Total1161162180

LoF = frameshift, stop gained/lost, canonical splice · Counts from ClinVar esearch · Updated hourly

View in ClinVar →

Protein Context — Lollipop Plot

ENKUR · protein map & ClinVar variants

Showing all ClinVar variants across the protein. Search a specific variant to highlight its position.

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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