CCDC47

Chr 17AR

coiled-coil domain containing 47

Also known as: GK001, MSTP041, THNS

The CCDC47 protein is a component of the multi-pass translocon complex that mediates insertion of multi-pass membrane proteins into the endoplasmic reticulum and regulates ER calcium homeostasis. Autosomal recessive mutations cause trichohepatoneurodevelopmental syndrome, affecting hair, liver, and neurodevelopment. This gene is not highly constrained against loss-of-function variants.

OMIMResearchSummary from RefSeq, OMIM, UniProt
LOFmechanismARLOEUF 0.881 OMIM phenotype
Clinical SummaryCCDC47
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
0.88LOEUF
pLI 0.000
Z-score 2.07
OE 0.58 (0.390.88)
Tolerant

Typical tolerance to LoF variation

Missense Constraint
1.04Z-score
OE missense 0.82 (0.730.92)
212 obs / 259.1 exp
Tolerant

Mild missense constraint

Observed / Expected Ratios
LoF OE0.58 (0.390.88)
00.351.4
Missense OE0.82 (0.730.92)
00.61.4
Synonymous OE0.98
01.21.6
LoF obs/exp: 16 / 27.8Missense obs/exp: 212 / 259.1Syn Z: 0.16

ClinVar Variant Classifications

0 submitted variants in ClinVar

Protein Context — Lollipop Plot

CCDC47 · 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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