KCTD17

Chr 22

potassium channel tetramerization domain containing 17

This gene encodes a protein that belongs to a conserved family of potassium channel tetramerization domain (KCTD)-containing proteins. The encoded protein functions in ciliogenesis by acting as a substrate adaptor for the cullin3-based ubiquitin-conjugating enzyme E3 ligase, and targets trichoplein, a keratin-binding protein, for degradation via polyubiquitinylation. A mutation in this gene is associated with autosomal dominant myoclonic dystonia 26. [provided by RefSeq, Nov 2016]

ResearchGenerating clinical summary…

Primary Disease Associations & Inheritance

UniProtDystonia 26, myoclonic
0
Active trials
8
Pubs (1 yr)
P/LP submissions
P/LP missense
0.81
LOEUF
Multiple*
Mechanism· predicted
Some data sources returned errors (1)

omim: Error: OMIM fetch failed: 429

Population Genetics & Constraint

gnomAD v4 — loss-of-function & missense intolerance

Tolerant — LoF & missense variants common in population
LoF Constraint?
0.81LOEUF
pLI 0.018
Z-score 2.04
OE 0.39 (0.200.81)
Tolerant

Typical tolerance to LoF variation

Missense Constraint?
1.58Z-score
OE missense 0.64 (0.540.76)
99 obs / 154.3 exp
Tolerant

Mild missense constraint

Observed / Expected Ratios?
LoF OE?0.39 (0.200.81)
00.351.4
Missense OE?0.64 (0.540.76)
00.61.4
Synonymous OE?1.02
01.21.6
LoF obs/exp: 5 / 12.9Missense obs/exp: 99 / 154.3Syn Z: -0.13

ClinVar Variant Classifications

0 submitted variants in ClinVar

Protein Context — Lollipop Plot

KCTD17 · 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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