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10 sources retrieved · Most recent: April 2026 · Index updated 15 days ago
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KCTD6
potassium channel tetramerization domain containing 6
Chromosome 3 · 3p14.3
NCBI Gene: 200845Ensembl: ENSG00000168301.14HGNC: HGNC:22235UniProt: Q8NC69
35PubMed Papers
20Diseases
0Drugs
0Pathogenic Variants
FUNCTIONAL ROLE
Tumor Suppressor
DATA QUALITY
✓ Experimental GO Evidence✓ Swiss-Prot Reviewed
cullin family protein bindingnegative regulation of smoothened signaling pathwayidentical protein bindingankyrin bindingseptic shockHyperhidrosissystemic lupus erythematosusirritable bowel syndrome
✦AI Summary

KCTD6 functions as a substrate-specific adapter protein for BCR (BTB-CUL3-RBX1) E3 ubiquitin-protein ligase complexes, mediating the ubiquitination and proteasomal degradation of target proteins 1. The protein specifically regulates turnover of muscle small ankyrin-1 isoform 5 (sAnk1.5) through cullin-3-dependent mechanisms, with this process modulated by posttranslational modifications including neddylation, ubiquitination, and acetylation 1. KCTD6 promotes ubiquitination of HDAC1 in an oligomerization-dependent manner with KCTD11, functioning as a tumor suppressor by antagonizing the Hedgehog signaling pathway 2. By degrading HDAC1, KCTD6 maintains GLI1 in an acetylated, transcriptionally inactive state, thereby inhibiting medulloblastoma cell growth and tumorigenicity 2. The protein demonstrates high-affinity binding to Cullin 3 through its BTB domain, forming stable 4:4 stoichiometric complexes 3. KCTD6 interacts with the deubiquitylase USP21 and is involved in Gli1 regulation at the centrosome 4. Additionally, KCTD6 has been implicated in keratinocyte differentiation regulation and identified as a potential biomarker in kidney transplant rejection 56.

Sources cited
1
KCTD6 functions as a substrate adapter for cullin-3 E3 ligase and regulates sAnk1.5 protein turnover
PMID: 22573887
2
KCTD6 promotes HDAC1 ubiquitination, antagonizes Hedgehog signaling, and inhibits medulloblastoma growth
PMID: 21472142
3
KCTD6 binds Cullin 3 with high affinity through its BTB domain
PMID: 25974686
4
KCTD6 interacts with USP21 deubiquitylase and is involved in Gli1 regulation
PMID: 27621083
5
KCTD6 is involved in keratinocyte differentiation regulation
PMID: 38250727
6
KCTD6 identified as potential biomarker in kidney transplant acute rejection
PMID: 32550916
Disease Associationsⓘ20
septic shockOpen Targets
0.31Weak
HyperhidrosisOpen Targets
0.27Weak
systemic lupus erythematosusOpen Targets
0.05Suggestive
irritable bowel syndromeOpen Targets
0.03Suggestive
knee fractureOpen Targets
0.03Suggestive
HypercholesterolemiaOpen Targets
0.02Suggestive
head and neck squamous cell carcinomaOpen Targets
0.02Suggestive
type 2 diabetes mellitusOpen Targets
0.02Suggestive
metabolic diseaseOpen Targets
0.02Suggestive
ovarian carcinomaOpen Targets
0.02Suggestive
hepatocellular carcinomaOpen Targets
0.01Suggestive
cancerOpen Targets
0.01Suggestive
medulloblastomaOpen Targets
0.01Suggestive
ataxia telangiectasiaOpen Targets
0.01Suggestive
atopic eczemaOpen Targets
0.01Suggestive
non-small cell lung carcinomaOpen Targets
0.01Suggestive
neoplasmOpen Targets
0.01Suggestive
hereditary spherocytosisOpen Targets
0.00Suggestive
acute disseminated encephalomyelitisOpen Targets
0.00Suggestive
Alzheimer diseaseOpen Targets
0.00Suggestive
Pathogenic Variants
No pathogenic variants reported on ClinVar for this gene.
View on ClinVar ↗
Related Genes
KCTD21Shared pathway73%CUL3Protein interaction72%UBE2MProtein interaction72%HERC4Shared pathway50%KCTD10Shared pathway40%KCTD1Shared pathway40%
Tissue Expression6 tissues
Liver
100%
Bone Marrow
85%
Ovary
77%
Brain
69%
Heart
58%
Lung
38%
Gene Interaction Network
Click a node to explore
KCTD6KCTD21CUL3UBE2MHERC4KCTD10KCTD1
PROTEIN STRUCTURE
Preparing viewer…
AlphaFoldAI-predicted · UniProt Q8NC69
View on AlphaFold ↗
Constraintⓘ
LOEUFⓘ
0.78LoF Tolerant
pLIⓘ
0.38Tolerant
Observed/Expected LoF0.39 [0.21–0.78]
RankingsWhere KCTD6 stands among ~20K protein-coding genes
  • #10,993of 20,598
    Most Researched35
  • #6,376of 17,882
    Most Constrained (LOEUF)0.78
Genes detectedKCTD6
Sources retrieved10 papers
Response time—
📄 Sources
10▼
1
Obscurin and KCTD6 regulate cullin-dependent small ankyrin-1 (sAnk1.5) protein turnover.
PMID: 22573887
Mol Biol Cell · 2012
1.00
2
Identification and characterization of KCASH2 and KCASH3, 2 novel Cullin3 adaptors suppressing histone deacetylase and Hedgehog activity in medulloblastoma.
PMID: 21472142
Neoplasia · 2011
0.90
3
An age-independent gene signature for monitoring acute rejection in kidney transplantation.
PMID: 32550916
Theranostics · 2020
0.80
4
Genome-Wide Association Transethnic Meta-Analyses Identifies Novel Associations Regulating Coagulation Factor VIII and von Willebrand Factor Plasma Levels.
PMID: 30586737
Circulation · 2019
0.70
5
GSDMD suppresses keratinocyte differentiation by inhibiting FLG expression and attenuating KCTD6-mediated HDAC1 degradation in atopic dermatitis.
PMID: 38250727
PeerJ · 2024
0.60