Target intelligence / Profile preview

Potassium channel tetramerization domain-containing protein 6 (KCTD6)

Target
KCTD6
Molecular classification
BTB/POZ domain-containing protein, Cullin-3 E3 ligase substrate adaptor, Other (not an ion channel, receptor, enzyme, transporter, or transcription factor, but an adaptor/scaffolding protein)
01

Overview

Potassium channel tetramerization domain-containing protein 6 (KCTD6) is a member of the KCTD protein family, named for their N-terminal BTB/POZ domain, which bears sequence similarity to potassium channel tetramerization domains. KCTD6 primarily serves as a substrate-specific adaptor for cullin-3 E3 ubiquitin ligase complexes, mediating the selective ubiquitination and proteasomal degradation of proteins such as muscle ankyrin-1 isoform 5 and HDAC1. This regulation influences processes such as muscle protein turnover and Hedgehog pathway signaling. KCTD6 forms parallel homotetrameric complexes via its BTB/POZ domain and is important for maintenance of muscle structure and potentially other cellular processes. Dysfunction or altered expression of KCTD6 has been linked to muscle disorders and may have relevance in developmental and oncogenic contexts.

Other names
KCTD6BTB/POZ domain-containing protein KCTD6KCASH3MGC27385Potassium channel tetramerization domain-containing protein 6KCASH3 proteinPotassium channel tetramerisation domain containing 6
02

Biological functions

Protein ubiquitination/adaptor for E3 ligase activityRegulation of protein turnover (specifically ankyrin-1 in muscle)Regulation of Hedgehog pathway signaling via GLI1 trafficking (in complex with KCTD11)Negative regulation of smoothened signaling pathway
03

Disease associations

Muscle diseases (regulation of ankyrin-1 turnover linked to hereditary spherocytosis)Potential role in Arthrogryposis, distal, type 1APotential involvement in oncogenesis via HDAC1 regulation and Hedgehog pathway antagonism

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