This entry represents the C-terminal region of BTB/POZ domain-containing protein KCTD11 (the KCASH1 protein, also known as REN: EGF-, NGF- and retinoic acid-responsive gene) and its homologue, KCTD21 (the KCASH2 protein).These members of the KCASH family (KCTD containing, Cullin3 adaptor, suppressor of Hedgehog) share high similarity and a BTB domain responsible for oligomerization and formation of a Cullin3 ubiquitin ligase complex. They are specifically expressed in brain and cerebellum where they have a role in neuronal differentiation, and suppressor activity on acetylation-dependent Hedgehog/Gli signalling through ubiquitination and degradation of histone deacetylase 1 (HDAC1). These proteins have been described as tumor suppressors that inhibit medulloblastoma growth by antagonizing Hedgehog signalling [, , ].
