FBXL17


Description

The FBXL17 (F-box and leucine rich repeat protein 17) is a protein-coding gene located on chromosome 5.

FBXL17 is a substrate-recognition component of the SCF(FBXL17) E3 ubiquitin ligase complex. This complex plays a key role in a quality control pathway that ensures the proper dimerization of BTB domain-containing proteins. FBXL17 specifically binds to a conserved degron sequence within aberrantly formed BTB dimers, marking them for ubiquitination and degradation by the proteasome. This degradation pathway is crucial for the differentiation and survival of neural crest and neuronal cells. The SCF(FBXL17) complex also regulates the hedgehog/smoothened (Hh) signaling pathway by ubiquitinating and degrading SUFU, thereby releasing GLI1 and enabling proper Hh signal transduction. Additionally, FBXL17 is involved in the degradation of other proteins including BACH1 and PRMT1.

FBXL17 is also known as FBXO13, Fbl17, Fbx13.

Associated Diseases



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