Kelch-like protein 13 (KLHL13)

Kelch-like protein 13 (KLHL13) is a substrate adaptor protein of the BTB-CUL3-RBX1 (BCR) E3 ubiquitin-protein ligase complex, necessary for proper mitotic progression and cytokinesis[2][3]. KLHL13 facilitates ubiquitination of specific substrate proteins, including Aurora kinase B (AURKB), thereby regulating chromosome segregation during mitosis[2]. This protein contains characteristic domains of the KLHL family: a BTB/POZ domain (protein binding and dimerization), a BACK domain (whose mutations are linked with disease), and five or six Kelch repeat motifs (involved in substrate recognition)[1]. KLHL family proteins play important roles in cellular protein degradation, cytoskeletal organization, and cellular morphology, and have been implicated in both hereditary diseases and cancer[1][4]. Mutations or dysfunction in KLHL13 or related proteins can perturb cell division and have been associated with neurological and oncological disorders such as Charcot-Marie-Tooth Disease and cancer[2]. Key notes: - No drugs are currently listed as direct KLHL13 antagonists/agonists. - KLHL13 is not a classical receptor, ion channel, or enzyme, but rather an E3 ligase complex adaptor, relevant for targeted protein degradation pathways[2][4]. - No known biomarkers or notable toxicity concerns specific to KLHL13 (apart from general disease association if mutated/disrupted). - The family and this gene are well-annotated, with no nomenclature errors.

Other names

Kelch like family member 13KLHL13BKLHD2KIAA1309FLJ10262BTB and kelch domain-containing protein 2kelch-like 13BTB and kelch domain containing 2

Functions as an adaptor protein with Cullin 3 for E3 ubiquitin ligase–directed substrate ubiquitination and degradation[2]