Kelch-like protein 31 (KLHL31)

Kelch-like protein 31 (KLHL31) is a muscle-specific member of the Kelch protein superfamily, functioning as a substrate adaptor for Cullin 3 (CUL3)-mediated E3 ubiquitin ligase complexes involved in ubiquitin-proteasome dependent degradation of proteins[1][2][4]. KLHL31 localizes to the Z-disc of sarcomeres in skeletal muscle and is critical for maintaining muscle fiber integrity, regulating protein turnover, and muscle growth[1][2]. KLHL31 interacts directly with CUL3 to mediate polyubiquitination (K48 linkage) of substrate proteins such as Filamin-C (FlnC), targeting them for degradation[1][2]. In mouse models, deletion of KLHL31 results in postnatal muscle growth defects, centronuclear myopathy, sarcomeric and sarcotubular disorganization, and protein accumulation within muscle fibers[1][2][4]. While genes of the KLHL family have been implicated in diverse cellular processes like cytoskeletal organization and transcriptional regulation, KLHL31 is predominantly implicated in skeletal muscle maintenance and congenital myopathies[1][2][3]. There are no approved drugs or known clinical interventions targeting KLHL31. Note: KLHL31 is *not* currently a recognized therapeutic target (such as an enzyme, receptor, transporter, or direct disease mediator), but is of research interest—particularly in muscle biology and the genetics of congenital myopathies[1][2][4].

Other names

Kelch-like family member 31KLHL31BKLHD6KBTBD1KLHLbA345L23.2BTB and kelch domain-containing protein 6Kelch repeat and BTB domain-containing protein 1kelch-like protein KLHL