Kelch-like protein 26 (KLHL26)

Kelch-like protein 26 (KLHL26) is a member of the Kelch-like (KLHL) family, which is characterized by the presence of a BTB/POZ domain for protein dimerization, a BACK domain of unclear function, and a series of Kelch repeats that form a β-propeller structure for substrate recognition. KLHL26 is predicted, by homology with better-studied KLHL proteins, to function as a substrate adaptor for the CUL3-based E3 ubiquitin ligase complex in the ubiquitin-proteasome system, directing protein substrates for ubiquitination and subsequent degradation. KLHL26 is highly expressed in muscle tissues, and a rare coding variant has been documented to segregate with Ebstein’s anomaly and left ventricular noncompaction in a multigenerational family, implicating it (though not conclusively proving it) in cardiac development through protein turnover pathways. No experimental drugs, biomarker uses, or broad disease roles beyond this rare cardiac defect have been described.

Other names

KLHL26Kelch-like protein 26Kelch-like family member 26kelch-like 26

Not applicable; no known drugs or inhibitors