KLHL13 (kelch-like 13), also known as BKLHD2 (BTB and kelch domain-containing protein 2), is a 604 amino acid protein that contains six Kelch repeats and one BTB/POZ domain. Expressed predominantly in brain, KLHL13 is believed to play a role in protein ubiquitination and may function as a substrate- specific adapter of an E3 ubiquitin-protein ligase complex. E3 ligases accept a ubiquitin residue from an E2 ubiquitin-conjugating enzyme and immediately transfer that residue to a protein that is targeted for degradation. Specifically, KLHL13 interacts with KLHL9 and CUL-3, a member of the cullin family of mediators that participate in the selective targeting of proteins for ubiquitin-mediated proteolysis. Via its BTB and C-terminal Kelch (BACK) motif, KLHL13 is thought to play a role in spatially orientating substrates in the CUL-3 ligase.
