Protein Domain : Kelch-like protein 41/41b, BTB/POZ domain IPR030571

Type	Domain
Description	The KLHL (Kelch-like) proteins generally have a BTB/POZ domain, a BACK domain, and five to six Kelch motifs. They constitute a subgroup at the intersection between the BTB/POZ domain and Kelch domain superfamilies. The BTB/POZ domain facilitates protein binding [ ], while the Kelch domain (repeats) form β-propellers. The Kelch superfamily of proteins can be subdivided into five groups: (1) N-propeller, C-dimer proteins, (2) N-propeller proteins, (3) propeller proteins, (4) N-dimer, C-propeller proteins, and (5) C-propeller proteins. KLHL family members belong to the N-dimer, C-propeller subclass of Kelch repeat proteins []. In addition to BTB/POZ and Kelch domains, the KLHL family members contain a BACK domain, first described as a 130-residue region of conservation observed amongst BTB-Kelch proteins []. Many of the Kelch-like proteins have been identified as adaptors for the recruitment of substrates to Cul3-based E3 ubiquitin ligases [, ].Kelch-like protein 41 (KLHL41), also known as sarcosin or Krp1, is involved in skeletal muscle development and differentiation [ , ]. KLHL41 binds to Nebulin, an actin-binding protein that regulates the actin filament length. It also binds Nebulin-related anchoring protein (N-RAP), an actin-binding protein expressed in skeletal and cardiac muscle tissues [, ]. KLHL41 is localised between laterally fusing myofibrils in adult skeletal muscle and plays an important role for the correct functioning of adult skeletal muscle cells [ ]. Mutations in its gene lead to nemaline myopathy-9 (NEM9), a muscle disorder characterized by onset of muscle weakness in early infancy [].This group of proteins also includes the orthologue from fish Kelch-like protein 41b (KL41B), which is involved in skeletal muscle development and maintenance [ ]. This entry represents the N-terminal BTB (Broad-Complex, Tramtrack and Bric a brac)/POZ (poxvirus and zinc finger) domain of mammal KLHL41, fish KL41B and related proteins from vertebrates.
Short Name	KLHL41_KL41B_BTB_POZ_dom