KLHL7

KLHL7

KLHL7: Human kelch-like 7
PDB Code: 3II7
Description
Kelch-like 7 is a member of the BTB/kelch (Broad-Complex, Tramtrack and Bric a brac) repeat family. The protein consists of a BTB domain, a BACK domain (BTB and C-terminal Kelch), and a C-terminal Kelch domain comprising six Kelch repeats.
KLHL7 is ubiquitously expressed with highest expression levels in heart, nervous system and testis.
KLHL7 was originally identified as an autoantigen in several cancers, neurological diseases as well as Sjögren's syndrome. More recently it has been shown that mutations in the BACK domain of KLHL7 can lead to autosomal-dominant retinitis pigmentosa. Although the precise function for KLHL7 is unknown it is thought by analogy with other family members to function as an E3 ubiquitin ligase in which the Kelch domain forms the substrate recognition unit, while the BTB domain complexes with Cullin3; the BACK domain is thought to represent a helical linker.
Here we report the structure of the Kelch domain of KLHL7 at 1.6 Å resolution.

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