Kelch Like Protein 1 (KLHL1)

The predicted 748-amino acid KLHL1 protein has a domain structure similar to that of Drosophila Kelch, including a POZ/BTB protein-protein dimerization domain, followed by an intervening sequence and 6 C-terminal Kelch motif repeats, which constitute the actin-binding domain of Kelch.

The human KLHL1 protein shares 95% amino acid identity with the 751-amino acid mouse protein. Northern and dot blot analyses showed that human KLHL1 was expressed primarily in brain tissues, including cerebellum, substantia nigra, frontal lobe, and medulla. Mouse Klhl1 showed a similar expression pattern, with relatively higher expression in fetal brain than in adult brain. Epitope-tagged KLHL1 localized to cytoplasm in transfected COS-1 cells.