Kelch Like Protein 32 (KLHL32)

Kelch is a 50-residue motif, named after the Drosophila mutant in which it was first identified . This sequence motif represents one beta-sheet blade, and several of these repeats can associate to form a beta-propeller. For instance, the motif appears 6 times in Drosophila egg-chamber regulatory protein, creating a 6-bladed beta-propeller. Seven open reading frames within this interval according to the presence of their orthologs in species that are known to express complex I activity. Only KLHL32 complied with this criteria; its orthologs were present in the genomes of the aerobic yeasts Y. lipolytica and D. Hansenii, which express complex I and were absent from the genomes of the nonaerobic yeasts S. cerevisiae, C. Glabrata, and K. Lactis. However, the sequence of the ten coding exons of the KLHL32 gene was normal in the patients' DNA.