Article
Decreased KLHL3 expression is involved in the pathogenesis of pseudohypoaldosteronism type II caused by cullin 3 mutation in vivo.
Clinical and experimental nephrology - 1 Dec 2018
Yoshida Sayaka, Araki Yuya, Mori Takayasu, Sasaki Emi, Kasagi Yuri, Isobe Kiyoshi, Susa Koichiro, Inoue Yuichi, Bomont Pascale, Okado Tomokazu, Rai Tatemitsu, Uchida Shinichi, Sohara Eisei
Abstract excerpt
BACKGROUND: Pseudohypoaldosteronism type II (PHAII) is a hereditary hypertensive disease caused by mutations in four genes: WNK1, WNK4, Kelch-like3 (KLHL3), and cullin3 (CUL3). Recently, it was revealed that CUL3-KLHL3 E3 ligase complex ubiquitinates WNK1 and WNK4, leading to their degradation, and that a common pathogenesis of PHAII is defective WNK degradation due to CUL3-KLHL3 E3 ligase complex impairment....
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