学術雑誌論文 Crystal structure of human WBSCR16, an RCC1-like protein in mitochondria

Koyama, Masako  ,  Sasaki, Taeko  ,  Sasaki, Narie  ,  Matsuura, Yoshiyuki

26 ( 9 )  , pp.1870 - 1877 , 2017-09 , Wiley
ISSN:0961-8368
内容記述
WBSCR16 (Williams-Beuren Syndrome Chromosomal Region 16) gene is located in alarge deletion region of Williams-Beuren syndrome (WBS), which is a neurodevelopmental disorder.Although the relationship between WBSCR16 and WBS remains unclear, it has been reported thatWBSCR16 is a member of a functional module that regulates mitochondrial 16S rRNA abundanceand intra-mitochondrial translation. WBSCR16 has RCC1 (Regulator of Chromosome Condensation1)-like amino acid sequence repeats but the function of WBSCR16 appears to be different fromthat of other RCC1 superfamily members. Here, we demonstrate that WBSCR16 localizes to mito-chondria in HeLa cells, and report the crystal structure of WBSCR16 deter mined to 2.0 A˚resolutionusing multi-wavelength anomalous diffraction. WBSCR16 adopts the seven-bladed b-propeller foldcharacteristic of RCC1-like proteins. A comparison of the WBSCR16 structure with that of RCC1and other RCC1-like proteins reveals that, although many of the residues buried in the core of theb-propeller are highly conserved, the surface residues are poorly conserved and conformationally divergent.
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