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Deprecated: Implicit conversion from float 231.6 to int loses precision in C:\Inetpub\vhosts\kidney.de\httpdocs\pget.php on line 534 Proc+Natl+Acad+Sci+U+S+A 2014 ; 111 (52): 18566-71 Nephropedia Template TP
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Regulation of the Rhp26ERCC6/CSB chromatin remodeler by a novel conserved leucine latch motif #MMPMID25512493
Wang L; Limbo O; Fei J; Chen L; Kim B; Luo J; Chong J; Conaway RC; Conaway JW; Ranish JA; Kadonaga JT; Russell P; Wang D
Proc Natl Acad Sci U S A 2014[Dec]; 111 (52): 18566-71 PMID25512493show ga
Cockayne syndrome group B protein/excision repair cross-complementation group 6 (CSB/ERCC6) belongs to a subfamily of SWI2/SNF2 (SWItch/sucrose nonfermentable)-related chromatin-remodeling complexes. Defects in the CSB protein result in Cockayne syndrome, which is a rare autosomal-recessive neurologic disorder clinically characterized with progeriod features, growth failure, and photosensitivity. However, it remains poorly understood how the CSB enzymatic activity is regulated for CSB to perform its desired biological functions. Here, we reveal the molecular mechanism of autoinhibition of CSB enzymatic activities. We identify a novel conserved motif (leucine latch motif) that interacts with lobe 2 of the core ATPase domain and hinge region and locks the enzyme into an inactive conformation as a latch. This work provides important novel mechanistic insights into the autoregulation of CSB/ERCC6 proteins.