نتایج جستجو برای: smarcal1

تعداد نتایج: 96  

2017
Beata S Lipska-Ziętkiewicz Jutta Gellermann Olivia Boyer Olivier Gribouval Szymon Ziętkiewicz Jameela A Kari Mohamed A Shalaby Fatih Ozaltin Jiri Dusek Anette Melk Aysun K Bayazit Laura Massella Lidia Hyla-Klekot Sandra Habbig Astrid Godron Maria Szczepańska Beata Bieniaś Dorota Drożdż Rasha Odeh Wioletta Jarmużek Katarzyna Zachwieja Agnes Trautmann Corinne Antignac Franz Schaefer

Schimke immuno-osseous dysplasia (SIOD) is a rare multisystem disorder with early mortality and steroid-resistant nephrotic syndrome (SRNS) progressing to end-stage kidney disease. We hypothesized that next-generation gene panel sequencing may unsurface oligosymptomatic cases of SIOD with potentially milder disease courses. We analyzed the renal and extrarenal phenotypic spectrum and genotype-p...

Journal: :EMBO reports 2011
Gargi Ghosal Jingsong Yuan Junjie Chen

Mutations in HepA-related protein (HARP, or SMARCAL1) cause Schimke immunoosseous dysplasia (SIOD). HARP has ATP-dependent annealing helicase activity, which helps to stabilize stalled replication forks and facilitate DNA repair during replication. Here, we show that the conserved tandem HARP (2HP) domain dictates this annealing helicase activity. Furthermore, chimeric proteins generated by fus...

Journal: :Biochimica et Biophysica Acta (BBA) - Gene Regulatory Mechanisms 2018

Journal: :Genes & development 2009
Robert Driscoll Karlene A Cimprich

In this issue of Genes & Development, four papers report that the annealing helicase HepA-related protein (HARP, also known as SMARCAL1 [SWI/SNF-related, matrix-associated, actin-dependent regulator of chromatin, subfamily a-like 1]) binds directly to the ssDNA-binding protein Replication protein A (RPA) and is recruited to sites of replicative stress. Knockdown of HARP results in hypersensitiv...

Journal: :Critical Reviews in Biochemistry and Molecular Biology 2017

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