نتایج جستجو برای: aldrich

تعداد نتایج: 3555  

Journal: :Indian Journal of Trauma and Emergency Pediatrics 2020

Journal: :Cell 2007
Rashmi Ahuja Roser Pinyol Nicole Reichenbach Laura Custer John Klingensmith Michael M. Kessels Britta Qualmann

Despite the wealth of different actin structures formed, only two actin nucleation factors are well established in vertebrates: the Arp2/3 complex and formins. Here, we describe a further nucleator, cordon-bleu (Cobl). Cobl is a brain-enriched protein using three Wiskott-Aldrich syndrome protein homology 2 (WH2) domains for actin binding. Cobl promotes nonbundled, unbranched filaments. Filament...

2010
Michael P. Blundell Austen Worth Gerben Bouma Adrian J. Thrasher

Wiskott-Aldrich syndrome (WAS) is a rare X-linked recessive primary immunodeficiency characterised by immune dysregulation, microthrombocytopaenia, eczema and lymphoid malignancies. Mutations in the WAS gene can lead to distinct syndrome variations which largely, although not exclusively, depend upon the mutation. Premature termination and deletions abrogate Wiskott-Aldrich syndrome protein (WA...

Journal: :Proceedings of the National Academy of Sciences of the United States of America 2007
Miguel A de la Fuente Yoji Sasahara Marco Calamito Inés M Antón Abdallah Elkhal Maria D Gallego Koduru Suresh Katherine Siminovitch Hans D Ochs Kenneth C Anderson Fred S Rosen Raif S Geha Narayanaswamy Ramesh

Wiskott-Aldrich syndrome protein (WASP) is in a complex with WASP-interacting protein (WIP). WASP levels, but not mRNA levels, were severely diminished in T cells from WIP(-/-) mice and were increased by introduction of WIP in these cells. The WASP binding domain of WIP was shown to protect WASP from degradation by calpain in vitro. Treatment with the proteasome inhibitors MG132 and bortezomib ...

Journal: :Cell 2002
Brian F. Volkman Kenneth E. Prehoda Jessica A. Scott Francis C. Peterson Wendell A. Lim

Missense mutants that cause the immune disorder Wiskott-Aldrich Syndrome (WAS) map primarily to the Enabled/VASP homology 1 (EVH1) domain of the actin regulatory protein WASP. This domain has been implicated in both peptide and phospholipid binding. We show here that the N-WASP EVH1 domain does not bind phosphatidyl inositol-(4,5)-bisphosphate, as previously reported, but does specifically bind...

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