Phosphorylation of WASp is a key regulator of activity and stability in vivo

被引:48
作者
Blundell, Michael P. [1 ,2 ,3 ]
Bouma, Gerben [1 ,2 ,3 ]
Metelo, Joao [1 ,2 ,3 ]
Worth, Austen [1 ,2 ,3 ]
Calle, Yolanda [4 ]
Cowell, Lucy A. [1 ,2 ,3 ]
Westerberg, Lisa S. [5 ]
Moulding, Dale A. [1 ,2 ,3 ]
Mirando, Samuel [1 ,2 ,3 ]
Kinnon, Christine [1 ,2 ,3 ]
Cory, Giles O. [6 ]
Jones, Gareth E. [7 ]
Snapper, Scott B. [5 ]
Burns, Siobhan O. [1 ,2 ,3 ]
Thrasher, Adrian J. [1 ,2 ,3 ]
机构
[1] UCL Inst Child Hlth, Mol Immunol Unit, Wolfson Ctr Gene Therapy Childhood Dis, London WC1N 1EH, England
[2] UCL Inst Child Hlth, Ctr Immunodeficiency, London WC1N 1EH, England
[3] Great Ormond St Hosp Sick Children, London WC1N 3JH, England
[4] Kings Coll London, Dept Haematol Med, Rayne Inst, London SE5 9NU, England
[5] Harvard Univ, Massachusetts Gen Hosp, Sch Med, Dept Med,GI Unit, Boston, MA 02114 USA
[6] Peninsula Coll Med & Dent, Peninsula Med Sch, Exeter EX1 2LU, Devon, England
[7] Kings Coll London, Randall Div Cell & Mol Biophys, London SE1 1UL, England
基金
英国惠康基金;
关键词
actin polymerization; immune deficiency; Wiskott-Aldrich syndrome; WISKOTT-ALDRICH-SYNDROME; INTERFERENCE REFLECTION MICROSCOPY; SYNDROME PROTEIN WASP; X-LINKED NEUTROPENIA; B-CELL ACTIVATION; N-WASP; T-CELL; CONGENITAL NEUTROPENIA; IMMUNOLOGICAL SYNAPSE; ACTIN POLYMERIZATION;
D O I
10.1073/pnas.0904346106
中图分类号
O [数理科学和化学]; P [天文学、地球科学]; Q [生物科学]; N [自然科学总论];
学科分类号
070301 [无机化学]; 070403 [天体物理学]; 070507 [自然资源与国土空间规划学]; 090105 [作物生产系统与生态工程];
摘要
The Wiskott-Aldrich syndrome protein (WASp) is a key cytoskeletal regulator in hematopoietic cells. Covalent modification of a conserved tyrosine by phosphorylation has emerged as an important potential determinant of activity, although the physiological significance remains uncertain. In a murine knockin model, mutation resulting in inability to phosphorylate Y293 (Y293F) mimicked many features of complete WASp-deficiency. Although a phosphomimicking mutant Y293E conferred enhanced actin-polymerization, the cellular phenotype was similar due to functional dysregulation. Furthermore, steady-state levels of Y293E-WASp were markedly reduced compared to wild-type WASp and Y293F-WASp, although partially recoverable by treatment of cells with proteasome inhibitors. Consequently, tyrosine phosphorylation plays a critical role in normal activation of WASp in vivo, and is indispensible for multiple tasks including proliferation, phagocytosis, chemotaxis, and assembly of adhesion structures. Furthermore, it may target WASp for proteasome-mediated degradation, thereby providing a default mechanism for self-limiting stimulation of the Arp2/3 complex.
引用
收藏
页码:15738 / 15743
页数:6
相关论文
共 41 条
[1]
Two novel activating mutations in the Wiskott-Aldrich syndrome protein result in congenital neutropenia [J].
Ancliff, Phil J. ;
Blundell, Michael P. ;
Cory, Giles O. ;
Calle, Yolanda ;
Worth, Austen ;
Kempski, Helena ;
Burns, Siobhan ;
Jones, Gareth E. ;
Sinclair, Jo ;
Kinnon, Christine ;
Hann, Ian M. ;
Gale, Rosemary E. ;
Linch, David C. ;
Thrasher, Adrian J. .
BLOOD, 2006, 108 (07) :2182-2189
[2]
WIP deficiency reveals a differential role for WIP and the actin cytoskeleton in T and B cell activation [J].
Antón, IM ;
de la Fuente, MA ;
Sims, TN ;
Freeman, S ;
Ramesh, N ;
Hartwig, JH ;
Dustin, ML ;
Geha, RS .
IMMUNITY, 2002, 16 (02) :193-204
[3]
Involvement of the Wiskott-Aldrich syndrome protein and other actin regulatory adaptors in T cell activation [J].
Badour, K ;
Zhang, JY ;
Siminovitch, KA .
SEMINARS IN IMMUNOLOGY, 2004, 16 (06) :395-407
[4]
A large kindred with X-linked neutropenia with an I294T mutation of the Wiskott-Aldrich syndrome gene [J].
Beel, Karolien ;
Cotter, Melanie M. ;
Blatny, Jan ;
Bond, Jonathan ;
Lucas, Geoff ;
Green, Frances ;
Vanduppen, Vik ;
Leung, Daisy W. ;
Rooney, Sean ;
Smith, Owen P. ;
Rosen, Michael K. ;
Vandenberghe, Peter .
BRITISH JOURNAL OF HAEMATOLOGY, 2009, 144 (01) :120-126
[5]
Improvement of migratory defects in a murine model of Wiskott-Aldrich syndrome gene therapy [J].
Blundell, Michael P. ;
Bouma, Gerben ;
Calle, Yolanda ;
Jones, Gareth E. ;
Kinnon, Christine ;
Thrasher, Adrian J. .
MOLECULAR THERAPY, 2008, 16 (05) :836-844
[6]
Fyn and PTP-PEST-mediated regulation of Wiskott-Aldrich syndrome protein (WASp) tyrosine phosphorylation is required for coupling T cell antigen receptor engagement to WASp effector function and T cell activation [J].
Bodour, K ;
Zhang, JY ;
Shi, F ;
Leng, S ;
Collins, M ;
Siminovitch, KA .
JOURNAL OF EXPERIMENTAL MEDICINE, 2004, 199 (01) :99-111
[7]
Configuration of human dendritic cell cytoskeleton by Rho GTPases, the WAS protein, and differentiation [J].
Burns, S ;
Thrasher, AJ ;
Blundell, MP ;
Machesky, L ;
Jones, GE .
BLOOD, 2001, 98 (04) :1142-1149
[8]
Inhibition of calpain stabilises podosomes and impairs dendritic cell motility [J].
Calle, Yolanda ;
Carragher, Neil O. ;
Thrasher, Adrian J. ;
Jones, Gareth E. .
JOURNAL OF CELL SCIENCE, 2006, 119 (11) :2375-2385
[9]
Phosphorylation of a Wiscott-Aldrich syndrome protein-associated signal complex is critical in osteoclast bone resorption [J].
Chellaiah, Meenakshi A. ;
Kuppuswamy, Dhandapani ;
Lasky, Larry ;
Linder, Stefan .
JOURNAL OF BIOLOGICAL CHEMISTRY, 2007, 282 (13) :10104-10116
[10]
WIP regulates the stability and localization of WASP to podosomes in migrating dendritic cells [J].
Chou, Hsiu-Chuan ;
Anton, Ines M. ;
Holt, Mark R. ;
Curcio, Claudia ;
Lanzardo, Stefania ;
Worth, Austen ;
Burns, Siobhan ;
Thrasher, Adrian J. ;
Jones, Gareth E. ;
Calle, Yolanda .
CURRENT BIOLOGY, 2006, 16 (23) :2337-2344