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Wiskott-Aldrich syndrome protein

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Human WASP WH2 domain (pink) complex with α-actin (grey), DNAse I (green), ATP, Ca+2 and Mg+2 ions, glycerol and formic acid (PDB code 2a3z)

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1 Function
2 Relevance
3 Disease
4 3D Structures of Wiskott-Aldrich syndrome protein
5 References

Function

Wiskott-Aldrich syndrome protein (WASP) is involved in nucleating of new F-actin. In the autoinhibited form of WASP a region of the N-terminal interacts with a region of its C-terminal. This interaction is disrupted by CDC42 and phosphatidylinositol 4,5-bisphosphate (PIP2) resulting in the active WASP. WASP domains include EVH1 domain in the N-terminal which bind proline-rich sequences in the WASP-interacting proteins; WH2 domain is ca. 18 residues long and interacts with actin; CRIB or GTPase-binding domain which interacts with CDC42.

N-WASP (Neural WASP) belongs to the WASP family of proteins. Human N-WASP stimulates the actin-nucleating activity^[1].
WAVE-1 facilitates neuronal actin remodeling^[2].
WAVE-2 facilitates cytoskeletal actin remodeling^[3].

Relevance

N-WASP induces actin polymerization in the Shigella bacterium^[4]. This bacterium causes dysentery.

Disease

Mutations in WASP can cause Wiskott-Aldrich syndrome which is a rare inherited disease characterized by immune disregulation^[5].

3D Structures of Wiskott-Aldrich syndrome protein

Wiskott-Aldrich syndrome protein 3D structures

References

↑ Westerberg LS, Dahlberg C, Baptista M, Moran CJ, Detre C, Keszei M, Eston MA, Alt FW, Terhorst C, Notarangelo LD, Snapper SB. Wiskott-Aldrich syndrome protein (WASP) and N-WASP are critical for peripheral B-cell development and function. Blood. 2012 Apr 26;119(17):3966-74. doi: 10.1182/blood-2010-09-308197. Epub 2012 , Mar 12. PMID:22411869 doi:http://dx.doi.org/10.1182/blood-2010-09-308197
↑ Soderling SH, Guire ES, Kaech S, White J, Zhang F, Schutz K, Langeberg LK, Banker G, Raber J, Scott JD. A WAVE-1 and WRP signaling complex regulates spine density, synaptic plasticity, and memory. J Neurosci. 2007 Jan 10;27(2):355-65. PMID:17215396 doi:10.1523/JNEUROSCI.3209-06.2006
↑ Nolz JC, Gomez TS, Zhu P, Li S, Medeiros RB, Shimizu Y, Burkhardt JK, Freedman BD, Billadeau DD. The WAVE2 complex regulates actin cytoskeletal reorganization and CRAC-mediated calcium entry during T cell activation. Curr Biol. 2006 Jan 10;16(1):24-34. PMID:16401421 doi:10.1016/j.cub.2005.11.036
↑ Suzuki T, Mimuro H, Suetsugu S, Miki H, Takenawa T, Sasakawa C. Neural Wiskott-Aldrich syndrome protein (N-WASP) is the specific ligand for Shigella VirG among the WASP family and determines the host cell type allowing actin-based spreading. Cell Microbiol. 2002 Apr;4(4):223-33. PMID:11952639
↑ Notarangelo LD, Miao CH, Ochs HD. Wiskott-Aldrich syndrome. Curr Opin Hematol. 2008 Jan;15(1):30-6. PMID:18043243 doi:http://dx.doi.org/10.1097/MOH.0b013e3282f30448