2024-03-29T14:54:52Zhttps://uvadoc.uva.es/oai/requestoai:uvadoc.uva.es:10324/98262021-06-23T09:49:21Zcom_10324_1133com_10324_931com_10324_894col_10324_1209
WIP is a chaperone for Wiskott–Aldrich syndrome protein (WASP)
Fuente García, Miguel Ángel de la
Sasahara, Yoji
Calamito, Marco
Antón, Inés María
Elkhal, Abdallah
Gallego, María Dolores
Suresh, Koduro
Siminovitch, Katherine
Ochs, Hans D.
Anderson, Kenneth C.
Rosen, Fred S.
Geha, Raif S.
Ramesh, Narayanaswamy
Wiskott Aldrich, Síndrome protéico
Producción Científica
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 increased WASP levels in T cells from WIP / mice
and in T and B lymphocytes from two WAS patients with missense
mutations (R86H and T45M) that disrupt WIP binding. The calpain
inhibitor calpeptin increased WASP levels in activated T and B cells
from the WASP patients, but not in primary T cells from the patients
or from WIP / mice. Despite its ability to increase WASP levels
proteasome inhibition did not correct the impaired IL-2 gene
expression and low F-actin content in T cells from the R86H WAS
patient. These results demonstrate that WIP stabilizes WASP and
suggest that it may also be important for its function
2015-03-23T12:36:37Z
2015-03-23T12:36:37Z
2007
info:eu-repo/semantics/article
Proceedings of the National Academy of Sciences U S A. 2007 Jan 16;104(3):926-31
0027-8424
http://uvadoc.uva.es/handle/10324/9826
10.1073/pnas.0610275104
926
3
931
Proceedings of the National Academy of Sciences
104
eng
https://www.pnas.org/content/104/3/926
Attribution-NonCommercial-NoDerivatives 4.0 International
info:eu-repo/semantics/openAccess
http://creativecommons.org/licenses/by-nc-nd/4.0/
application/pdf
National Academy of Sciences
https://uvadoc.uva.es/bitstream/10324/9826/6/8.M.%20Angel%20de%20Fuente%20PD-221.pdf.jpg
Hispana
TEXT
http://creativecommons.org/licenses/by-nc-nd/4.0/
UVaDOC. Repositorio Documental de la Universidad de Valladolid
http://uvadoc.uva.es/handle/10324/9826