RT info:eu-repo/semantics/article
T1 WIP is a chaperone for Wiskott–Aldrich syndrome protein (WASP)
A1 Fuente García, Miguel Ángel de la
A1 Sasahara, Yoji
A1 Calamito, Marco
A1 Antón, Inés María
A1 Elkhal, Abdallah
A1 Gallego, María Dolores
A1 Suresh, Koduro
A1 Siminovitch, Katherine
A1 Ochs, Hans D.
A1 Anderson, Kenneth C.
A1 Rosen, Fred S.
A1 Geha, Raif S.
A1 Ramesh, Narayanaswamy
K1 Wiskott Aldrich, Síndrome protéico
AB Wiskott–Aldrich syndrome protein (WASP) is in a complex withWASP-interacting protein (WIP). WASP levels, but not mRNA levels,were severely diminished in T cells from WIP /  mice and wereincreased by introduction of WIP in these cells. The WASP bindingdomain of WIP was shown to protect WASP from degradation bycalpain in vitro. Treatment with the proteasome inhibitors MG132and bortezomib increased WASP levels in T cells from WIP /  miceand in T and B lymphocytes from two WAS patients with missensemutations (R86H and T45M) that disrupt WIP binding. The calpaininhibitor calpeptin increased WASP levels in activated T and B cellsfrom the WASP patients, but not in primary T cells from the patientsor from WIP /  mice. Despite its ability to increase WASP levelsproteasome inhibition did not correct the impaired IL-2 geneexpression and low F-actin content in T cells from the R86H WASpatient. These results demonstrate that WIP stabilizes WASP andsuggest that it may also be important for its function
PB National Academy of Sciences
SN 0027-8424
YR 2007
FD 2007
LK http://uvadoc.uva.es/handle/10324/9826
UL http://uvadoc.uva.es/handle/10324/9826
LA eng
NO Proceedings of the National Academy of Sciences U S A. 2007 Jan 16;104(3):926-31
NO Producción Científica
DS UVaDOC
RD 25-abr-2024