XBP-1 expression in common variable immunodeficiency (2002)
- Authors:
- USP affiliated authors: RIZZO, LUIZ VICENTE - ICB ; CAMARGO, MARISTELA MARTINS DE - ICB
- Unidade: ICB
- Subjects: IMUNOLOGIA; FARMACOLOGIA
- Language: Inglês
- Abstract: Common Variable Immunodeficiency (CVID) is a primary immunodeficiency characterized by total hypogammaglobulinemia or absence of at least two antibody classes in serum. This deficiency is poorly understood. We are studying an immunodeficient group with the aim to investigate the transcription factor X-box binding protein 1 (XBP-1), which is known to be essential for terminal differentiation of B lymphocytes in antibody-secreting plasma cells. Chimera mice (RAG-/- and XBP-1-/-) present severe impairment in immunoglobulin synthesis as well as plasma cell formation. Since this phenotype is also observed in our CVID patients we are analyzing the expression and functionality of XBP-1 in these patients. Peripheral blood mononuclear cells were used as protein source from twelve CVID patients (Ambulatório de Imunologia Clínica, FM-USP) and four normal individuals. Cellular lysates were prepared and XBP-1 was analyzed by SDS-PAGE followed by Western-blotRabbit anti-human XBP-1 revealed a ~30 kDa band in all patients except one. Half of our deficient patients and two controls presented also an untypical ~60 kDa band in their cells. No bands were observed in one patient suggesting a possible involvement of XBP-1 in his pathology. Recently Calfon et al. (Nature 415:92, 2002) demonstrated that cells deficient for IRE1, an endonuclease responsible for XBP-1 mRNA splicing, produce a ~54 kDa transcription factor. Considering this finding XBP-1 was immunoprecipitated from CVIDlysates and after electrophoresis the corresponding bands were excised, digested with trypsin and the amino acid sequence was analyzed in a MALDI-TOF mass spectrometer. We're currently investigating the possibility that an abnormal splicing of the XBP-1 mRNA could be one of the factors responsible for this disease
- Imprenta:
- Publisher: Comissão de Cultura e Extensão Universitária do ICB/USP
- Publisher place: São Paulo
- Date published: 2002
- Source:
- Título do periódico: Resumos
- Conference titles: Congresso Instituto Ciências Biomédicas, IV
-
ABNT
BARACHO, G V et al. XBP-1 expression in common variable immunodeficiency. 2002, Anais.. São Paulo: Comissão de Cultura e Extensão Universitária do ICB/USP, 2002. . Acesso em: 11 maio 2024. -
APA
Baracho, G. V., Errante, P. R., Kokron, C., Lamond, D., Rizzo, L. V., & Camargo, M. M. de. (2002). XBP-1 expression in common variable immunodeficiency. In Resumos. São Paulo: Comissão de Cultura e Extensão Universitária do ICB/USP. -
NLM
Baracho GV, Errante PR, Kokron C, Lamond D, Rizzo LV, Camargo MM de. XBP-1 expression in common variable immunodeficiency. Resumos. 2002 ;[citado 2024 maio 11 ] -
Vancouver
Baracho GV, Errante PR, Kokron C, Lamond D, Rizzo LV, Camargo MM de. XBP-1 expression in common variable immunodeficiency. Resumos. 2002 ;[citado 2024 maio 11 ] - Molecular characterization of common variable immunodeficiency in brazilian patients
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