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(Referência obtida automaticamente do Web of Science, por meio da informação sobre o financiamento pela FAPESP e o número do processo correspondente, incluída na publicação pelos autores.)

The Autoimmune Regulator (AIRE), Which Is Defective in Autoimmune Polyendocrinopathy-Candidiasis-Ectodermal Dystrophy Patients, Is Expressed in Human Epidermal and Follicular Keratinocytes and Associates With the Intermediate Filament Protein Cytokeratin 17

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Autor(es):
Kumar, Vipul [1] ; Pedroza, Luis A. [2, 1, 3] ; Mace, Emily M. [1] ; Seeholzer, Steven [1] ; Cotsarelis, George [4] ; Condino-Neto, Antonio [2] ; Payne, Aimee S. [4] ; Orange, Jordan S. [1]
Número total de Autores: 8
Afiliação do(s) autor(es):
[1] Univ Penn, Sch Med, Childrens Hosp Philadelphia, Div Immunol, Dept Pediat, Philadelphia, PA 19104 - USA
[2] Univ Sao Paulo, Inst Biomed Sci, Dept Immunol, Sao Paulo - Brazil
[3] Univ Estadual Campinas, Sch Med, Ctr Invest Pediat, Sao Paulo - Brazil
[4] Univ Penn, Sch Med, Dept Dermatol, Philadelphia, PA 19104 - USA
Número total de Afiliações: 4
Tipo de documento: Artigo Científico
Fonte: American Journal of Pathology; v. 178, n. 3, p. 983-988, MAR 2011.
Citações Web of Science: 11
Resumo

Autoimmune polyendocrinopathy-candidiasis-ectodermal dystrophy (APECED) syndrome, which is caused by mutation of the autoimmune regulator (AIRE) gene, is a highly variable disease characterized by multiple endocrine failure, chronic mucocutaneous candidiasis, and various ectodermal defects. AIRE is a transcriptional regulator classically expressed in medullary thymic epithelial cells, monocytes, macrophages, and dendritic cells. Previous studies have suggested that AIRE can shuttle between the nucleus and cytoplasm of cells, although its cytoplasmic functions are poorly characterized. Through mass spectrometry analysis of proteins co-immunoprecipitating with cytoplasmic AIRE, we identified a novel association of AIRE with the intermediate filament protein cytokeratin 17 (K17) in the THP-1 monocyte cell line. We confirmed AIRE expression in HaCaT epidermal keratinocytes, as well as its interaction with K17. Confocal microscopy of human fetal and adult scalp hair follicles demonstrated a cytoplasmic pattern of AIRE staining that moderately colocalized with K17. The cytoplasmic association of AIRE with the intermediate filament network in human epidermal and follicular keratinocytes may provide a new path to understanding the ectodermal abnormalities associated with the APECED syndrome. (Am J Pathol 2011, 178:983-988; DOI: 10.1016/j.ajpath.2010.12.007) (AU)

Processo FAPESP: 09/51747-3 - Estudo dos eventos relacionados à ativação imediata da via dectina-1 em deficiência de AIRE
Beneficiário:Luis Alberto Pedroza
Modalidade de apoio: Bolsas no Brasil - Pós-Doutorado