RESUMEN

X-linked adrenoleukodystrophy (X-ALD) is a severe neurological disease characterized by progressive demyelination within the CNS, adrenal insufficiency, and is associated with an accumulation of saturated very long chain fatty acids in plasma and tissues of patients. iNKT cells, a distinct lineage of T cells recognizing glycolipid antigens through CD1d molecules, exert immunoregulatory functions and can prevent various immune mediated-pathologies. In ALD patients, but not in ALD deficient mice, iNKT cell frequency and CD1d expression on the surface of B cells are slightly decreased. However, such minor differences might not influence the pathogenesis of the disease.

Asunto(s)

Adrenoleucodistrofia/patología , Células T Asesinas Naturales/clasificación , Células T Asesinas Naturales/fisiología , Adolescente , Adulto , Anciano , Animales , Células Presentadoras de Antígenos/fisiología , Antígenos CD1/metabolismo , Antígenos CD1d/metabolismo , Brefeldino A/farmacología , Niño , Citocinas/metabolismo , Modelos Animales de Enfermedad , Citometría de Flujo/métodos , Glicoproteínas/metabolismo , Glicoesfingolípidos/metabolismo , Humanos , Metabolismo de los Lípidos/efectos de los fármacos , Masculino , Ratones , Ratones Endogámicos C57BL , Persona de Mediana Edad , Células T Asesinas Naturales/efectos de los fármacos , Inhibidores de la Síntesis de la Proteína/farmacología , Adulto Joven