Alveolar Macrophage Dysregulation in Hermansky-Pudlak Syndrome Type 1

AUTOR(ES)

Rouhani, Farshid N.

FONTE

American Thoracic Society

RESUMO

Rationale: Individuals with Hermansky-Pudlak syndrome type 1 (HPS-1), an autosomal recessive disorder characterized by defective biogenesis of lysosome-related organelles, develop an accelerated form of progressive fibrotic lung disease. The etiology of pulmonary fibrosis associated with HPS-1 is unknown.

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