Aberrant lung structure, composition, and function in a murine model of Hermansky-Pudlak syndrome.
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Genetic interstitial lung diseaseAbnormal epithelial structure and chronic lung inflammation after repair of chlorine-induced airway injuryOrphan G protein-coupled receptor GPR116 regulates pulmonary surfactant pool sizeRab38 targets to lamellar bodies and normalizes their sizes in lung alveolar type II epithelial cells.Exogenous gene transfer of Rab38 small GTPase ameliorates aberrant lung surfactant homeostasis in Ruby rats.Comparative proteomic analysis of lung lamellar bodies and lysosome-related organellesChronic cigarette smoke exposure generates pathogenic T cells capable of driving COPD-like disease in Rag2-/- mice.Epithelial stress and apoptosis underlie Hermansky-Pudlak syndrome-associated interstitial pneumoniaTLR and NKG2D signaling pathways mediate CS-induced pulmonary pathologiesEarly alveolar epithelial dysfunction promotes lung inflammation in a mouse model of Hermansky-Pudlak syndrome.The Slc35d3 gene, encoding an orphan nucleotide sugar transporter, regulates platelet-dense granules.Interstitial lung disease and pulmonary fibrosis in Hermansky-Pudlak syndrome type 2, an adaptor protein-3 complex diseaseMurine Hermansky-Pudlak syndrome genes: regulators of lysosome-related organelles.Activation of sterol-response element-binding proteins (SREBP) in alveolar type II cells enhances lipogenesis causing pulmonary lipotoxicity.Cellular stress failure in ventilator-injured lungsCD8+ T cells contribute to macrophage accumulation and airspace enlargement following repeated irritant exposure.Pulmonary fibrosis in hermansky-pudlak syndrome. a case report and review.The Peter Pan paradigm.Functional characterization of T cell populations in a mouse model of chronic obstructive pulmonary diseaseIncreased surfactant protein-D and foamy macrophages in smoking-induced mouse emphysema.Regulation of surfactant secretion in alveolar type II cells.Impaired Lysosomal Integral Membrane Protein 2-dependent Peroxiredoxin 6 Delivery to Lamellar Bodies Accounts for Altered Alveolar Phospholipid Content in Adaptor Protein-3-deficient pearl Mice.A mutation in Rab38 small GTPase causes abnormal lung surfactant homeostasis and aberrant alveolar structure in mice.Sustained CTL activation by murine pulmonary epithelial cells promotes the development of COPD-like disease.Chronic alcohol induces M2 polarization enhancing pulmonary disease caused by exposure to particulate air pollutionLung-restricted macrophage activation in the pearl mouse model of Hermansky-Pudlak syndrome.Defective surfactant secretion in a mouse model of Hermansky-Pudlak syndromePseudomonas aeruginosa exotoxin pyocyanin causes cystic fibrosis airway pathogenesis.Regulation of secretory granule size by the precise generation and fusion of unit granulesPulmonary Fibrosis in Hermansky-Pudlak Syndrome.Susceptibility of Hermansky-Pudlak mice to bleomycin-induced type II cell apoptosis and fibrosis.Histochemical and cellular changes accompanying the appearance of lung fibrosis in an experimental mouse model for Hermansky Pudlak syndrome.Hermansky-Pudlak syndrome interstitial pneumonia: it's the epithelium, stupid!Altered lung surfactant system in a Rab38-deficient rat model of Hermansky-Pudlak syndrome.A Caenorhabditis elegans model of orotic aciduria reveals enlarged lysosome-related organelles in embryos lacking umps-1 function.Insights from human genetic studies of lung and organ fibrosis.Inflammatory response and cathepsins in silica-exposed Hermansky-Pudlak syndrome model pale ear mice.Simple chronic colitis model using hypopigmented mice with a Hermansky-Pudlak syndrome 5 gene mutation.
P2860
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P2860
Aberrant lung structure, composition, and function in a murine model of Hermansky-Pudlak syndrome.
description
2003 nî lūn-bûn
@nan
2003年の論文
@ja
2003年論文
@yue
2003年論文
@zh-hant
2003年論文
@zh-hk
2003年論文
@zh-mo
2003年論文
@zh-tw
2003年论文
@wuu
2003年论文
@zh
2003年论文
@zh-cn
name
Aberrant lung structure, compo ...... of Hermansky-Pudlak syndrome.
@en
type
label
Aberrant lung structure, compo ...... of Hermansky-Pudlak syndrome.
@en
prefLabel
Aberrant lung structure, compo ...... of Hermansky-Pudlak syndrome.
@en
P2093
P2860
P1476
Aberrant lung structure, compo ...... of Hermansky-Pudlak syndrome.
@en
P2093
Edward K Novak
Gerald Jahreis
Michael Borchers
Michael E Rusiniak
Patricia Ohtake
Richard T Swank
Timothy A Lyerla
P2860
P304
P356
10.1152/AJPLUNG.00024.2003
P577
2003-05-30T00:00:00Z