Aberrant lung structure, composition, and function in a murine model of Hermansky-Pudlak syndrome. - Wikidata - awgldk - TriplyDB

Aberrant lung structure, composition, and function in a murine model of Hermansky-Pudlak syndrome.

Aberrant lung structure, composition, and function in a murine model of Hermansky-Pudlak syndrome.

http://www.wikidata.org/entity/Q40584900

about

Genetic interstitial lung disease Abnormal epithelial structure and chronic lung inflammation after repair of chlorine-induced airway injury Orphan G protein-coupled receptor GPR116 regulates pulmonary surfactant pool size Rab38 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 organelles Chronic 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 pneumonia TLR and NKG2D signaling pathways mediate CS-induced pulmonary pathologies Early 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 disease Murine 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 lungs CD8+ 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 disease Increased 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 pollution Lung-restricted macrophage activation in the pearl mouse model of Hermansky-Pudlak syndrome.Defective surfactant secretion in a mouse model of Hermansky-Pudlak syndrome Pseudomonas aeruginosa exotoxin pyocyanin causes cystic fibrosis airway pathogenesis.Regulation of secretory granule size by the precise generation and fusion of unit granules Pulmonary 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.

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P2860

Aberrant lung structure, composition, and function in a murine model of Hermansky-Pudlak syndrome.

http://www.wikidata.org/entity/Q40584900

description

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2003年论文

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Aberrant lung structure, compo ...... of Hermansky-Pudlak syndrome.

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American Journal of Physiology - Lung Cellular and Molecular Physiology

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Aberrant lung structure, compo ...... of Hermansky-Pudlak syndrome.

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Edward K Novak

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Gerald Jahreis

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Jian Tan

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Michael E Rusiniak

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Richard T Swank

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Timothy A Lyerla

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P2860

The beta3A subunit gene (Ap3b1) of the AP-3 adaptor complex is altered in the mouse hypopigmentation mutant pearl, a model for Hermansky-Pudlak syndrome and night blindness

Altered trafficking of lysosomal proteins in Hermansky-Pudlak syndrome due to mutations in the beta 3A subunit of the AP-3 adaptor

BLOC-1, a novel complex containing the pallidin and muted proteins involved in the biogenesis of melanosomes and platelet-dense granules

Pallidin is a component of a multi-protein complex involved in the biogenesis of lysosome-related organelles

Positional cloning of a gene for Hermansky-Pudlak syndrome, a disorder of cytoplasmic organelles

Functional redundancy of Rab27 proteins and the pathogenesis of Griscelli syndrome

The mouse organellar biogenesis mutant buff results from a mutation in Vps33a, a homologue of yeast vps33 and Drosophila carnation

The gene for the muted (mu) mouse, a model for Hermansky-Pudlak syndrome, defines a novel protein which regulates vesicle trafficking

Altered stability of pulmonary surfactant in SP-C-deficient mice

Mutation in AP-3 delta in the mocha mouse links endosomal transport to storage deficiency in platelets, melanosomes, and synaptic vesicles

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HPS1, biogenesis of lysosomal organelles complex 3 subunit 1

Adaptor-related protein complex 3, beta 1 subunit