HPA010928

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Showing 1-13 of 13 results for "HPA010928" within Papers

Recapitulating idiopathic pulmonary fibrosis related alveolar epithelial dysfunction in a human iPSC-derived air-liquid interface model.

Eva Schruf et al.

FASEB journal : official publication of the Federation of American Societies for Experimental Biology, 34(6), 7825-7846 (2020-04-17)

Idiopathic pulmonary fibrosis (IPF) is a fatal disease of unknown cause that is characterized by progressive fibrotic lung remodeling. An abnormal emergence of airway epithelial-like cells within the alveolar compartments of the lung, herein termed bronchiolization, is often observed in

Integrative analysis of cell state changes in lung fibrosis with peripheral protein biomarkers.

Christoph H Mayr et al.

EMBO molecular medicine, 13(4), e12871-e12871 (2021-03-03)

The correspondence of cell state changes in diseased organs to peripheral protein signatures is currently unknown. Here, we generated and integrated single-cell transcriptomic and proteomic data from multiple large pulmonary fibrosis patient cohorts. Integration of 233,638 single-cell transcriptomes (n = 61) across

Recombinant BRICHOS chaperone domains delivered to mouse brain parenchyma by focused ultrasound and microbubbles are internalized by hippocampal and cortical neurons

Galan-Acosta L, et al.

Molecular and Cellular Neurosciences, 103498-103498 (2020)

Genetic deletions in sputum as diagnostic markers for early detection of stage I non-small cell lung cancer.

Ruiyun Li et al.

Clinical cancer research : an official journal of the American Association for Cancer Research, 13(2 Pt 1), 482-487 (2007-01-27)

Analysis of molecular genetic markers in biological fluids has been proposed as a powerful tool for cancer diagnosis. We have characterized in detail the genetic signatures in primary non-small cell lung cancer, which provided potential diagnostic biomarkers for lung cancer.

Nonspecific interstitial pneumonia, alveolar proteinosis, and abnormal proprotein trafficking resulting from a spontaneous mutation in the surfactant protein C gene.

Paul A Stevens et al.

Pediatric research, 57(1), 89-98 (2004-11-24)

Human surfactant protein C (hSP-C(1-197)) is synthesized as a 197 amino acid proprotein and cleaved to a mature 3.7 kD form. Although interstitial lung disease in patients with mutations of the hSP-C gene is becoming increasingly recognized, the mechanisms linking

Alveolar regeneration through a Krt8+ transitional stem cell state that persists in human lung fibrosis.

Maximilian Strunz et al.

Nature communications, 11(1), 3559-3559 (2020-07-18)

The cell type specific sequences of transcriptional programs during lung regeneration have remained elusive. Using time-series single cell RNA-seq of the bleomycin lung injury model, we resolved transcriptional dynamics for 28 cell types. Trajectory modeling together with lineage tracing revealed

Recapitulating idiopathic pulmonary fibrosis related alveolar epithelial dysfunction in a human iPSC-derived air-liquid interface model

Schruf E, et al.

Faseb Journal, 103498-103498 (2020)

The surfactant protein C mutation A116D alters cellular processing, stress tolerance, surfactant lipid composition, and immune cell activation.

Ralf Zarbock et al.

BMC pulmonary medicine, 12, 15-15 (2012-03-31)

Surfactant protein C (SP-C) is important for the function of pulmonary surfactant. Heterozygous mutations in SFTPC, the gene encoding SP-C, cause sporadic and familial interstitial lung disease (ILD) in children and adults. Mutations mapping to the BRICHOS domain located within

Combined pulmonary fibrosis and emphysema syndrome associated with familial SFTPC mutation.

Vincent Cottin et al.

Thorax, 66(10), 918-919 (2011-01-21)

Surfactant protein C G100S mutation causes familial pulmonary fibrosis in Japanese kindred.

S Ono et al.

The European respiratory journal, 38(4), 861-869 (2011-08-11)

Several mutations in the surfactant protein C (SP-C) gene (SFTPC) have been reported as causing familial pulmonary fibrosis (FPF). However, the genetic background and clinical features of FPF are still not fully understood. We identified one Japanese kindred, in which

Variable phenotype associated with SP-C gene mutations: fatal case with the I73T mutation.

S Percopo et al.

The European respiratory journal, 24(6), 1072-1073 (2004-12-02)

Interstitial lung disease in a baby with a de novo mutation in the SFTPC gene.

F Brasch et al.

The European respiratory journal, 24(1), 30-39 (2004-08-06)

Mutations in the surfactant protein C gene (SFTPC) were recently reported in patients with interstitial lung disease. In a 13-month-old infant with severe respiratory insufficiency, a lung biopsy elicited combined histological patterns of nonspecific interstitial pneumonia and pulmonary alveolar proteinosis.

Antigen retrieval and clearing for whole-organ immunofluorescence by FLASH.

Hendrik A Messal et al.

Nature protocols, 16(1), 239-262 (2020-11-29)

Advances in light-sheet and confocal microscopy now allow imaging of cleared large biological tissue samples and enable the 3D appreciation of cell and protein localization in their native organ environment. However, the sample preparations for such imaging are often onerous

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