Pro-fibrotic phenotype of human Hermansky-Pudlak syndrome lung fibroblasts

B. Gochuico (Bethesda, United States of America), J. Wang (Bethesda, United States of America), L. Smith (Bethesda, United States of America), J. Abdalla (Bethesda, United States of America), M. Zuo (Bethesda, United States of America), T. Franklin (Bethesda, United States of America), S. Abudi (Bethesda, United States of America), R. Cinar (Bethesda, United States of America), M. Malicdan (Bethesda, United States of America), C. Han (Bethesda, United States of America)

Source: Virtual Congress 2021 – Molecular pathology and cell biology of pulmonary diseases
Session: Molecular pathology and cell biology of pulmonary diseases
Session type: E-poster
Number: 3613

Congress or journal article abstractE-poster

Rating: 0
You must login to grade this presentation.

Share or cite this content

Citations should be made in the following way:
B. Gochuico (Bethesda, United States of America), J. Wang (Bethesda, United States of America), L. Smith (Bethesda, United States of America), J. Abdalla (Bethesda, United States of America), M. Zuo (Bethesda, United States of America), T. Franklin (Bethesda, United States of America), S. Abudi (Bethesda, United States of America), R. Cinar (Bethesda, United States of America), M. Malicdan (Bethesda, United States of America), C. Han (Bethesda, United States of America). Pro-fibrotic phenotype of human Hermansky-Pudlak syndrome lung fibroblasts. 3613

You must login to share this Presentation/Article on Twitter, Facebook, LinkedIn or by email.

Member's Comments

No comment yet.
You must Login to comment this presentation.


Related content which might interest you:
Deficient autophagy in Hermansky-Pudlak syndrome associated interstitial pneumonia (HPSIP)
Source: Annual Congress 2013 –The vulnerable patient and ageing cells
Year: 2013

Hermansky-Pudlak syndrome type 4 with interstitial pneumonia
Source: Annual Congress 2013 –Diffuse parenchymal lung disease I
Year: 2013


Early pulmonary phenotype in Hermansky-Pudlak syndrome: Analysis of the chILD-EU registry
Source: International Congress 2016 – Paediatric bronchology I
Year: 2016


Suppression of epithelial abnormalities by nintedanib in induced-rheumatoid arthritis-associated interstitial lung disease mouse model
Source: ERJ Open Res, 7 (4) 00345-2021; 10.1183/23120541.00345-2021
Year: 2021



Reactivation of developmental pathways in idiopathic pulmonary fibrosis: FGF9 and FGF18 modulate the phenotype of control and fibrotic human lung fibroblasts in vitro
Source: International Congress 2015 – IPF: from bench to bedside
Year: 2015

Establishment of alveolar epithelial progenitor cells from the lung with ABCA3 mutations
Source: International Congress 2015 – Pulmonary fibrosis: mechanisms of disease
Year: 2015


Ehlers-Danlos syndrome type IV with few extrathoracic findings: a newly recognized point mutation in the COL3A1 gene
Source: Eur Respir J 2002; 19: 195-198
Year: 2002



Mesenchymal Tsc2 deficiency causes retarded lung alveolarization and polycystic kidney lesion
Source: International Congress 2017 – Molecular pathways in lung development
Year: 2017

Hypereosinophilic syndromes and lung involvement
Source: Eur Respir Monogr 2022; 95: 153-176
Year: 2022


The lung in the antiphospholipid syndrome
Source: Eur Respir J 2004; 24: Suppl. 48, 536s
Year: 2004

Cellular origin of pro-coagulant and (anti)-fibrinolytic factors in bleomycin-injured lungs
Source: Eur Respir J 2007; 29: 1105-1114
Year: 2007



Progressive phenotype in primary Sjögren's syndrome-associated interstitial lung diseases: Prognostic significance
Source: Virtual Congress 2020 – Diagnostic and prognostic markers for disease-associated interstitial lung disease and hypersensitivity pneumonitis
Year: 2020

A rare diagnosis: Hermansky-Pudlak syndrome in a patient with pulmonary fibrosis, oculocutaneous albinism and thrombocytopathy
Source: International Congress 2019 – The changing face of rare diffuse parenchymal lung diseases: recent advances in pathogenesis, clinical manifestations and treatment
Year: 2019

Lung fibroblast function in patients with Birt-Hogg-Dubé syndrome
Source: Annual Congress 2012 - COPD inflammation and genes
Year: 2012

Hereditary pulmonary alveolar proteinosis caused by recessive CSF2RB mutations
Source: Eur Respir J 2011; 37: 201-204
Year: 2011


Identification of KCNRG, a bronchial autoantigen, in a children with IPEX syndrome
Source: Annual Congress 2012 - Paediatric bronchology
Year: 2012


Mesenchymal stem cells isolated from fibrotic human lung tissue inhibit collagen expression by lung fibroblasts
Source: International Congress 2016 – ILD: from the bench to the bedside
Year: 2016

Lung abnormalities in marfan syndrome: a pathogenetic model of early-onset emphysema?
Source: Annual Congress 2009 - Respiratory physiology
Year: 2009

Induction of mesenchymal cell phenotypes in lung epithelial cells by adenovirus E1A
Source: Eur Respir J 2006; 28: 1106-1117
Year: 2006



Alveolar lymphocyte phenotype and respiratory distress syndrome
Source: Annual Congress 2011 - Lung cell injury and repair
Year: 2011