(Q36850698)

16 August 2017

Pirfenidone for the treatment of Hermansky-Pudlak syndrome pulmonary fibrosis (English)

1 reference

Hermansky-Pudlak syndrome

16 August 2017

1 reference

1 reference

Kevin O'Brien

1

1 reference

16 August 2017

James Troendle

2

1 reference

16 August 2017

Bernadette R Gochuico

3

1 reference

16 August 2017

Thomas C Markello

4

1 reference

16 August 2017

Jose Salas

5

1 reference

16 August 2017

Hilda Cardona

6

1 reference

16 August 2017

Jianhua Yao

7

1 reference

16 August 2017

Isa Bernardini

8

1 reference

16 August 2017

Richard Hess

9

1 reference

16 August 2017

William A Gahl

10

1 reference

16 August 2017

21 March 2011

1 reference

Molecular Genetics and Metabolism

16 August 2017

1 reference

16 August 2017

103

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16 August 2017

2

1 reference

16 August 2017

128-134

1 reference

Non-steroid agents for idiopathic pulmonary fibrosis

16 August 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3656407

Epithelial stress and apoptosis underlie Hermansky-Pudlak syndrome-associated interstitial pneumonia

29 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3656407

Pirfenidone in idiopathic pulmonary fibrosis

29 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3656407

Disorders of lysosome-related organelle biogenesis: clinical and molecular genetics

29 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3656407

Hermansky-Pudlak syndrome: a disease of protein trafficking and organelle function

29 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3656407

Double-blind, placebo-controlled trial of pirfenidone in patients with idiopathic pulmonary fibrosis

29 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3656407

Idiopathic pulmonary fibrosis: emerging concepts on pharmacotherapy

29 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3656407

Albinism associated with hemorrhagic diathesis and unusual pigmented reticular cells in the bone marrow: report of two cases with histochemical studies

29 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3656407

Hermansky-Pudlak syndrome type 4 (HPS-4): clinical and molecular characteristics

29 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3656407

The Hermansky-Pudlak syndrome 1 (HPS1) and HPS4 proteins are components of two complexes, BLOC-3 and BLOC-4, involved in the biogenesis of lysosome-related organelles

29 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3656407

Mutation of a new gene causes a unique form of Hermansky-Pudlak syndrome in a genetic isolate of central Puerto Rico

29 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3656407

Effects of pirfenidone on procollagen gene expression at the transcriptional level in bleomycin hamster model of lung fibrosis.

29 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3656407

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

29 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3656407

Beta3A-adaptin, a subunit of the adaptor-like complex AP-3

29 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3656407

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

29 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3656407

Albinism and Hermansky-Pudlak syndrome in Puerto Rico

29 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3656407

Anti-inflammatory effect of pirfenidone in the bleomycin-hamster model of lung inflammation

29 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3656407

Progressive preclinical interstitial lung disease in rheumatoid arthritis.

27 June 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3656407

Hermansky-Pudlak syndrome: radiography and CT of the chest compared with pulmonary function tests and genetic studies.

4 September 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3656407

Effect of pirfenidone on the pulmonary fibrosis of Hermansky-Pudlak syndrome

4 September 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3656407

Pulmonary function and high-resolution CT findings in patients with an inherited form of pulmonary fibrosis, Hermansky-Pudlak syndrome, due to mutations in HPS-1.

4 September 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3656407

Intestinal disease in Hermansky-Pudlak syndrome: occurrence of colitis and relation to genotype

4 September 2018

1 reference

https://api.crossref.org/works/10.1016%2FJ.YMGME.2011.02.003

Genetic defects and clinical characteristics of patients with a form of oculocutaneous albinism (Hermansky-Pudlak syndrome)

7 January 2021

1 reference

https://api.crossref.org/works/10.1016%2FJ.YMGME.2011.02.003

Reliability of absent platelet dense bodies as a diagnostic criterion for Hermansky-Pudlak syndrome

7 January 2021

1 reference

https://api.crossref.org/works/10.1016%2FJ.YMGME.2011.02.003

Successful bilateral lung transplantation for pulmonary fibrosis associated with the Hermansky-Pudlak syndrome

7 January 2021

1 reference

https://api.crossref.org/works/10.1016%2FJ.YMGME.2011.02.003

Pirfenidone diminishes cyclophosphamide-induced lung fibrosis in mice

7 January 2021

1 reference

https://api.crossref.org/works/10.1016%2FJ.YMGME.2011.02.003

Treatment of idiopathic pulmonary fibrosis with a new antifibrotic agent, pirfenidone: results of a prospective, open-label Phase II study

7 January 2021

1 reference

https://api.crossref.org/works/10.1016%2FJ.YMGME.2011.02.003

Mouse models of Hermansky Pudlak syndrome: a review

7 January 2021

1 reference

https://api.crossref.org/works/10.1016%2FJ.YMGME.2011.02.003

10.1016/J.YMGME.2011.02.003

7 January 2021

Identifiers

DOI

1 reference

16 August 2017

1 reference

16 August 2017

PubMed publication ID

21420888

1 reference