Aberrant lung structure, composition, and function in a murine model of Hermansky-Pudlak syndrome (Q40584900)

21 September 2017

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

1 reference

HPS1, biogenesis of lysosomal organelles complex 3 subunit 1

21 September 2017

1 reference

GOA release 2020-03-11

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GOA release 2020-03-11

author name string

Timothy A Lyerla

1

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21 September 2017

Michael E Rusiniak

2

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21 September 2017

Michael Borchers

3

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21 September 2017

Gerald Jahreis

4

1 reference

21 September 2017

Jian Tan

5

1 reference

21 September 2017

Patricia Ohtake

6

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21 September 2017

Edward K Novak

7

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21 September 2017

Richard T Swank

8

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21 September 2017

30 May 2003

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American Journal of Physiology - Lung Cellular and Molecular Physiology

21 September 2017

1 reference

21 September 2017

285

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21 September 2017

3

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21 September 2017

L643-53

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

21 September 2017

1 reference

https://api.crossref.org/works/10.1152%2FAJPLUNG.00024.2003

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

21 January 2018

1 reference

https://api.crossref.org/works/10.1152%2FAJPLUNG.00024.2003

New concepts in chronic obstructive pulmonary disease

21 January 2018

1 reference

https://api.crossref.org/works/10.1152%2FAJPLUNG.00024.2003

Functional redundancy of Rab27 proteins and the pathogenesis of Griscelli syndrome

21 January 2018

1 reference

https://api.crossref.org/works/10.1152%2FAJPLUNG.00024.2003

Defective lysosomal enzyme secretion in kidneys of Chediak-Higashi (beige) mice

21 January 2018

1 reference

https://api.crossref.org/works/10.1152%2FAJPLUNG.00024.2003

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.

21 January 2018

1 reference

https://api.crossref.org/works/10.1152%2FAJPLUNG.00024.2003

Lysosome-related organelles

21 January 2018

1 reference

https://api.crossref.org/works/10.1152%2FAJPLUNG.00024.2003

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

21 January 2018

1 reference

https://api.crossref.org/works/10.1152%2FAJPLUNG.00024.2003

Rab geranylgeranyl transferase alpha mutation in the gunmetal mouse reduces Rab prenylation and platelet synthesis

21 January 2018

1 reference

https://api.crossref.org/works/10.1152%2FAJPLUNG.00024.2003

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

21 January 2018

1 reference

https://api.crossref.org/works/10.1152%2FAJPLUNG.00024.2003

Mouse pale ear (ep) is homologous to human Hermansky-Pudlak syndrome and contains a rare 'AT-AC' intron.

21 January 2018

1 reference

https://api.crossref.org/works/10.1152%2FAJPLUNG.00024.2003

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

21 January 2018

1 reference

https://api.crossref.org/works/10.1152%2FAJPLUNG.00024.2003

On the imperfect elasticity of lung tissue

21 January 2018

1 reference

https://api.crossref.org/works/10.1152%2FAJPLUNG.00024.2003

The mouse pale ear (ep) mutation is the homologue of human Hermansky-Pudlak syndrome

21 January 2018

1 reference

https://api.crossref.org/works/10.1152%2FAJPLUNG.00024.2003

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

21 January 2018

1 reference

https://api.crossref.org/works/10.1152%2FAJPLUNG.00024.2003

Comparative respiratory system mechanics in rodents

21 January 2018

1 reference

https://api.crossref.org/works/10.1152%2FAJPLUNG.00024.2003

The pallid gene encodes a novel, syntaxin 13-interacting protein involved in platelet storage pool deficiency

21 January 2018

1 reference

https://api.crossref.org/works/10.1152%2FAJPLUNG.00024.2003

Hermansky-Pudlak syndrome: vesicle formation from yeast to man.

21 January 2018

1 reference

https://api.crossref.org/works/10.1152%2FAJPLUNG.00024.2003

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

21 January 2018

1 reference

https://api.crossref.org/works/10.1152%2FAJPLUNG.00024.2003

Sorting out signals in fly endosomes

21 January 2018

1 reference

https://api.crossref.org/works/10.1152%2FAJPLUNG.00024.2003

The pathogenesis of pulmonary fibrosis: is there a fibrosis gene?

21 January 2018

1 reference

https://api.crossref.org/works/10.1152%2FAJPLUNG.00024.2003

Pulmonary pathologies in pallid mice result from nonhematopoietic defects.

21 January 2018

1 reference

https://api.crossref.org/works/10.1152%2FAJPLUNG.00024.2003

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

21 January 2018

1 reference

https://api.crossref.org/works/10.1152%2FAJPLUNG.00024.2003

Interstitial pneumonia in Hermansky-Pudlak syndrome: significance of florid foamy swelling/degeneration (giant lamellar body degeneration) of type-2 pneumocytes.

21 January 2018

1 reference

https://api.crossref.org/works/10.1152%2FAJPLUNG.00024.2003

The regulation of platelet-dense granules by Rab27a in the ashen mouse, a model of Hermansky-Pudlak and Griscelli syndromes, is granule-specific and dependent on genetic background

21 January 2018

1 reference

https://api.crossref.org/works/10.1152%2FAJPLUNG.00024.2003

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

21 January 2018

1 reference

https://api.crossref.org/works/10.1152%2FAJPLUNG.00024.2003

Susceptibility of lysosomes to rupture is a determinant for plasma membrane disruption in tumor necrosis factor alpha-induced cell death

21 January 2018

1 reference

https://api.crossref.org/works/10.1152%2FAJPLUNG.00024.2003

Overexpression of TGF-alpha increases lung tissue hysteresivity in transgenic mice.

21 January 2018

1 reference

https://api.crossref.org/works/10.1152%2FAJPLUNG.00024.2003

21 January 2018

Adaptor-related proteins

1 reference

https://api.crossref.org/works/10.1152%2FAJPLUNG.00024.2003

Multi-organellar disorders of pigmentation: intracellular traffic jams in mammals, flies and yeast

21 January 2018

1 reference

https://api.crossref.org/works/10.1152%2FAJPLUNG.00024.2003

Normal and abnormal secretion by haemopoietic cells

21 January 2018

1 reference

https://api.crossref.org/works/10.1152%2FAJPLUNG.00024.2003

The gene mutated in cocoa mice, carrying a defect of organelle biogenesis, is a homologue of the human Hermansky-Pudlak syndrome-3 gene

21 January 2018

1 reference

https://api.crossref.org/works/10.1152%2FAJPLUNG.00024.2003

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

21 January 2018

1 reference

https://api.crossref.org/works/10.1152%2FAJPLUNG.00024.2003

Response of alveolar cells to mechanical stress

21 January 2018

1 reference

https://api.crossref.org/works/10.1152%2FAJPLUNG.00024.2003

Biogenesis of lamellar bodies, lysosome-related organelles involved in storage and secretion of pulmonary surfactant

21 January 2018

1 reference

https://api.crossref.org/works/10.1152%2FAJPLUNG.00024.2003

A mutation in Rab27a causes the vesicle transport defects observed in ashen mice

21 January 2018

1 reference

https://api.crossref.org/works/10.1152%2FAJPLUNG.00024.2003

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

21 January 2018

1 reference

https://api.crossref.org/works/10.1152%2FAJPLUNG.00024.2003

Ru2 and Ru encode mouse orthologs of the genes mutated in human Hermansky-Pudlak syndrome types 5 and 6

21 January 2018

1 reference

https://api.crossref.org/works/10.1152%2FAJPLUNG.00024.2003

10.1152/AJPLUNG.00024.2003

21 January 2018

Identifiers

DOI

1 reference

21 September 2017

1 reference