Discover Community Create You

Loading publication...

Noodle always counts identifier-free feature events for service operation. With your permission, Google Analytics can also estimate audience and engagement. Search text, publication identifiers, cookies, IP addresses and browser fingerprints are never included in Noodle's first-party event counters.

Privacy details

Article

Modeling of lung phenotype of Hermansky-Pudlak syndrome type I using patient-specific iPSCs.

Respiratory research - 4 Nov 2021

Suezawa Takahiro, Kanagaki Shuhei, Korogi Yohei, Nakao Kazuhisa, Hirai Toyohiro, Murakami Koji, Hagiwara Masatoshi, Gotoh Shimpei

Abstract excerpt

BACKGROUND: Somatic cells differentiated from patient-specific human induced pluripotent stem cells (iPSCs) could be a useful tool in human cell-based disease research. Hermansky-Pudlak syndrome (HPS) is an autosomal recessive genetic disorder characterized by oculocutaneous albinism and a platelet dysfunction. HPS patients often suffer from lethal HPS associated interstitial pneumonia (HPSIP). Lung...

Read the complete abstract on PubMed

Topics

Share this publication in a Topic to start or enrich a Post.

Share to Alveolar Epithelial Cells
Share to Cells, Cultured

Methodology Data sources Editorial policy Privacy Delete account & data About Contact Helena Bioinformatics

Share to Disease Models, Animal

Share to Hermanski-Pudlak Syndrome

Share to Induced Pluripotent Stem Cells

Share to Membrane Proteins

Share to Proteomics

Source

Open in PubMed Open full text

Identifiers

PMID: 34736469
DOI: 10.1186/s12931-021-01877-8
PMCID: PMC8570015

Provenance

PubMed metadata and Literature Corpus relationships. Corpus snapshot updated 7 Sept 2026. Manifest publication-graph-cohort-2026-09-08.1.

Source receipt: pubmed, retrieved 7 Sept 2026.

Related research

Semantic proximity does not establish scientific evidence. Graph snapshot: 2026-09-29.

Research trail→

Click a neighbor to travelStep 1 · 12 closest

Modeling of lung phenotype of Hermansky-Pudlak syndrome type I using patient-specific iPSCs.PMID 34736469Read ↗

Evaluating the Use of iPSC-Derived Models in Understanding the Pathogenesis of Childhood Interstitial Lung Disease.Semantic proximity Read ↗

Hermansky-Pudlak Syndrome and Lung Disease: Pathogenesis and TherapeuticsSemantic proximity Read ↗

Modeling of fibrotic lung disease using 3D organoids derived from human pluripotent stem cellsSemantic proximity Read ↗

Modeling of Fibrotic Lung Disease Using 3D Organoids Derived from Human Pluripotent Stem CellsSemantic proximity Read ↗

Patient-specific iPSCs carrying an <i>SFTPC</i> mutation reveal the intrinsic alveolar epithelial dysfunction at the inception of interstitial lung diseaseSemantic proximity Read ↗

Human pluripotent stem cells for the modelling and treatment of respiratory diseasesSemantic proximity Read ↗

Gene-edited MLE-15 Cells as a Model for the Hermansky-Pudlak Syndromes.Semantic proximity Read ↗

A novel homozygous HPS4 mutation in Hermansky-Pudlak syndrome: case report and literature review.Semantic proximity Read ↗

A stem cell-based platform for functional analysis of genetic variants in lung diseaseSemantic proximity Read ↗

Generation of Hermansky-Pudlak Syndrome Type 1 (HPS1) induced pluripotent stem cells (iPSCs).Semantic proximity Read ↗

Induced Pluripotent Stem Cells as a Tool for Modeling Hematologic Disorders and as a Potential Source for Cell-Based TherapiesSemantic proximity Read ↗

Human hepatic organoids for the analysis of human genetic diseasesSemantic proximity Read ↗

⌁Select a neighboring publication to make it the new centre.