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Article

Inflammatory response and cathepsins in silica-exposed Hermansky-Pudlak syndrome model pale ear mice.

Pathology international - 1 May 2004

Yoshioka Yasuko, Kumasaka Toshio, Ishidoh Kazumi, Kominami Eiki, Mitani Keiko, Hosokawa Yoshinori, Fukuchi Yoshinosuke

Abstract excerpt

Hermansky-Pudlak syndrome (HPS) is a hereditary disorder involving the sorting processes of intracellular organelles such as lysosomes of reticuloendothelial cells. Pale ear (ep) mouse is known to have the HPS1 gene mutation, which is seen in patients with HPS and pulmonary fibrosis. As pulmonary fibrosis is not spontaneously observed in ep mice, we hypothesized that external stimuli are necessary for the genetic...

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Source

Identifiers

PMID: 15086836
DOI: 10.1111/j.1440-1827.2004.01626.x

Genes

HPS1

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.

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