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Article

Natural killer cell activity and dysfunction in Hermansky-Pudlak syndrome.

British journal of haematology - 1 Jan 2017

Gil-Krzewska Aleksandra, Murakami Yousuke, Peruzzi Giovanna, O'Brien Kevin J, Merideth Melissa A, Cullinane Andrew R, Gahl William A, Coligan John E, Gochuico Bernadette R, Krzewski Konrad

Abstract excerpt

Hermansky-Pudlak syndrome (HPS) encompasses disorders with abnormal function of lysosomes and lysosome-related organelles, and some patients who develop immunodeficiency. The basic mechanisms contributing to immune dysfunction in HPS are ill-defined. We analysed natural killer (NK) cells from patients diagnosed with HPS-1, HPS-2, HPS-4, and an unreported HPS subtype. NK cells from an HPS-2 and an unreported HPS...

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Methodology Data sources Editorial policy Privacy About Contact Helena Bioinformatics

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Identifiers

PMID: 27766632
DOI: 10.1111/bjh.14390
PMCID: PMC5177518

Provenance

PubMed metadata and Literature Corpus relationships. Corpus snapshot updated 6 Sept 2026. Manifest publication-graph-cohort-2026-09-06.1.

Source receipt: pubmed, retrieved 6 Sept 2026.

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