Lack of glucose recycling between endoplasmic reticulum and cytoplasm underlies cellular dysfunction in glucose-6-phosphatase-β–deficient neutrophils in a congenital neutropenia syndrome

G6PC3 deficiency, characterized by neutropenia and neutrophil dysfunction, is caused by deficiencies in the endoplasmic reticulum (ER) enzyme glucose-6-phosphatase-β (G6Pase-β or G6PC3) that converts glucose-6-phosphate (G6P) into glucose, the primary energy source of neutrophils. Enhanced neutrophi...

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Detalhes bibliográficos
Main Authors: Jun, Hyun Sik, Lee, Young Mok, Cheung, Yuk Yin, McDermott, David H., Murphy, Philip M., De Ravin, Suk See, Mansfield, Brian C., Chou, Janice Y.
Formato: Artigo
Idioma:Inglês
Publicado em: American Society of Hematology 2010
Assuntos:
Phagocytes, Granulocytes, and Myelopoiesis
Acesso em linha:https://ncbi.nlm.nih.gov/pmc/articles/PMC2974586/
https://ncbi.nlm.nih.gov/pubmed/20498302
https://ncbi.nlm.nih.govhttp://dx.doi.org/10.1182/blood-2009-12-258491
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