Failure to eliminate a phosphorylated glucose analog leads to neutropenia in patients with G6PT and G6PC3 deficiency.
Animals
Antiporters
/ metabolism
Cell Death
/ physiology
Cell Line
Endoplasmic Reticulum
/ metabolism
Female
Glucose
/ metabolism
Glucose-6-Phosphatase
/ metabolism
HEK293 Cells
Humans
Male
Mice
Mice, Inbred C57BL
Monosaccharide Transport Proteins
/ metabolism
Neutropenia
/ metabolism
Neutrophils
/ metabolism
Phosphorylation
/ physiology
Rats, Wistar
1,5-anhydroglucitol
SLGT2 inhibitors
glucose-6-phosphatase-β
metabolite repair
neutropenia
Journal
Proceedings of the National Academy of Sciences of the United States of America
ISSN: 1091-6490
Titre abrégé: Proc Natl Acad Sci U S A
Pays: United States
ID NLM: 7505876
Informations de publication
Date de publication:
22 01 2019
22 01 2019
Historique:
pubmed:
11
1
2019
medline:
26
3
2019
entrez:
11
1
2019
Statut:
ppublish
Résumé
Neutropenia represents an important problem in patients with genetic deficiency in either the glucose-6-phosphate transporter of the endoplasmic reticulum (G6PT/SLC37A4) or G6PC3, an endoplasmic reticulum phosphatase homologous to glucose-6-phosphatase. While affected granulocytes show reduced glucose utilization, the underlying mechanism is unknown and causal therapies are lacking. Using a combination of enzymological, cell-culture, and in vivo approaches, we demonstrate that G6PT and G6PC3 collaborate to destroy 1,5-anhydroglucitol-6-phosphate (1,5AG6P), a close structural analog of glucose-6-phosphate and an inhibitor of low-
Identifiants
pubmed: 30626647
pii: 1816143116
doi: 10.1073/pnas.1816143116
pmc: PMC6347702
doi:
Substances chimiques
Antiporters
0
Monosaccharide Transport Proteins
0
SLC37A4 protein, human
0
Glucose-6-Phosphatase
EC 3.1.3.9
G6PC3 protein, human
EC 3.1.3.9.
Glucose
IY9XDZ35W2
Types de publication
Journal Article
Research Support, Non-U.S. Gov't
Langues
eng
Sous-ensembles de citation
IM
Pagination
1241-1250Informations de copyright
Copyright © 2019 the Author(s). Published by PNAS.
Déclaration de conflit d'intérêts
The authors declare no conflict of interest.
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