Failure to eliminate a phosphorylated glucose analog leads to neutropenia in patients with G6PT and G6PC3 deficiency

Nit1 is a metabolite repair enzyme that hydrolyzes deaminated glutathione

(2017) Alessio Peracchi et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

NAXE Mutations Disrupt the Cellular NAD(P)HX Repair System and Cause a Lethal Neurometabolic Disorder of Early Childhood

(2016) Laura S. Kremer et al.   AMERICAN JOURNAL OF HUMAN GENETICS

A conserved phosphatase destroys toxic glycolytic side products in mammals and yeast

(2016) François Collard et al.   Nature Chemical Biology

The Structural and Functional Characterization of Mammalian ADP-dependent Glucokinase

(2015) Jan P. Richter et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

A Mouse Model of L-2-Hydroxyglutaric Aciduria, a Disorder of Metabolite Repair

(2015) Rim Rzem et al.   PLoS One

Metabolite Proofreading in Carnosine and Homocarnosine Synthesis

(2014) Maria Veiga-da-Cunha et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Metabolite proofreading, a neglected aspect of intermediary metabolism

(2013) Emile Van Schaftingen et al.   JOURNAL OF INHERITED METABOLIC DISEASE

Metabolite damage and its repair or pre-emption

(2013) Carole L Linster et al.   Nature Chemical Biology

Glucose-6-phosphatase- , implicated in a congenital neutropenia syndrome, is essential for macrophage energy homeostasis and functionality

(2012) H. S. Jun et al.   BLOOD

T cell Activation Is Driven by an ADP-Dependent Glucokinase Linking Enhanced Glycolysis with Mitochondrial Reactive Oxygen Species Generation

(2012) Marcin M. Kamiński et al.   Cell Reports

G6PC3 mutations are associated with a major defect of glycosylation: a novel mechanism for neutrophil dysfunction

(2011) B. Hayee et al.   GLYCOBIOLOGY

A Novel GDP-d-glucose Phosphorylase Involved in Quality Control of the Nucleoside Diphosphate Sugar Pool inCaenorhabditis elegansand Mammals

(2011) Lital N. Adler et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Ethylmalonyl-CoA Decarboxylase, a New Enzyme Involved in Metabolite Proofreading

(2011) Carole L. Linster et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

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

(2010) H. S. Jun et al.   BLOOD

SGLT2 inhibition — a novel strategy for diabetes treatment

(2010) Edward C. Chao et al.   NATURE REVIEWS DRUG DISCOVERY

A Syndrome with Congenital Neutropenia and Mutations inG6PC3

(2009) Kaan Boztug et al.   NEW ENGLAND JOURNAL OF MEDICINE

The anhydrofructose pathway of glycogen catabolism

(2008) Shukun Yu   IUBMB LIFE