Stuart Moore
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View article: An ALG12-CDG patient with a novel homozygous intronic mutation associated with low ALG12 mRNA
An ALG12-CDG patient with a novel homozygous intronic mutation associated with low ALG12 mRNA Open
View article: Transport of <i>N</i>-acetylchitooligosaccharides and fluorescent <i>N</i>-acetylchitooligosaccharide analogs into rat liver lysosomes
Transport of <i>N</i>-acetylchitooligosaccharides and fluorescent <i>N</i>-acetylchitooligosaccharide analogs into rat liver lysosomes Open
Free polymannose-type oligosaccharides (fOS) are processed by cytosolic enzymes to generate Man5GlcNAc which is transferred to lysosomes and degraded. Lysosomal fOS import was demonstrated in vitro but is poorly characterized in part due t…
View article: A national flood awareness system for ungauged catchments in complex topography for Aoetearoa New Zealand.
A national flood awareness system for ungauged catchments in complex topography for Aoetearoa New Zealand. Open
Floods cause over $40 Billion of damage worldwide every year. In Aotearoa New Zealand, it is the most frequent natural disaster, with an average annual cost of NZ$100 million for residential properties. Effectively forecasting and communic…
View article: Design of a Small-Scale Supercritical Water Oxidation Reactor. Part II: Numerical Modeling and Validation
Design of a Small-Scale Supercritical Water Oxidation Reactor. Part II: Numerical Modeling and Validation Open
The experimental data from the laboratory-scale supercritical water oxidation reactor was leveraged to validate the CFD approach allowing for efficient and accurate modeling of the process. The reactor operating on ethanol as a pilot fuel …
View article: Design of a Small-Scale Supercritical Water Oxidation Reactor. Part II: Numerical Modeling and Validation
Design of a Small-Scale Supercritical Water Oxidation Reactor. Part II: Numerical Modeling and Validation Open
The experimental data from the laboratory-scale supercritical water oxidation reactor was leveraged to validate the CFD approach allowing for efficient and accurate modeling of the process. The reactor operating on ethanol as a pilot fuel …
View article: Design of a Small-Scale Supercritical Water Oxidation Reactor. Part I: Experimental Performance and Characterization
Design of a Small-Scale Supercritical Water Oxidation Reactor. Part I: Experimental Performance and Characterization Open
A small-scale supercritical water oxidation reactor is designed and fabricated to study the destruction of hazardous wastes. The downward bulk flow is heated with the introduction of pilot fuel (ethanol/water mixture), and oxidant (H2…
View article: Synthetic Route to Glycosyl β-1C-(phosphino)-phosphonates as Unprecedented Stable Glycosyl Diphosphate Analogs and Their Preliminary Biological Evaluation
Synthetic Route to Glycosyl β-1C-(phosphino)-phosphonates as Unprecedented Stable Glycosyl Diphosphate Analogs and Their Preliminary Biological Evaluation Open
The synthesis of glycosyl-β-1C-(phosphino)-phosphonates is a challenge since it has not yet been described. In this paper, we report an innovative synthetic method for their preparation from Glc-, Man-, and GlcNAc- lactone derivatives. The…
View article: Defects in Galactose Metabolism and Glycoconjugate Biosynthesis in a UDP-Glucose Pyrophosphorylase-Deficient Cell Line Are Reversed by Adding Galactose to the Growth Medium
Defects in Galactose Metabolism and Glycoconjugate Biosynthesis in a UDP-Glucose Pyrophosphorylase-Deficient Cell Line Are Reversed by Adding Galactose to the Growth Medium Open
UDP-glucose (UDP-Glc) is synthesized by UGP2-encoded UDP-Glc pyrophosphorylase (UGP) and is required for glycoconjugate biosynthesis and galactose metabolism because it is a uridyl donor for galactose-1-P (Gal1P) uridyltransferase. Chinese…
View article: Bacterial Lipid II Analogs: Novel In Vitro Substrates for Mammalian Oligosaccharyl Diphosphodolichol Diphosphatase (DLODP) Activities
Bacterial Lipid II Analogs: Novel In Vitro Substrates for Mammalian Oligosaccharyl Diphosphodolichol Diphosphatase (DLODP) Activities Open
Mammalian protein N-glycosylation requires the transfer of an oligosaccharide containing 2 residues of N-acetylglucosamine, 9 residues of mannose and 3 residues of glucose (Glc3Man9 GlcNAc2) from Glc3Man9GlcNAc2-diphospho (PP)-dolichol (DL…
View article: Wide clinical spectrum in ALG8-CDG: clues from molecular findings suggest an explanation for a milder phenotype in the first-described patient
Wide clinical spectrum in ALG8-CDG: clues from molecular findings suggest an explanation for a milder phenotype in the first-described patient Open
View article: A case of fatal Type I congenital disorders of glycosylation (CDG I) associated with low dehydrodolichol diphosphate synthase (DHDDS) activity
A case of fatal Type I congenital disorders of glycosylation (CDG I) associated with low dehydrodolichol diphosphate synthase (DHDDS) activity Open
View article: Brefeldin A promotes the appearance of oligosaccharyl phosphates derived from Glc3Man9GlcNAc2-PP-dolichol within the endomembrane system of HepG2 cells
Brefeldin A promotes the appearance of oligosaccharyl phosphates derived from Glc3Man9GlcNAc2-PP-dolichol within the endomembrane system of HepG2 cells Open
We reported an oligosaccharide diphosphodolichol (DLO) diphosphatase (DLODP) that generates dolichyl-phosphate and oligosaccharyl phosphates (OSPs) from DLO in vitro. This enzyme could underlie cytoplasmic OSP generation and promote dolich…
View article: Demonstration of an oligosaccharide-diphosphodolichol diphosphatase activity whose subcellular localization is different than those of dolichyl-phosphate-dependent enzymes of the dolichol cycle
Demonstration of an oligosaccharide-diphosphodolichol diphosphatase activity whose subcellular localization is different than those of dolichyl-phosphate-dependent enzymes of the dolichol cycle Open
View article: Abnormal Glycosylation Profile and High Alpha-Fetoprotein in a Patient with Twinkle Variants
Abnormal Glycosylation Profile and High Alpha-Fetoprotein in a Patient with Twinkle Variants Open