(Q37906514)

25 August 2017

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title

The structural and molecular biology of type I galactosemia: Enzymology of galactose 1-phosphate uridylyltransferase (English)

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25 August 2017

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2

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25 August 2017

Thomas J McCorvie

1

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25 August 2017

25 July 2011

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25 August 2017

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25 August 2017

63

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25 August 2017

9

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25 August 2017

694-700

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Congenital galactosemia, a single enzymatic block in galactose metabolism

25 August 2017

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Genetic basis of transferase-deficient galactosaemia in Ireland and the population history of the Irish Travellers.

21 January 2018

1 reference

Large-scale molecular screening for galactosemia alleles in a pan-ethnic population

21 January 2018

1 reference

Low prevalence of classical galactosemia in Korean population

21 January 2018

1 reference

Molecular basis for phenotypic heterogeneity in galactosaemia: prediction of clinical phenotype from genotype in Japanese patients.

21 January 2018

1 reference

Classical galactosaemia in Chinese: A case report and review of disease incidence.

21 January 2018

1 reference

Galactokinase: structure, function and role in type II galactosemia

21 January 2018

1 reference

The structural and molecular biology of type III galactosemia.

21 January 2018

1 reference

Epimerase-deficiency galactosemia is not a binary condition

21 January 2018

1 reference

Analysis of UDP-galactose 4'-epimerase mutations associated with the intermediate form of type III galactosaemia

21 January 2018

1 reference

Classical galactosaemia revisited.

21 January 2018

1 reference

Identification and characterisation of human aldose 1-epimerase

21 January 2018

1 reference

Modulation of rat tissue galactose-1-phosphate uridyltransferase by uridine and uridine triphosphate.

21 January 2018

1 reference

Hint, Fhit, and GalT: function, structure, evolution, and mechanism of three branches of the histidine triad superfamily of nucleotide hydrolases and transferases

21 January 2018

1 reference

Galactose-1-phosphate uridylyltransferase: rate studies confirming a uridylyl-enzyme intermediate on the catalytic pathway

21 January 2018

1 reference

Galactose-1-phosphate uridylyltransferase: isolation and properties of a uridylyl-enzyme intermediate

21 January 2018

1 reference

Nucleophile in the active site of Escherichia coli galactose-1-phosphate uridylyltransferase: degradation of the uridylyl-enzyme intermediate to N3-phosphohistidine

21 January 2018

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Three-dimensional structure of galactose-1-phosphate uridylyltransferase from Escherichia coli at 1.8 A resolution

21 January 2018

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The structure of nucleotidylated histidine-166 of galactose-1-phosphate uridylyltransferase provides insight into phosphoryl group transfer

21 January 2018

1 reference

Structural analysis of the H166G site-directed mutant of galactose-1-phosphate uridylyltransferase complexed with either UDP-glucose or UDP-galactose: detailed description of the nucleotide sugar binding site

21 January 2018

1 reference

Structure and mechanism of an ADP-glucose phosphorylase from Arabidopsis thaliana

21 January 2018

1 reference

Galactose-1-phosphate uridylyltransferase from Escherichia coli, a zinc and iron metalloenzyme.

21 January 2018

1 reference

Significance of metal ions in galactose-1-phosphate uridylyltransferase: an essential structural zinc and a nonessential structural iron.

21 January 2018

1 reference

Functional requirements of the active site position 185 in the human enzyme galactose-1-phosphate uridylyltransferase.

21 January 2018

1 reference

The in vitro effects of galactose and its derivatives on rat brain Mg2+-ATPase activity

21 January 2018

1 reference

Expression of human inositol monophosphatase suppresses galactose toxicity in Saccharomyces cerevisiae: possible implications in galactosemia

21 January 2018

1 reference

ARHI: A new target of galactose toxicity in Classic Galactosemia

21 January 2018

1 reference

Involvement of endoplasmic reticulum stress in a novel Classic Galactosemia model

21 January 2018

1 reference

GALT deficiency causes UDP-hexose deficit in human galactosemic cells

21 January 2018

1 reference

Characterization of a novel biochemical abnormality in galactosemia: deficiency of glycolipids containing galactose or N-acetylgalactosamine and accumulation of precursors in brain and lymphocytes

21 January 2018

1 reference

Ganglioside patterns in galactosemia.

21 January 2018

1 reference

Clinical features of galactokinase deficiency:A review of the literature

21 January 2018

1 reference

High-throughput screening for human galactokinase inhibitors

21 January 2018

1 reference

Molecular and biochemical characterization of human galactokinase and its small molecule inhibitors

21 January 2018

1 reference

Evidence for alternate galactose oxidation in a patient with deletion of the galactose-1-phosphate uridyltransferase gene

21 January 2018

1 reference

Metabolic fate of administered [13C]galactose in tissues of galactose-1-phosphate uridyl transferase deficient mice determined by nuclear magnetic resonance.

21 January 2018

1 reference

UDP-galactose pyrophosphorylase in mice with galactose-1-phosphate uridyltransferase deficiency

21 January 2018

1 reference

21 January 2018

Identifiers

DOI

10.1002/IUB.511

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25 August 2017

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PubMed ID

21793161