(Q33939364)

30 July 2017

title

Impairment of the Golgi GDP-L-fucose transport and unresponsiveness to fucose replacement therapy in LAD II patients (English)

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30 July 2017

Berninsone P

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22 October 2018

Sturla L

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30 July 2017

Puglielli L

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30 July 2017

Tonetti M

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30 July 2017

Hirschberg CB

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30 July 2017

De Flora A

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30 July 2017

Etzioni A

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30 July 2017

language of work or name

English

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publication date

1 April 2001

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30 July 2017

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30 July 2017

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30 July 2017

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30 July 2017

page(s)

537-542

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Brief report: recurrent severe infections caused by a novel leukocyte adhesion deficiency

30 July 2017

cites work

1 reference

https://api.crossref.org/works/10.1203%2F00006450-200104000-00016

Rambam-Hasharon syndrome of psychomotor retardation, short stature, defective neutrophil motility, and Bombay phenotype

21 January 2018

1 reference

https://api.crossref.org/works/10.1203%2F00006450-200104000-00016

Leukocyte Adhesion Deficiency Type II is a generalized defect of de novo GDP-fucose biosynthesis. Endothelial cell fucosylation is not required for neutrophil rolling on human nonlymphoid endothelium

21 January 2018

1 reference

https://api.crossref.org/works/10.1203%2F00006450-200104000-00016

ELAM-1 Mediates Cell Adhesion by Recognition of a Carbohydrate Ligand, Sialyl-Le x

21 January 2018

1 reference

https://api.crossref.org/works/10.1203%2F00006450-200104000-00016

21 January 2018

1 reference

https://api.crossref.org/works/10.1203%2F00006450-200104000-00016

Selectin ligands, leukocyte trafficking, and fucosyltransferase genes

21 January 2018

1 reference

https://api.crossref.org/works/10.1203%2F00006450-200104000-00016

In vivo behavior of neutrophils from two patients with distinct inherited leukocyte adhesion deficiency syndromes

21 January 2018

1 reference

https://api.crossref.org/works/10.1203%2F00006450-200104000-00016

Neutrophil adhesion in leukocyte adhesion deficiency syndrome type 2

21 January 2018

1 reference

https://api.crossref.org/works/10.1203%2F00006450-200104000-00016

Molecular cloning of human GDP-mannose 4,6-dehydratase and reconstitution of GDP-fucose biosynthesis in vitro

21 January 2018

1 reference

https://api.crossref.org/works/10.1203%2F00006450-200104000-00016

Molecular cloning and expression of GDP-D-mannose-4,6-dehydratase, a key enzyme for fucose metabolism defective in Lec13 cells

21 January 2018

1 reference

https://api.crossref.org/works/10.1203%2F00006450-200104000-00016

Structural and enzymatic characterization of human recombinant GDP-D-mannose-4,6-dehydratase.

21 January 2018

1 reference

https://api.crossref.org/works/10.1203%2F00006450-200104000-00016

Synthesis of GDP-L-fucose by the human FX protein

21 January 2018

1 reference

https://api.crossref.org/works/10.1203%2F00006450-200104000-00016

Defective intracellular activity of GDP-D-mannose-4,6-dehydratase in leukocyte adhesion deficiency type II syndrome

21 January 2018

1 reference

https://api.crossref.org/works/10.1203%2F00006450-200104000-00016

A rapid and sensitive method for the quantitation of microgram quantities of protein utilizing the principle of protein-dye binding

21 January 2018

1 reference

https://api.crossref.org/works/10.1203%2F00006450-200104000-00016

Purification to apparent homogeneity and properties of pig kidney L-fucose kinase

21 January 2018

1 reference

https://api.crossref.org/works/10.1203%2F00006450-200104000-00016

GDP-L-fucose pyrophosphorylase. Purification, cDNA cloning, and properties of the enzyme

21 January 2018

1 reference

https://api.crossref.org/works/10.1203%2F00006450-200104000-00016

Transporters of nucleotide sugars, ATP, and nucleotide sulfate in the endoplasmic reticulum and Golgi apparatus

21 January 2018

1 reference

https://api.crossref.org/works/10.1203%2F00006450-200104000-00016

Transport of UDP-galactose into the Golgi lumen regulates the biosynthesis of proteoglycans

21 January 2018

1 reference

https://api.crossref.org/works/10.1203%2F00006450-200104000-00016

10.1203/00006450-200104000-00016

21 January 2018

Identifiers

DOI

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Dimensions Publication ID

30 July 2017

1029983475

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1 reference