(Q33778386)

29 July 2017

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title

Aspartylglycosaminuria: biochemistry and molecular biology (English)

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29 July 2017

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N N Aronson

1

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29 July 2017

language of work or name

English

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publication date

1 October 1999

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29 July 2017

number of pages

16

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Biochimica et Biophysica Acta

inferred from page(s)

published in

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29 July 2017

volume

1455

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29 July 2017

issue

2-3

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29 July 2017

page(s)

139-154

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Three-dimensional structure of human lysosomal aspartylglucosaminidase

29 July 2017

cites work

1 reference

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7 January 2021

Aspartylglycosaminuria. An inborn error of metabolism associated with mental defect.

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7 January 2021

Glycoasparagine metabolites in patients with aspartylglycosaminuria: comparison between english and finnish patients with special reference to storage materials

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7 January 2021

Early clinical symptoms and incidence of aspartylglucosaminuria in Finland

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7 January 2021

Fibroblast expression of collagens and proteoglycans is altered in aspartylglucosaminuria, a lysosomal storage disease.

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7 January 2021

Lysosomal storage diseases in adults

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7 January 2021

Two Japanese cases with aspartylglycosaminuria: clinical and morphological features

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7 January 2021

Amniotic fluid glycoasparagines in fetal aspartylglycosaminuria

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7 January 2021

Mammalian alpha-mannosidases--multiple forms but a common purpose?

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7 January 2021

A human lysosomal alpha(1----6)-mannosidase active on the branched trimannosyl core of complex glycans

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7 January 2021

The core-specific lysosomal alpha(1-6)-mannosidase activity depends on aspartamidohydrolase activity

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7 January 2021

Specificity studies of 4-L-aspartylglycosylamine amido hydrolase

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7 January 2021

Isolation and structure of the core oligosaccharide sequences of IgM

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7 January 2021

Influence of L-fucose attached alpha 1-->6 to the asparagine-linked N-acetylglucosamine on the hydrolysis of the N-glycosidic linkage by human glycosylasparaginase

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7 January 2021

Characterization and 400-MHz 1H-NMR analysis of urinary fucosyl glycoasparagines in fucosidosis

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7 January 2021

Cloning and sequence analysis of a cDNA for human glycosylasparaginase

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7 January 2021

Lysosomal aspartylglucosaminidase is processed to the active subunit complex in the endoplasmic reticulum

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7 January 2021

Purification and characterization of rat liver glycosylasparaginase

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7 January 2021

Linkage of aspartylglucosaminuria (AGU) to marker loci on the long arm of chromosome 4

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7 January 2021

Chromosomal localization of the human glycoasparaginase gene to 4q32?q33

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7 January 2021

Genomic structure of human lysosomal glycosylasparaginase

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7 January 2021

Expression and regulation of the human and mouse aspartylglucosaminidase gene

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7 January 2021

A mouse model for the human lysosomal disease aspartylglycosaminuria.

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7 January 2021

Mice with an aspartylglucosaminuria mutation similar to humans replicate the pathophysiology in patients

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7 January 2021

Purification, biochemistry and molecular cloning of an insect glycosylasparaginase from Spodoptera frugiperda

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7 January 2021

Molecular cloning and sequence analysis of Flavobacterium meningosepticum glycosylasparaginase: a single gene encodes the alpha and beta subunits

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7 January 2021

Molecular cloning, chromosomal assignment, and expression of the mouse aspartylglucosaminidase gene

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7 January 2021

The isolation and characterisation of a cDNA clone encoding L-asparaginase from developing seeds of lupin (Lupinus arboreus)

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7 January 2021

Crystal structure of glycosylasparaginase from Flavobacterium meningosepticum

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7 January 2021

Crystal structures of Flavobacterium glycosylasparaginase. An N-terminal nucleophile hydrolase activated by intramolecular proteolysis

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7 January 2021

Aspartylglucosaminuria among Palestinian Arabs

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7 January 2021

Primary Folding of Aspartylglucosaminidase

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7 January 2021

Localization of the disulfide bond involved in post-translational processing of glycosylasparaginase and disrupted by a mutation in the Finnish-type aspartylglycosaminuria

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7 January 2021

Convenient and quantitative determination of the frequency of a mutant allele using solid-phase minisequencing: application to aspartylglucosaminuria in Finland

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7 January 2021

Aspartylglucosaminuria in northern Norway: a molecular and genealogical study

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7 January 2021

Identification of a novel mutation causing aspartylglucosaminuria reveals a mutation hotspot region in the aspartylglucosaminidase gene

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7 January 2021

Aspartylglucosaminuria in the United States

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7 January 2021

Deletion of exon 8 causes glycosylasparaginase deficiency in an African American aspartylglucosaminuria (AGU) patient

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7 January 2021

Spectrum of mutations in aspartylglucosaminuria

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7 January 2021

Splicing defect of the glycoasparaginase gene in two Japanese siblings with apartylglucosaminuria

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7 January 2021

Characterization of three alleles causing aspartylglycosaminuria: two from a British family and one from an American patient

1 reference

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7 January 2021

Aspartylglucosaminuria in a Puerto Rican family: additional features of a panethnic disorder.

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7 January 2021

Characterization of a point mutation in aspartylglucosaminidase gene: evidence for a readthrough of a translational stop codon

1 reference

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7 January 2021

Single base deletion in exon 7 of the glycosylasparaginase gene causes a mild form of aspartylglycosaminuria in a patient of Mauritian origin

1 reference

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7 January 2021

Deletion of the C-terminal end of aspartylglucosaminidase resulting in a lysosomal accumulation disease: evidence for a unique genomic rearrangement

1 reference

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7 January 2021

Characterization and functional analysis of the cis-autoproteolysis active center of glycosylasparaginase

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7 January 2021

Aspartylglycosaminuria in the Finnish population: identification of two point mutations in the heavy chain of glycoasparaginase

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7 January 2021

Molecular background of the Finnish disease heritage

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7 January 2021

Bone-marrow transplantation in aspartylglucosaminuria

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7 January 2021

Two novel mutations in a Canadian family with aspartylglucosaminuria and early outcome post bone marrow transplantation

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7 January 2021

Correction of deficient enzyme activity in a lysosomal storage disease, aspartylglucosaminuria, by enzyme replacement and retroviral gene transfer

1 reference

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7 January 2021

Characterization of four monosialo and a novel disialo Asn N-glycosides from the urine of a patient with aspartylglycosaminuria

1 reference

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7 January 2021

Recombinant glycosylasparaginase and in vitro correction of aspartylglycosaminuria.

1 reference

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7 January 2021

Expression and endocytosis of lysosomal aspartylglucosaminidase in mouse primary neurons.

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7 January 2021

A protein catalytic framework with an N-terminal nucleophile is capable of self-activation

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7 January 2021

Lysosomal glycosylasparaginase: a member of a family of amidases that employ a processed N-terminal threonine, serine or cysteine as a combined base-nucleophile catalyst

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7 January 2021

Structure of the allosteric regulatory enzyme of purine biosynthesis

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7 January 2021

Substrate binding is required for assembly of the active conformation of the catalytic site in Ntn amidotransferases: evidence from the 1.8 A crystal structure of the glutaminase domain of glucosamine 6-phosphate synthase

1 reference

https://api.crossref.org/works/10.1016%2FS0925-4439%2899%2900076-9

7 January 2021

Crystal structure of the 20S proteasome from the archaeon T. acidophilum at 3.4 A resolution

1 reference

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7 January 2021

Activation and oligomerization of aspartylglucosaminidase.

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7 January 2021

Glycosylasparaginase-catalyzed synthesis and hydrolysis of beta-aspartyl peptides

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7 January 2021

Aspartylglycosaminuria: protein chemistry and molecular biology of the most common lysosomal storage disorder of glycoprotein degradation

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7 January 2021

Aspartylglucosaminuria (AGU): protein and gene structure of normal and mutated aspartylglucosaminidase

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7 January 2021

Mutations causing aspartylglucosaminuria (AGU): a lysosomal accumulation disease

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7 January 2021

Phenotypic characterization of mice with targeted disruption of glycosylasparaginase gene: a mouse model for aspartylglycosaminuria

1 reference

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7 January 2021

Progressive neurodegeneration in aspartylglycosaminuria mice

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7 January 2021

Monitoring the CNS pathology in aspartylglucosaminuria mice.

1 reference

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7 January 2021

Adenovirus-mediated gene transfer results in decreased lysosomal storage in brain and total correction in liver of aspartylglucosaminuria (AGU) mouse

1 reference

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7 January 2021

10.1016/S0925-4439(99)00076-9

Identifiers

DOI

1 reference

29 July 2017

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