Aspartylglycosaminuria: An inborn error of glycoprotein catabolism

ASPARTYLGLYCOSAMINURIA

1968 • 266 citations

Glycoprotein catabolism in rat liver. Lysosomal digestion of iodinated asialo-fetuin

1975 • 145 citations

Structure of a Carbohydrate Moiety of a Unit A Glycopeptide of Calf Thyroglobulin1

1977 • 106 citations

Enzymatic cleavage of glycopeptides

1966 • 100 citations

Endoglycosidases acting on carbohydrate moieties of glycoproteins: Demonstration in mammalian tissue

1974 • 94 citations

Cytosolic location of an endo-N-acetyl-β-D-glucosaminidase activity in rat liver and kidney

1979 • 91 citations

An improved thin-layer chromatographic method for urinary oligosaccharide screening

1979 • 82 citations

The purification and properties of a β-aspartyl N-acetylglucosylamine amidohydrolase from hen oviduct

1969 • 80 citations

β-Aspartylglucosylamine Amido Hydrolase of Rat Liver and Kidney

1967 • 77 citations

[lysosomes and storage disease].

1981 • 70 citations

The glycoasparagines in urine of a patient with aspartylglycosaminuria

1974 • 52 citations

Structural Determination of Three Glycoasparagines Isolated from the Urine of a Patient with Aspartylglycosaminuria

1976 • 40 citations

Enzymatic Diagnosis and Carrier Detection of Aspartylglucosaminuria Using Blood Samples

1976 • 40 citations

Aspartylglycosaminuria: A generalized storage disease

1975 • 37 citations

Enzymatic cleavage of 2-acetamido-1-(β'-l-aspartamido)-1,2-dideoxy-β-d-glucose by human plasma and seminal fluid. Failure to detect the heterozygous state for aspartylgly-cosaminuria

1969 • 37 citations

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

1973 • 35 citations

High-performance liquid chromatographic analysis of oligosaccharides and glycopeptides accumulating in lysosomal storage disorders

1980 • 35 citations

Aspartylglucosaminuria: Deficiency of aspartylglucosaminidase in cultured fibroblasts of patients and their heterozygous parents

1973 • 33 citations

Aspartylglycosaminuria. Analysis of thirty-four patients.

1972 • 33 citations

ELEVEN NEW CASES OF ASPARTYLGLUCOSAMINURIA*

1970 • 33 citations

Distribution, purification and properties of 1-aspartamido-β-N-acetylglucosamine amidohydrolase

1969 • 33 citations

Aspartylglucosaminuria II: biochemical studies on brain, liver, kidney and spleen

1972 • 32 citations

Detection of heterozygotes for aspartylglucosaminuria (AGU) in cultured fibroblasts

1974 • 30 citations

Characterization of One Neutral and Two Acidic Glycoasparagines Isolated from the Urine of Patients with Aspartylglycosylaminuria (AGU)1

1976 • 29 citations

Purification and some properties of 1-aspartamido-β-N-acetylglucosamine amidohydrolase from human liver

1977 • 26 citations

Distribution of cytoplasmic vacuoles in blood T and B lymphocytes in two lysosomal disorders

1975 • 26 citations

Distribution of glycopeptide-degrading enzyme in tissue and cells

1968 • 24 citations

Thin-layer chromatographic demonstration of aspartylglycosylamine and a novel acidic carbohydrate in human tissues

1972 • 19 citations

Accumulation of two glycoasparagines in the liver in aspartylglycosaminuria.

1979 • 18 citations

Accumulation of glycoprotein-derived metabolites in neural and visceral tissue in aspartylglycosaminuria.

1980 • 17 citations

Aspartylglycosaminuria (AGU). Further aspects on its clinical picture, mode of inheritance and epidemiology based on a series of 57 patients.

1973 • 16 citations

Quantitative determination of 4-N-2-acetamido-2-deoxy-beta-D-glucopyranosyl-L-asparagine in the urine of patients with aspartylglycosaminuria by gas-liquid chromatography.

1979 • 15 citations

Characterization of Two Glycoasparagines Isolated from the Urine of Patients with Aspartylglycosylaminuria (AGU)1

1975 • 14 citations

Characterization of a mannose‐containing glycoasparagine isolated from urine of a patient with aspartylglycosylaminuria (AGU)

1976 • 13 citations

Variation of urinary excretion of aspartylglucosamine and associated clinical findings in aspartylglucosaminuria

1980 • 12 citations

Measurement of 1-aspartamido-β-N-acetylglucosamine amidohydrolase activity in human tissues

1977 • 10 citations

Identification of in biological materials by gas chromatography-mass spectrometry

1979 • 10 citations

Isolation of 2-acetamido-1- -(L- -aspartamido)-1,2-dideoxy-D-glucose from normal human urine.

1972 • 9 citations

Isolation of 2-Acetamido-1-β-(L-β-Aspartamido)-1,2-Dideoxy-D-Glucose from Normal Human Urine

1972 • 9 citations

Isolation of the liver N-aspartyl-β-glucosaminidase in aspartylglucosaminuria

1976 • 7 citations

Enzymatic determination of urinary aspartylglycosylamine: A rapid and sensitive method to detect aspartylclycosylaminuria (AGU)

1976 • 7 citations

N-Acetylglucosamine-asparagine levels in tissues of patients with aspartylglycosaminuria

1980 • 7 citations

Characterization of the Storage Material of Peripheral Lymphocytes in Aspartylglycosaminuria

1980 • 7 citations

Studies on N-aspartyl-β-glucosaminidase in aspartylglycosaminuria

1975 • 5 citations

Regional distribution of glycoasparagine storage material in the brain in aspartylglycosaminuria

1981 • 5 citations

Urinary sialic acid levels in aspartylglycosaminuria

1981 • 4 citations

Automated ion-exchange chromatography in the detection of aspartylglucosaminuria

1980 • 3 citations

Detection of aspartylglycosaminuria by gas--liquid chromatography.

1981 • 3 citations