Isolation and characterization of major urinary amino acid O-glycosides and a dipeptide O-glycoside from a new lysosomal storage disorder (Kanzaki disease). Excessive excretion of serine- and threonine-linked glycan in the patient urine.

@article{Hirabayashi1990IsolationAC,
  title={Isolation and characterization of major urinary amino acid O-glycosides and a dipeptide O-glycoside from a new lysosomal storage disorder (Kanzaki disease). Excessive excretion of serine- and threonine-linked glycan in the patient urine.},
  author={Yoshio Hirabayashi and Yukie Matsumoto and Marissa L. Matsumoto and Toshihiko Toida and Noriko Iida and T Matsubara and Tamotsu Kanzaki and Michiko Yokota and Ineo Ishizuka},
  journal={The Journal of biological chemistry},
  year={1990},
  volume={265 3},
  pages={
          1693-701
        }
}
Isolation and Characterization of Major Urinary Amino Acid O-Glycosides and a Dipeptide O-Glycoside from a New Lysosomal Storage Disorder (Kanzaki Disease)
Yoshio Hirabayashi@, Yukie Matsumoto$, Makoto Matsumoto$, Toshihiko Toidall, Naoko Iidan, Toshiko Matsubaraii , Tamotsu Kanzaki**, Michiko Yokota**, and Ineo Ishizukaq From the $Department of
Characterization of four monosialo and a novel disialo Asn N-glycosides from the urine of a patient with aspartylglycosaminuria
TLDR
A novel disialo Asn N-glycoside (AG-5) has been isolated from the urine of one of the patients with aspartylglycosaminuria by gel filtration and anion exchange chromatography in this study.
Release of O-glycans by Enzymatic Methods
Endo-type glycosidase is an indispensable tool for obtaining an intact sugar chain from glycoprotein and also to prepare intact deglycosylated proteins. To release the O-glycan sugar chain from
The molecular lesion in the alpha-N-acetylgalactosaminidase gene that causes angiokeratoma corporis diffusum with glycopeptiduria.
TLDR
To identify the mutation causing the phenotypically distinct adult-onset disorder, Southern and Northern hybridization analyses of DNA and RNA from the affected homozygote were performed which revealed a grossly normal alpha-GalNAc gene structure and normal transcript size and abundancy.
One‐Pot Synthesis of Sialo‐Containing Glycosyl Amino Acids by Use of an N‐Trichloroethoxycarbonyl‐β‐thiophenyl Sialoside
TLDR
Both protected glycosyl amino acids were converted into the fully deprotected 2,6- and 2,3-sialyl T antigens linked to serine in good yields.
Structural and immunocytochemical studies on α-N-acetylgalactosaminidase deficiency (Schindler/Kanzaki disease)
TLDR
Structural and immunocytochemical studies suggest that a prototype of α-NAGA deficiency in Kanzaki disease and factors other than the defect of α -NAGA may contribute to severe neurological disorders, and KanZaki disease is thought to be caused by a single enzyme deficiency.
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