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Human leukocyte glycosylasparaginase: cell-to-cell transfer and properties in correction of aspartylglycosaminuria.

Abstract
Aspartylglycosaminuria (AGU), a severe lysosomal storage disease, is caused by the deficiency of the lysosomal enzyme, glycosylasparaginase (GA), and accumulation of aspartylglucosamine (GlcNAc-Asn) in tissues. Here we show that human leukocyte glycosylasparaginase can correct the metabolic defect in Epstein-Barr virus (EBV)-transformed AGU lymphocytes rapidly and effectively by mannose-6-phosphate receptor-mediated endocytosis or by contact-mediated cell-to-cell transfer from normal EBV-transformed lymphocytes, and that 2-7% of normal activity is sufficient to correct the GlcNAc-Asn metabolism in the cells. Cell-to-cell contact is obligatory for the transfer of GA since normal transformed lymphocytes do not excrete GA into extracellular medium. The combined evidence indicates that cell-to-cell transfer of GA plays a main role in enzyme replacement therapy of AGU by normal lymphocytes.
AuthorsU Dunder, I Mononen
JournalFEBS letters (FEBS Lett) Vol. 499 Issue 1-2 Pg. 77-81 (Jun 15 2001) ISSN: 0014-5793 [Print] England
PMID11418116 (Publication Type: Journal Article, Research Support, Non-U.S. Gov't)
Chemical References
  • Culture Media, Conditioned
  • Mannosephosphates
  • Receptor, IGF Type 2
  • N-acetylglucosaminylasparagine
  • mannose-6-phosphate
  • Aspartylglucosylaminase
  • Acetylglucosamine
Topics
  • Acetylglucosamine (analogs & derivatives, metabolism)
  • Aspartylglucosaminuria
  • Aspartylglucosylaminase (genetics, metabolism)
  • Cell Line, Transformed
  • Coculture Techniques
  • Culture Media, Conditioned (metabolism)
  • Endocytosis (drug effects)
  • Fibroblasts (cytology, drug effects, metabolism)
  • Fluorescent Antibody Technique
  • Herpesvirus 4, Human (physiology)
  • Humans
  • Leukocytes (cytology, drug effects, enzymology, metabolism)
  • Lymphocytes (cytology, drug effects, enzymology, metabolism)
  • Lysosomal Storage Diseases (enzymology, genetics)
  • Lysosomes (drug effects, metabolism)
  • Mannosephosphates (metabolism, pharmacology)
  • Protein Transport (drug effects)
  • Receptor, IGF Type 2 (metabolism)
  • Y Chromosome (genetics)

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