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Related Experiment Videos

Mouse "protective protein". cDNA cloning, sequence comparison, and expression.

N J Galjart1, N Gillemans, D Meijer

  • 1Department of Cell Biology and Genetics, Erasmus University, Rotterdam, The Netherlands.

The Journal of Biological Chemistry
|March 15, 1990
PubMed
Summary
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Researchers identified the murine protective protein cDNA, revealing high conservation with human sequences. This protein precursor corrects enzyme deficiencies in galactosialidosis cells, offering potential therapeutic insights.

Area of Science:

  • Biochemistry
  • Genetics
  • Molecular Biology

Background:

  • The protective protein is a glycoprotein essential for lysosomal enzyme function.
  • Deficiency of protective protein causes the genetic disorder galactosialidosis.
  • Human protective protein shares homology with yeast serine carboxypeptidases.

Purpose of the Study:

  • To isolate and characterize the full-length cDNA encoding murine protective protein.
  • To investigate the conservation and functional domains between human and murine protective proteins.
  • To assess the therapeutic potential of murine protective protein in correcting galactosialidosis.

Main Methods:

  • cDNA cloning and sequencing for murine protective protein.
  • Sequence homology analysis between human and mouse proteins.

Related Experiment Videos

  • Transient expression in COS-1 cells and functional assays in fibroblasts.
  • Northern blot analysis for tissue-specific mRNA expression.

    • Main Results:

    • Murine protective protein cDNA was isolated, showing high nucleotide and amino acid sequence conservation with humans.
    • Protease functional domains are identical between human and mouse proteins.
    • Expressed murine protective protein precursor corrects beta-galactosidase and neuraminidase activities in galactosialidosis fibroblasts.
    • Tissue-specific expression patterns of protective protein mRNA were observed in mice.

    Conclusions:

    • The high conservation suggests a critical role for protective protein in mammals.
    • Murine protective protein demonstrates potential for gene therapy in galactosialidosis.
    • Further research into tissue-specific expression may elucidate disease mechanisms.