Skip to main content
SpringerLink
Log in

Reduced phospholipase activity, peptide storage and the pathogenesis of canine neuronal ceroid-lipofuscinosis

  • NCL Symposium — Biochemical And Molecular Advances
  • Published:
Journal of Inherited Metabolic Disease

This is a preview of subscription content, log in via an institution to check access.

Access this article

Log in via an institution

Price excludes VAT (USA)
Tax calculation will be finalised during checkout.

Instant access to the full article PDF.

Institutional subscriptions

References

  • Dawson G (1982) Evidence for two distinct forms of mammalian β-mannosidase.J Biol Chem 257: 3369–3371.

    Google Scholar 

  • Dawson G, Dawson SA, Siakotos AN (1990) Phospholipases and the molecular basis for formation of ceroid in Batten disease.Adv Exp Biol Med 266: 259–271.

    Google Scholar 

  • Hall NA, Patrick AD (1988) Accumulation of dolichol-linked oligosaccharides in ceroid lipofuscinosis.Am J Med Genet Suppl 5: 222–232.

    Google Scholar 

  • Hershko A (1988) Ubiquitin-mediated protein degradation.J Biol Chem 263: 15237–15240.

    Google Scholar 

  • Hostetler KY, Gardiner MF, Giordano JR (1986) Purification of lysosomal phospholipase A and demonstration of proteins that inhibit phospholipase A in a lysosomal fraction from rat kidney cortex.Biochemistry 25: 6456–6461.

    Google Scholar 

  • Jolly RD (1993)J Inher Metab Dis 16 280–283.

    Google Scholar 

  • Katz ML, Rodrigues M (1991) Juvenile ceroid lipofuscinosis: Evidence for methyllysine in neural storage body protein.Am J Pathol 138: 323–332.

    Google Scholar 

  • Leighton F, Poole B, Beaufay RL (1968) The large-scale separation of peroxisomes, mitochondria, and lysosomes from the livers of rats injected with Triton WR-1339.J Cell Biol 37: 482–513.

    Google Scholar 

  • Palmer DN, Martius RD, Cooper SM, Midwinter GG, Reid JC, Jolly RD (1989) Ovine ceroid lipofuscinosis. The major lipopigment and the lipid-binding subunit of mitochondrial ATP synthase have the same NH2-terminal sequence.J Biol Chem 264: 5736–5740.

    Google Scholar 

  • Palmer DN, Fearnley IM, Walker JE et al (1992) Mitochondrial ATP synthase storage in the ceroid lipofuscinoses (Batten disease).Am J Med Genet 42: 561–567.

    Google Scholar 

  • Palmer DN et al (1993)J Inher Metab Dis 16 292–295.

    Google Scholar 

  • Rider JA, Dawson G, Siakotos AN (1992) Perspective of biochemical research in the neuronal ceroid lipofuscinoses.Am J Med Genet 42: 519–524.

    Google Scholar 

  • Schagger H, von Jagow G (1987) Tricine-sodium dodecylsulfate-polyacrylamide gel electrophoresis for the separation of proteins in the range from 1 to 100 kDa.Anal Biochem 166: 368–379.

    Google Scholar 

  • Siakotos AN, Bray R, Dratz E, van Kuijk E, Sevainen A, Koppang N (1988) 4-Hydroxynonenal: a specific indicator for canine neuronal retinal ceroidosis.Am J. Med Genet 5(Suppl): 171–181.

    Google Scholar 

Download references

Author information

Authors and Affiliations

  1. Departments of Pediatrics, Biochemistry and Molecular Biology, University of Chicago, 60637, Chicago, IL, USA

    G. Dawson & J. Kilkus

  2. Department of Pathology, Indiana University School of Medicine, 46223, Indianapolis, IN, USA

    A. N. Siakotos

Authors
  1. G. Dawson
    View author publications

    You can also search for this author in PubMed Google Scholar

  2. J. Kilkus
    View author publications

    You can also search for this author in PubMed Google Scholar

  3. A. N. Siakotos
    View author publications

    You can also search for this author in PubMed Google Scholar

Rights and permissions

Reprints and permissions

About this article

Cite this article

Dawson, G., Kilkus, J. & Siakotos, A.N. Reduced phospholipase activity, peptide storage and the pathogenesis of canine neuronal ceroid-lipofuscinosis. J Inherit Metab Dis 16, 330–334 (1993). https://doi.org/10.1007/BF00710276

Download citation

  • Issue Date:

  • DOI: https://doi.org/10.1007/BF00710276

Keywords

Search

Navigation