Biochemical Studies on Brain Explants and Fibroblast Cultures in Batten’s Disease

  • John H. Menkes
  • Don R. Harris
  • Natalie Stein
Part of the Advances in Experimental Medicine and Biology book series (AEMB, volume 19)


Batten’s disease is a syndrome of mental, motor, and visual deterioration having its onset between the second and fifth year of life. Through combined ultrastructural and biochemical examinations at least five different entities are currently known to assume a clinical course corresponding to that described by Batten in 1914 (1).


Linoleic Acid Stearic Acid Fatty Acid Ester Fibroblast Culture Label Fatty Acid 
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  1. 1.
    Batten, F.E.: Family cerebral degeneration with macular change (so-called juvenile form of family amaurotic idiocy) Quart. Journ. Med. 7: 444, 1914.Google Scholar
  2. 2.
    Zeman, W., and Donahue, S.: Fine structure of the lipid bodies in juvenile amaurotic idiocy. Acta Neuropath. 3: 144, 1963.PubMedCrossRefGoogle Scholar
  3. 3.
    Duffy, P.E., Kornfeld, M. and Suzuki, K.: Neurovisceral storage disease with curvilinear bodies. J. Neuropath. Exp. Neurol. 27: 351, 1968.CrossRefGoogle Scholar
  4. 4.
    Zeman, W.: Second Conference on Batten’s Disease, Proceedings, San Francisco, California, January 23, 1971.Google Scholar
  5. 5.
    Hagberg, B., Sourander, P. and Svennerholm, L.: Late infantile progressive encephalopathy with disturbed polyunsaturated fat metabolism. Acta Paediat. Scand. 57: 495, 1968.Google Scholar
  6. 6.
    Andrews, J.M., Sorenson, V., Cancilla, P.A., Price, H.M. and Menkes, J.H.: Late infantile neurovisceral storage disease with curvilinear bodies. Neurology 21: 207, 1971.PubMedCrossRefGoogle Scholar
  7. 7.
    Menkes, J.H.: Lipid metabolism in brain tissue explants. J. Neurochem. 18: 1433, 1971.PubMedCrossRefGoogle Scholar
  8. 8.
    Menkes, J.H.: Lipid metabolism of brain tissue in culture. Ciba Foundation Symposium: Lipids and Developing Brain London, 1971 (In Press).Google Scholar
  9. 9.
    Fillerup, D.L., Migliore, J.C. and Mead, J.F.: The uptake of lipoproteins by ascites tumor cells. J. Biol. Chem. 233: 98, 1958.PubMedGoogle Scholar
  10. 10.
    Tellez-Nagel, I. and Menkes, J.H. (In Preparation).Google Scholar
  11. 11.
    Blass, J.P. and Menkes, J.H. (In Preparation).Google Scholar
  12. 12.
    Gornall, A.G., Bardawill, C.J. and David, M.M.: Determination of serum proteins by means of the biuret reaction. J. Biol. Chem. 177: 751, 1949.PubMedGoogle Scholar
  13. 13.
    Morrison, W.R. and Smith, L.M.: Preparation of fatty acid methyl esters and dimethylacetals from lipids with boron fluoride-methanol. Journ. Lipid Res. 5: 600, 1964.Google Scholar

Copyright information

© Springer Science+Business Media New York 1972

Authors and Affiliations

  • John H. Menkes
    • 1
  • Don R. Harris
    • 1
  • Natalie Stein
    • 1
  1. 1.Division of Pediatric NeurologyUniversity of California at Los Angeles and Brentwood V.A. HospitalUSA

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