A Fast Moving Protein in Tay-Sachs Disease

  • Denise Karcher
  • A. Lowenthal
  • W. Zeman
Part of the Advances in Experimental Medicine and Biology book series (AEMB, volume 19)


In 1968 Adriaenssen et al.1 demonstrated a hitherto unknown hydrosoluble protein in the forebrain of two patients with Tay-Sachs disease and suggested that it might represent a disease-specific substance. Later on, Zeman et al. (1970)13 showed that this protein was not present in the brains of all patients with Tay-Sachs disease although the resolution of their techniques was probably not sufficient to detect concentrations of less than 10% of the total of hydrosoluble proteins. Nevertheless, these authors established a direct relationship between the brain weights of patients with Tay-Sachs disease and the relative concentration of the unusual protein, indicating a possible relationship with the edema that, according to Aronson and Volk (1962),2 accounts for the megalobarencephaly of Tay-Sachs disease.


Spinal Cord Human Serum Albumin Brain Extract Precipitation Line Normal Spinal Cord 
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Copyright information

© Springer Science+Business Media New York 1972

Authors and Affiliations

  • Denise Karcher
    • 1
  • A. Lowenthal
    • 2
  • W. Zeman
    • 2
  1. 1.Fondation Born-BungeBerchem-AntwerpenBelgium
  2. 2.Indiana University School of MedicineIndianapolisUSA

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