6
views
0
recommends
+1 Recommend
0 collections
    0
    shares
      • Record: found
      • Abstract: found
      • Article: not found

      Different patterns of hydrophobic protein storage in different forms of neuronal ceroid lipofuscinosis (NCL, Batten disease).

      Neuropediatrics

      Vacuoles, pathology, Adolescent, Animals, Brain, Child, metabolism, Child, Preschool, Dogs, Female, Glycoproteins, Humans, Infant, Male, Mice, Mice, Neurologic Mutants, Microscopy, Electron, Multienzyme Complexes, physiology, Neuronal Ceroid-Lipofuscinoses, Phosphotransferases (Phosphate Group Acceptor), Saposins, Sheep, Species Specificity, Sphingolipid Activator Proteins, ATP Synthetase Complexes, Adenosine Triphosphatases

      Read this article at

      ScienceOpenPublisherPubMed
      Bookmark
          There is no author summary for this article yet. Authors can add summaries to their articles on ScienceOpen to make them more accessible to a non-specialist audience.

          Abstract

          Since the discovery of mitochondrial ATP synthase subunit c storage in different forms of neuronal ceroid lipofuscinosis (NCL, Batten disease), it has been found that other hydrophobic proteins also accumulate in different forms. Costorage of subunit c of vacuolar ATPase is observed in "mnd/mnd" mice and in English Setters, Border Collies and Tibetan Terriers. A small amount is stored in the ovine disease and none in the human late-infantile disease. It is a storage body matrix component. An additional 8 kDa component immunoreactive to vacuolar ATPase subunit c antibodies is found in brain-derived storage bodies. The sphingolipid activator proteins, SAPs A and D, are stored in the human infantile disease and a form in Miniature Schnauzer dogs, but neither of the c subunits are. These results suggest two classes of NCL, the subunit c-storing diseases, related by a series of lesions in a subunit c-turnover pathway, and the SAP-storing diseases.

          Related collections

          Author and article information

          Journal
          10.1055/s-2007-973666
          9151321

          Comments

          Comment on this article