Increased expression of subunit c of mitochondrial ATP synthase in brain tissue from neuronal ceroid lipofuscinoses and mucopolysaccharidosis cases but not in long-term fibroblast cultures.

Recent data showed storage of subunit c of mitochondrial ATP synthase in late infantile, juvenile, and adult forms of neuronal ceroid lipofuscinosis (NCL). The present study demonstrates that the expression of subunit c in NCL fibroblasts in long-term cultures, both grown in standard conditions and after leupeptin and ammonium chloride treatment, is not greater than in controls. It indicates that as a result of yet undefined factors, NCL fibroblasts in long-term cultures, lose their ability to accumulate subunit c. Moreover, both Western blot analysis of brain tissue homogenates and immunohistochemistry showed increased immunoreactivity to subunit c in mucopolysaccharidosis type I and III. This increased subunit c expression in a disorder with impaired lysosomal function other than the NCL supports the hypothesis that accumulation of this proteolipid might be related to its defective degradation.
AuthorsE Kida, K E Wisniewski, A A Golabek
JournalNeuroscience letters (Neurosci Lett) Vol. 164 Issue 1-2 Pg. 121-4 (Dec 24 1993) ISSN: 0304-3940 [Print] IRELAND
PMID8152585 (Publication Type: Journal Article)
Chemical References
  • Proton-Translocating ATPases
  • Blotting, Western
  • Brain (cytology, enzymology)
  • Cells, Cultured
  • Electrophoresis, Polyacrylamide Gel
  • Fibroblasts (enzymology)
  • Humans
  • Immunohistochemistry
  • Mitochondria (enzymology)
  • Mucopolysaccharidoses (enzymology)
  • Neuronal Ceroid-Lipofuscinoses (enzymology)
  • Proton-Translocating ATPases (biosynthesis)

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