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.

Abstract

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.

• Authors: E Kida, K E Wisniewski, A A Golabek
• Journal: Neuroscience letters (Neurosci Lett) Vol. 164 Issue 1-2 Pg. 121-4 (Dec 24 1993) ISSN: 0304-3940 [Print] Ireland
• PMID: 8152585 (Publication Type: Journal Article)

Chemical References

• Proton-Translocating ATPases

Topics

• 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)