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.
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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)
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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)
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