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PMID:7613514
| Citation |
Tanner, A and Dice, JF (1995) Batten disease fibroblasts in culture accumulate mitochondrial ATP synthase subunit 9. Cell Biol. Int. 19:71-5 |
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| Abstract |
Batten Disease is a lysosomal storage disease in which the major component that accumulates is subunit 9 of mitochondrial ATP synthase. Whether or not fibroblasts in culture exhibit this phenotype is controversial. We show that fibroblasts from a human Batten Disease patient and from a mouse model of this disease exhibit autofluorescent inclusion bodies. We also demonstrate that levels of ATP synthase subunit 9 are elevated in these diseased fibroblasts when compared to control cells. However, the exact growth state of the human fibroblasts was critical, and this factor probably accounts for discrepencies in the literature. |
| Links |
PubMed Online version:10.1006/cbir.1995.1010 |
| Keywords |
Animals; Cells, Cultured/metabolism; Disease Models, Animal; Fibroblasts/metabolism; Fluorescence; Humans; Immunoassay; Mice; Mice, Inbred C57BL; Mitochondria/enzymology; Neuronal Ceroid-Lipofuscinoses/metabolism; Neuronal Ceroid-Lipofuscinoses/pathology; Peptide Fragments/metabolism; Proton-Translocating ATPases/metabolism; Proton-Translocating ATPases/ultrastructure |
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Significance
Annotations
| Gene product | Qualifier | GO ID | GO term name | Evidence Code | with/from | Aspect | Notes | Status |
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See also
References
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