Batten disease fibroblasts in culture accumulate mitochondrial ATP synthase subunit 9.
作者:
Allison Tanner,
J. Fred Dice,
期刊:
Cell Biology International
(WILEY Available online 1995)
卷期:
Volume 19,
issue 1
页码: 71-76
ISSN:1065-6995
年代: 1995
DOI:10.1006/cbir.1995.1010
出版商: Blackwell Publishing Ltd
数据来源: WILEY
摘要:
AbstractBatten 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.
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