Abstract
In late infantile and juvenile forms of neuronal ceroid lipofuscinosis, commonly known as Batten disease (BD), ATP synthase subunit c accumulates in the lysosomes of neural cells. By using polyclonal antibodies, raised against bovine liver subunit c and an image analysis system for the quantification of antibody-linked alkaline phosphatase reaction, we have demonstrated that polymorphonucleocytes (PMN) from a late infantile and a juvenile BD patient stored several-fold more subunit c as compared to normal PMN.
Original language | English (US) |
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Pages (from-to) | 455-462 |
Number of pages | 8 |
Journal | International Journal of Developmental Neuroscience |
Volume | 13 |
Issue number | 5 |
DOIs | |
State | Published - Aug 1995 |
Externally published | Yes |
Keywords
- ATP synthase subunit c
- Batten disease
- polymorphonucleocytes
ASJC Scopus subject areas
- Developmental Neuroscience
- Developmental Biology