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.
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More From: International Journal of Developmental Neuroscience
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