ATP SYNTHASE SUBUNIT-C STORAGE IN THE POLYMORPHONUCLEOCYTES OF LATE INFANTILE AND JUVENILE BATTEN PATIENTS

In late infantile and juvenile forms of neuronal ceroid lipofuscinosis , commonly known as Batten disease (BD), ATP synthase subunit c accumu lates in the lysosomes of neural cells. By using polyclonal antibodies , raised against bovine liver subunit c and an image analysis system f or the quantification of antibody-linked alkaline phosphatase reaction , we have demonstrated that polymorphonucleocytes (PMN) from a late in fantile and a juvenile BD patient stored several-fold more subunit c a s compared to normal PMN.