ATP synthase subunit c storage in the polymorphonucleocytes of late infantile and juvenile batten patients |
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| Authors: | Naseem H. Ansari Jay D. Cook Peeyush Khanna Satish K. Srivastava |
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| Affiliation: | †Departments of Human Biological Chemistry and Genetics, University of Texas Medical Branch, Galveston, TX 7755-0355, U.S.A.;‡Department of Ophthalmology, University of Texas Medical Branch, Galveston, TX 77555-0355, U.S.A.;cDepartment of Pediatric Neurology, University of Texas Medical Branch, Galveston, TX 77555-0355, U.S.A. |
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| 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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| Keywords: | Batten disease ATP synthase subunit c polymorphonucleocytes |
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