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 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.
D017668:Age of Onset; D000595:Amino Acid Sequence; D000818:Animals; D015153:Blotting, Western; D002417:Cattle; D002648:Child; D002675:Child, Preschool; D006801:Humans; D007091:Image Processing, Computer-Assisted; D015151:Immunoblotting; D007223:Infant; D008969:Molecular Sequence Data; D009472:Neuronal Ceroid-Lipofuscinoses / Q000201:enzymology*; D009504:Neutrophils / Q000201:enzymology*; D006180:Proton-Translocating ATPases / Q000737:chemistry / Q000378:metabolism*
D006180:Proton-Translocating ATPases