|1.||Jadot, Michel: 1 article (06/2010)|
|2.||Sleat, David E: 1 article (06/2010)|
|3.||Lobel, Peter: 1 article (06/2010)|
|4.||Xu, Su: 1 article (06/2010)|
|5.||Ryazantsev, Sergey: 1 article (04/2007)|
|6.||Zhao, Hui-Zhi: 1 article (04/2007)|
|7.||Yu, Wei-Hong: 1 article (04/2007)|
|8.||Neufeld, Elizabeth F: 1 article (04/2007)|
|9.||Ohmi, Kazuhiro: 1 article (04/2007)|
|10.||Palmer, D N: 1 article (10/2002)|
|1.||Neuronal Ceroid-Lipofuscinoses (Neuronal Ceroid Lipofuscinosis)
04/01/1997 - "Immunohistochemical and biochemical studies of subunit c of mitochondrial ATP synthase (SCMAS) storage were carried out in neuronal ceroid lipofuscinosis (NCL) and in a series of unrelated inherited and acquired lysosomal disorders. "
02/01/1997 - "Immunohistochemical and ultrastructural studies were undertaken to determine whether accumulation of subunit c of mitochondrial ATP synthase could be detected microscopically in fibroblasts cultured from patients with late infantile and with juvenile Batten disease. "
10/01/2002 - "The staining reactions, autofluorescence and ultrastructure of storage material allowed the diagnosis of neuronal ceroid-lipofuscinosis associated with the accumulation of subunit-c of mitochondrial ATP synthase. "
01/01/2001 - "Potential interaction of CLN3 with subunit c of mitochondrial ATP synthase, the major component of the storage material that accumulates in Batten disease patients, was also tested. "
06/01/2000 - "In addition to characteristic electron microscopic findings, enlarged neurons showed strong immunoreactivity against subunit c of mitochondrial ATP synthase which has been reported previously in only a few adult cases of neuronal ceroid-lipofuscinosis. "
|2.||Neurodegenerative Diseases (Neurodegenerative Disease)
06/15/2010 - "Classical late-infantile neuronal ceroid lipofuscinosis (LINCL) is a fatal neurodegenerative disease of children caused by mutations in TPP1, the gene encoding the lysosomal protease tripeptidyl peptidase 1. LINCL is characterized by lysosomal accumulation of storage material of which only a single protein component, subunit c of mitochondrial ATP synthase, has been well established to date. "
|3.||Mucopolysaccharidosis III (Sanfilippo Syndrome)