ER and oxidative stresses are common mediators of apoptosis in both neurodegenerative and non-neurodegenerative lysosomal storage disorders and are alleviated by chemical chaperone... von Wei, Hui, Kim, Sung-Jo, Zhang, Zhongjian, Tsai, Pei-Chih, Wisniewski, Krystyna E., Mukherjee, Anil B.

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Current State of Clinical and Morphological Features in Human NCL von Goebel, Hans H, Wisniewski, Krystyna E.

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Disruption of PPT1 or PPT2 Causes Neuronal Ceroid Lipofuscinosis in Knockout Mice von Gupta, Praveena, Soyombo, Abigail A., Atashband, Armita, Wisniewski, Krystyna E., Shelton, John M., Richardson, James A., Hammer, Robert E., Hofmann, Sandra L.

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N-Glycosylation Is Crucial for Folding, Trafficking, and Stability of Human Tripeptidyl-peptidase I von Wujek, Peter, Kida, Elizabeth, Walus, Marius, Wisniewski, Krystyna E., Golabek, Adam A.

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Carbonic Anhydrase II in the Developing and Adult Human Brain von Kida, Elizabeth, Palminiello, Sonia, Golabek, Adam A, Walus, Mariusz, Wierzba-Bobrowicz, Teresa, Rabe, Ausma, Albertini, Giorgio, Wisniewski, Krystyna E

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Pheno/genotypic correlations of neuronal ceroid lipofuscinoses von WISNIEWSKI, Krystyna E, ZHONG, Nanbert, PHILIPPART, Michel

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Prosegment of Tripeptidyl Peptidase I Is a Potent, Slow-binding Inhibitor of Its Cognate Enzyme von Golabek, Adam A., Dolzhanskaya, Natalia, Walus, Marius, Wisniewski, Krystyna E., Kida, Elizabeth

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Increased levels of carbonic anhydrase II in the developing Down syndrome brain von Palminiello, Sonia, Kida, Elizabeth, Kaur, Kulbir, Walus, Marius, Wisniewski, Krystyna E, Wierzba-Bobrowicz, Teresa, Rabe, Ausma, Albertini, Giorgio, Golabek, Adam A

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Biosynthesis, glycosylation, and enzymatic processing in vivo of human tripeptidyl-peptidase I von Golabek, Adam A, Kida, Elizabeth, Walus, Marius, Wujek, Peter, Mehta, Pankaj, Wisniewski, Krystyna E

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Ser475, Glu272, Asp276, Asp327, and Asp360 are involved in catalytic activity of human tripeptidyl-peptidase I von Walus, Mariusz, Kida, Elizabeth, Wisniewski, Krystyna E., Golabek, Adam A.

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Maturation of Human Tripeptidyl-peptidase I in Vitro von Golabek, Adam A, Wujek, Peter, Walus, Marius, Bieler, Sylvain, Soto, Claudio, Wisniewski, Krystyna E, Kida, Elizabeth

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Glycosaminoglycans Modulate Activation, Activity, and Stability of Tripeptidyl-peptidase I in Vitro and in Vivo von Golabek, Adam A, Walus, Marius, Wisniewski, Krystyna E, Kida, Elizabeth

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Lysosomal ceroid depletion by drugs: Therapeutic implications for a hereditary neurodegenerative disease of childhood von Mukherjee, Anil B, Zhang, Zhongjian, Butler, Jean DeB, Levin, Sondra W, Wisniewski, Krystyna E, Brooks, Susan S

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Disruption of PPT2 in Mice Causes an Unusual Lysosomal Storage Disorder with Neurovisceral Features von Gupta, Praveena, Soyombo, Abigail A., Shelton, John M., Wilkofsky, Ian G., Wisniewski, Krystyna E., Richardson, James A., Hofmann, Sandra L.

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Distribution of Tripeptidyl Peptidase I in Human Tissues Under Normal and Pathological Conditions von KIDA, ELIZABETH, GOLABEK, ADAM A, WALUS, MARIUSZ, WUJEK, PETER, KACZMARSKI, WOJCIECH, WISNIEWSKI, KRYSTYNA E

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Confronting the issues of therapeutic misconception, enrollment decisions, and personal motives in genetic medicine-based clinical research studies for fatal disorders von ARKIN, Lisa M, SONDHI, Dolan, HEIER, Linda A, BALLON, Douglas J, SHUNGU, Dikoma C, WISNIEWSKI, Krystyna E, GREENWALD, Bruce M, HOLLMANN, Charleen, CRYSTAL, Ronald G, WORGALL, Stefan, SUH, Lily Hyon K, HACKETT, Neil R, KAMINSKY, Stephen M, HOSAIN, Syed A, SOUWEIDANE, Mark M, KAPLITT, Michael G, DYKE, Jonathan P

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Identification of three novel mutations of the palmitoyl-protein thioesterase-1 (PPT1) gene in children with neuronal ceroid-lipofuscinosis von Waliany, Sophie, Das, Amit K., Gaben, Ahmad, Wisniewski, Krystyna E., Hofmann, Sandra L.

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Intraneuronal Aβ42 accumulation in Down syndrome brain von Mori, Chica, Spooner, Edward T., Wisniewski, Krystyna E., Wisniewski, Thomas M., Yamaguchi, Haruyasu, Saido, Takaomi C., Tolan, Dean R., Selkoe, Dennis J., Lemere, Cynthia A.

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Temporal Accrual of Complement Proteins in Amyloid Plaques in Down's Syndrome with Alzheimer's Disease von Stoltzner, Sarah E., Grenfell, Trelawney J., Mori, Chica, Wisniewski, Krystyna E., Wisniewski, Thomas M., Selkoe, Dennis J., Lemere, Cynthia A.

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Biosynthesis, Glycosylation, and Enzymatic Processingin Vivo of Human Tripeptidyl-peptidase I von Golabek, Adam A., Kida, Elizabeth, Walus, Marius, Wujek, Peter, Mehta, Pankaj, Wisniewski, Krystyna E.

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