Results 191 to 200 of about 1,105,094 (212)

Distribution of Saposins (Sphingolipid Activator Proteins) in Tissues of Lysosomal Storage Disease Patients

Journal of Molecular Neuroscience, 1992
Saposins are a group of small glycoproteins derived from a single precursor protein, prosaposin. Each of the four saposins are involved in lysosomal hydrolysis of various sphingolipids. Our recent investigations have shown that saposins accumulate in tissues of several lysosomal storage diseases patients, including those with Tay-Sachs disease and ...
M, Tayama, J S, O'Brien, Y, Kishimoto
openaire   +2 more sources

Synthesis and characterization of a bioactive 82-residue sphingolipid activator protein, saposin C

Journal of Molecular Neuroscience, 1993
The sphingolipid activator protein, saposin C (also termed SAP 2), was chemically synthesized, purified, and characterized. The fully protected 82-residue protein was synthesized by automated solid-phase methods, with multiple recoupling steps resulting in a high average coupling efficiency of 98.8%.
S, Weiler, W, Carson, Y, Lee, D B, Teplow, Y, Kishimoto, J S, O'Brien, J A, Barranger, J M, Tomich   +7 more
openaire   +2 more sources

[Lysosomal enzymes, sphingolipid activator proteins, and protective protein].

Nihon rinsho. Japanese journal of clinical medicine, 1996
The lysosome is an intracytoplasmic acidic vacuole containing more than 60 hydrolytic enzymes for digestion of macromolecules, such as nucleic acids, proteins, lipids and complex carbohydrates. Expression of lysosomal enzyme activities is regulated by various intracellular environmental factors.
openaire   +1 more source

Structure of the lysosomal sphingolipid activator protein 1 by homology with influenza virus neuraminidase

Biochemical and Biophysical Research Communications, 1988
The sphingolipid activator protein 1 (SAP-1) increases the rate of hydrolysis of sphingolipids in the lysosome by apparently bringing together the substrate and the corresponding hydrolytic enzyme. This implies specific recognition of both the substrate and enzyme by SAP-1.
openaire   +2 more sources

Accumulation of sphingolipid activator proteins (SAPs) A and D in granular osmiophilic deposits in miniature Schnauzer dogs with ceroid-lipofuscinosis

Journal of Inherited Metabolic Disease, 1997
D. Palmer, J. Tyynelä, H. C. van Mil, V. J. Westlake, R. Jolly   +4 more
semanticscholar   +1 more source

Sphingolipid activator proteins in the neuronal ceroid-lipofuscinoses: an immunological study

Acta Neuropathologica, 2004
J. Tyynelä, M. Baumann, M. Henseler, K. Sandhoff, M. Haltia   +4 more
semanticscholar   +1 more source

Sphingolipid Activator Protein Deficiency

2009
Alexander K. C. Leung, Cham Pion Kao, Andrew L. Wong, Alexander K. C. Leung, Thomas Kolter, Ute Schepers, Konrad Sandhoff, Ute Schepers, Thomas Kolter, Konrad Sandhoff, Ute Schepers, Thomas Kolter, Konrad Sandhoff, Ute Schepers, Thomas Kolter, Konrad Sandhoff, Bennett Myers, David N. Herrmann, Vikram K. Mahajan, Nand Lal Sharma, Surender Kashyap, C. Ronald Scott, Natalie Gall, Katrin Welt, Karin Scharffetter-Kochanek, Matthew Harries, Tobias Fischer, Ralf Paus, Cornelius F. Boerkoel, Leah I. Elizondo, Thomas Lücke, Markus M. Nöthen, Marcella Rietschel, Undine E. Lang, Sophie Nicole, Bertrand Fontaine, Alexander K. C. Leung, William Lane M. Robson, Thomas Krieg, Julia Weihrauch, Sergio A. Jimenez, Hugo Ten Cate, Itshak Zusman, Pavel Gurevich, Herzl Ben-Hur, Jutta Keller, Jiri Litzman, Yaofeng Zhao, Lennart Hammarstöm, Margherita Bergomi, Carlotta Malagoli, Marco Vinceti, Margherita Bergomi, Carlotta Malagoli, Marco Vinceti, Marie-Estelle Dupont, Olivier Guilbaud, Hermann-Josef Gröne, Zoran V. Popovic, Mario Mancini, Batya B. Davidovici, Batsheva Marcos, Edith Orion, Ronni Wolf, Jean-Louis Vincent, Giora Z. Feuerstein, James C. Keith, Robert R. Ruffolo, Cord Sunderkötter, M. Murat Naki, Cevahir Tekcan, M. Murat Naki, Cevahir Tekcan, Amy S. Rawls, Daniel C. Link, Carolina Reitzenstein, Jürgen Kopitz, Michael Cantz, Michael Cantz, Johannes Uhl, Jürgen Kopitz, John-John B. Schnog, Victor E. A. Gerdes, Bärbel Schütte, Samuel J. Arbes, Darryl C. Zeldin, Alessandra Baumer, Hans-Peter Zenner, Brandy J. Harvey, Jyh-Yeuan Lee, Ina L. Urbatsch, Jan Rémi, Soheyl Noachtar, Alexander K. C. Leung, Sarah H. Elsea, Santhosh Girirajan, Chee-Keong Toh, Cord Sunderkötter, Rebecca Schüle, Ludger Schöls, Niels Borregaard, Jack B. Cowland, John-John B. Schnog, Victor E. A. Gerdes, Alexander K. C. Leung, Laura E. Mitchell, Analee J. Etheredge, Denise S. Hill, Richard H. Finnell, Meinhard Schiller, Dorothee Nashan, Cord Sunderkötter, Stefan Reuter, David Geneviève, Valérie Cormier-Daire, Jozef Gécz, Ravi Savarirayan, Yves Le Loir, Michel Gautier, Cord Sunderkötter, Jean-Michel Rozet, Josseline Kaplan, Katarzyna Lukasiuk, Asla Pitkänen, Stephan Haas, Sören Siegmund, Manfred V. Singer, Henryk Dancygier, Mark Oette, Vicente Andrés, Rainer Wessely, Alexander K. C. Leung, Deborah P. Merke, Alexander K. C. Leung, Kam-Lun Ellis Hon, Jutta Engel, Hans-Michael Meinck, Amei Ludwig, Hartmut Hengel, John-John B. Schnog, Victor E. A. Gerdes, Giora Z. Feuerstein, Xinkang Wang, Ivor L. Gerber, Eric Sibley, Akihiro Takatsu, Brigitta Bondy, Daniel Markovich, Ting-Ting Huang, Charles J. Epstein, Hilmar Prange   +150 more
openaire   +1 more source

Isolation, characterization, and proteolysis of human prosaposin, the precursor of saposins (sphingolipid activator proteins).

Archives of Biochemistry and Biophysics, 1993
Masao Hiraiwa, John S. O'Brien, Yasuo Kishimoto, M. Galdzicka, Arvan L. Fluharty, E. Ginns, Brian M. Martin   +6 more
semanticscholar   +1 more source

Sphingolipid Activator Proteins

, 2002
Michael Nettersheim, K. Sandhoff
semanticscholar   +1 more source

Sphingolipid hydrolases and activator proteins.

Methods in enzymology, 1999
U, Bierfreund, T, Kolter, K, Sandhoff
openaire   +1 more source