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of 581
pro vyhledávání: '"J. Alroy"'
Publikováno v:
Journal of Lipid Research, Vol 46, Iss 4, Pp 744-751 (2005)
II3NeuAc-GgOse4Cer (GM1) gangliosidosis is an incurable lysosomal storage disease caused by a deficiency in acid β-galactosidase (β-gal), resulting in the accumulation of ganglioside GM1 and its asialo derivative GgOse4Cer (GA1) in the central nerv
Externí odkaz:
https://doaj.org/article/ca0629307d7343c7a4295dffd03ffc5c
Akademický článek
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Akademický článek
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Publikováno v:
American Journal of Transplantation. 11:1169-1175
Transplantation of donation after cardiac death (DCD) livers has higher rates of organ failure and complications, specifically ischemic biliary injuries. Reported large animal DCD models all employ active means to halt circulation, contrary to human
Publikováno v:
Journal of Lipid Research, Vol 46, Iss 4, Pp 744-751 (2005)
II3NeuAc-GgOse4Cer (GM1) gangliosidosis is an incurable lysosomal storage disease caused by a deficiency in acid beta-galactosidase (beta-gal), resulting in the accumulation of ganglioside GM1 and its asialo derivative GgOse4Cer (GA1) in the central
Publikováno v:
Biochemistry. Biokhimiia. 79(7)
More than 50 hereditary lysosomal storage disorders (LSDs) are currently described. Most of these disorders are due to a deficiency of certain hydrolases/glycosidases and subsequent accumulation of nonhydrolyzable carbohydrate-containing compounds in
Publikováno v:
Pathobiology of Human Disease ISBN: 9780123864574
Lysosomal storage diseases are a group of inherited disorders characterized by deficient activity of lysosomal enzymes. They are classified, according to the chemical nature of the accumulated substrate, into glycolipid and glycoprotein storage disea
Externí odkaz:
https://explore.openaire.eu/search/publication?articleId=doi_________::9eb69941ba3196aaaf6b25c1405f06b7
https://doi.org/10.1016/b978-0-12-386456-7.03112-9
https://doi.org/10.1016/b978-0-12-386456-7.03112-9
Autor:
S. Abbasi, N.L. Adolphi, E. Aikawa, H. Akbar, S. Akilesh, M.I. Aladjem, M. Allocca, G. Alpini, J. Alroy, B.J. Altman, P. Andujar, Z.A. Antonello, M. Antsiferova, B.S. Apica, I. Ariel, B.J. Aronow, J.W. Ashley, I.R. Badell, A. Bagg, M. Bajaj, S. Banerjee, J.S. Barbieri, E.E. Bardes, L. Barisoni, J.A. Barletta, D.G. Baskin, R.A. Bastarrachea, A. Bayat, P. Bayrak-Toydemir, A.H. Beck, D.C. Beebe, H. Beltran, G. Benichou, M. Bergman, S.A. Bernard, P. Bernardi, D.H. Best, H.C. Blair, P. Bonaldo, J. Bondy, F.T. Bosman, B.E. Bouma, M.L. Brandi, S.C. Bresler, M.T. Brewer, C.J. Britto, J.E. Brock, L.A.A. Brosens, H. Budge, E.M. Burd, M.L. Burness, T. Bushnell, J. Byrd, A. Calderone, M.J. Campbell, D. Cao, W. Capell, R. Cardigan, P.M. Carey, F. Carneiro, S.A. Carp, A.M. Carter, M.J. Cascio, R.J. Castellani, J. Castellanos, J.M. Caviglia, F. Cecconi, S. Chamarthy, E. Chamma, A. Chang, A.Y. Chang, N.C. Chang, D.G. Chapman, A.K. Charles, D. Chen, D.F. Chen, P. Chen, J. Cheng, R.D. Chernock, S. Cheruvu, J. Chiang, G.V. Childs, Y.-B. Cho, A.M.K. Choi, J.K. Choi, N.A. Cipriani, J.O.S.H. Cleary, E. Clementi, G.A. Clines, M.L. Cohen, W.B. Coleman, D.K. Coletta, A.M.B. Collie, L. Cooling, E. Coron, D. Côté, L.M. Coussens, B.J. Crielaard, R.Q. Cron, C.P. Crum, N.M. Cruz, S.H. Dairkee, C.A. Daly, C.V. Dang, M.I. Danila, A. Daradich, C.M. Darnell, D.A. Dartt, A. Das, F. D’Asta, R. DeFronzo, G. De Hertogh, C.S. Dela Cruz, L. de la Cruz-Merino, C. De Palma, A.J. Demetris, S. DeMorrow, P.-D. Denechaud, M.F. Di Carli, E.F. DiCarlo, I. Dikic, A. Dimberg, M.L. Dowell, L.A. Doyle, C.B. Drachenberg, E. Driskell, D.G. Duda, J. Duker, J.R.B. Dyck, C. Ecker, J.M. Elifritz, T.M. Elsheikh, A. Ensari, L.M. Ernst, K.J. Esch, L. Fajas-Coll, Q. Fang, N.A. Farhat, G. Farshid, O.M. Faye-Petersen, M.G. Fehlings, F. Fend, X. Feng, H. Fernandes, J.C. Fernandez-Checa, B.P. Ferreira, I.J. Fidler, J.A. Finn, A. Fischer, M.C. Fishbein, H.B. Fleit, M. Flomenbaum, A. Folkins, H. Francis, K.M. Frank, C.W. Frevert, A.E. Frias, J.R. Friedman, D. Fukumura, M.B. Furie, A.L. Gaffo, F. Galateau-Sallé, E.C. Gallegos-Cabriales, C.R. Gandhi, M. Gannon, M.L. García-Moliner, J.M. Gardner, C.A. Gasper, P. Gaulard, J.P. Gaut, G. Gavia-García, C. Gerrard, A.P. Ghosh, A.B.S Giersch, S.R. Gilbert, J.R. Gill, F. Giusti, J.M. Glorioso, M.C. González-Torres, C.L. Goolsby, M.J. Gora, I.O. Gordon, A.I. Gotlieb, A.M. Gouw, A. Goyal, M. Grégoire, B.B. Graham, D.N. Granger, A.K. Greene, J.J. Greenlee, R. Griffiths, A.R. Guimarães, M. Gulati, A. Gullet, S. Gupta, N.B. Haider, M.K. Halushka, T.M. Hambuch, S.M. Hamza, Y. Han, W.P. Hansen, R. Hard, B.T. Harris, J.E. Harris, M.E. Hartnett, R.P. Hasserjian, G.M. Hatch, M.M. Hefti, D.S. Heller, J.A. Hemminger, J.E. Hendrickson, K.D. Henley, E. Herzog, J.R. Hess, C.E. Hill, J. Hipp, R. Hobbs, D. Höller, R.R. Hodges, R.J. Homer, N. Horowitz, E.D. Hsi, A.L. Hsieh, J.M. Hunt, S. Hure, A.N. Husain, S. Hussey, J.D. Hutcheson, R.M. Hutson, A. Illescas-Vacas, C.G. Irvin, F.A. Jaffer, R. Jäger, R.K. Jain, S. Jain, J. James, M. Jansen, J.A. Jarzembowski, M.-C. Jaurand, D. Jean, A.G. Jegga, K.A. Jellinger, K.-Y. Jen, V.Y. Jo, B. Johnson, R.L. Jones, T.A. Kalfa, M. Kamionek, D. Kang, C. Kantari, P.F. Kantor, G. Kanzaki, R. Karns, P.J. Katzman, T. Kawai, T.W. Kelley, J.W. Kent, E.H. Kerr, R.R. Kew, M. Khalighi, T.H. Khanh Vu, T.Y. Khong, B.S. Kim, J. Kim, M.J. Klein, S.J. Knechtle, B.A. Konkle, J. Kowalewska, L.J. Kricka, B. Krishnan, A. Kumar, S. Kumar, P. Kvietys, R.Y. Kwong, E. Lafont, A.C. Laga, S. Lagarrigue, A. Lakin, Z.G. Laszik, G.Y. Lauwers, N.V. Laver, M.W. Lawlor, J.A. Lederer, R.E. Lee, W.M. Lee, R. LeGallo, E. Leich, B. Lemmens, F. Le Pimpec-Barthes, L. Leval, B.D. Levy, J.S. Lewis, T.L. Lewis, D. Leyva-Illades, L. Li, Y.-P. Li, E.S. Lianidou, L. Liao, H. Liapis, J.B. Lin, A.-L. Lin, M.E. Lindsay, E. Liu, T. Longacre, J.C. Lopez-Alvarenga, I. Lopez-Mejía, G. Lozanski, M.S. Lucia, E. Luk, G.A. Lutty, R.A. Maclellan, A. Madabhushi, A. Mahindra, E. Malek, C. Mammucari, H. Mani, S.A. Mao, C.C. Marboe, M. Marí, F. Marini, A. Markou, A.H. Marshall, S.J. Martin, M. Marzioni, S. Masli, K.E. Matsukuma, U.A. Matulonis, J. Mayfield, J.P. McCoy, C.J. McDougle, M.R. McGinnis, A. McGuire, K.K. McKinstry, B.M. McManus, A.L. Means, G.M. Meny, N. Merchant, E.E.K Meserve, A.M. Mess, M.I. Minervini, R.N. Mitchell, S.E. Monaco, S.P. Monga, H.-Y. Monica Way, C. Montecucco, K.T. Montone, E.A. Morgan, T.K. Morgan, K. Morrissey, R.M. Mortensen, S.A. Moser, J.M. Mosquera, B.T. Mossman, A.C.F. Motta, E. Mullins, G.F. Murphy, L. Murray, I.U. Mysorekar, B. Nadel, A.S. Nadon, N. Nagathihalli, O. Nájera-Medina, M.A. Nalesnik, C.C. Nast, Y. Natkunam, J.C. Nault, E.J. Nava-González, R. Nayar, R.D. Nerenz, H. Neumann, H. Ni, K.B. Nolte, L. Norton, J. Nowak, C. Nucera, S.L. Nyberg, S.A. Oakes, G.J.A. Offerhaus, S. Ojha, H. Okabe, A.M. Oliveira, E.A. Osborn, P. O'Tierney-Ginn, G. Ott, A. Ozcan, R.F. Padera, M.B. Pagano, E.K. Page, A.S. Paintal, J.-C. Pairon, J.C. Papadimitriou, H.-J. Park, J.Y. Park, L.N. Parsons, D. Patra, A. Peclovits, P.M. Peeters, T.N. Perkins, G. Perry, A. Perumbeti, C.A. Petersen, I. Petrache, M.G. Petroff, J.R. Pettus, M.M. Picken, C.R. Pierson, M.E. Pittman, J. Pogoriler, K. Politi, S.M. Pollack, L. Quintanilla-Martínez, M.F. Rai, S. Ramkissoon, P.S. Randhawa, J.R. Rangel, A. Rasola, B. Reeves, A. Reheman, D.G. Remick, N.L. Reynaert, J.M. Richmond, S. Rivella, A.G. Rivenbark, R. Rizzuto, K.A. Roberts, D.A. Robin, L.J. Robinson, D.C. Rockey, A. Rosenwald, O. Rossetto, K.A. Roth, J. Roy-Chowdhury, N. Roy-Chowdhury, M.A. Rubin, M.A. Rudnicki, D.S. Russell, S.W. Ryter, D.R. Saban, R.A. Sacher, D.B. Sacks, X. Sagaert, A. Sagdeo, B. Sahay, A. Sahin, A. Samali, B. Sampson, R. Sánchez-Escribano, M. Sandri, A. Sanyal, E. Sasatomi, V. Sauer, A. Scherpereel, E.P. Schmidt, R.F. Schwabe, L. Scorrano, M.G. Scott, J.C. Scull, M.A. Seidman, A. Seki, T.J. Sellati, K. Serban, C.N. Serhan, S.V. Seshan, A. Seth, J.T. Seykora, N. Sharma, C. Shi, S.-R. Shi, M. Shimada, A. Shimizu, D.B. Singer, K. Sitko, R.F. Smallwood, D.J. Smiraglia, B.R. Smith, H. Smola, M. Soubeyrand, W.L. Stahl, M. Stajić, S.J. Stanworth, N. Stathatos, K.M. Stemler, T.M. Stevens, Z.E. Stine, M.L. Stoll, A. Strati, T.M. Strutt, M. Sund, M.M. Sung, M.E. Symonds, S. Tabar, N. Takahashi, J.E. Talmadge, V. Tang, M. Tangrea, C. Tarango, J.D. Tario, C.R. Taylor, R. Taylor, G.J. Tearney, K. Tefera, S. Thomas, K.L. Thornburg, C.A. Tirado, A.A.R. Tobian, J.E. Tomaszewski, C.A. Tormey, R. Torres, M.-H. Tran, E.E. Tredget, N.S. Treister, J. Trotter, D. Troyer, L. Truong, R.R. Tubbs, S. Turakhia, C.I. Unglert, T. Utheim, A. Vahabzadeh, A. van Bokhoven, T. Vanden Berghe, P. Vandenabeele, I.J. van der Klei, V.K. Vanguri, C.J.F Van Noorden, C. Van Poznak, R.R. Vassallo, R. Vawda, M. Vieth, D.W. Visscher, S.W. Volk, G.N. Vyas, S.N. Waggoner, H. Walczak, D.H. Walker, P.K. Wallace, K.A. Wanat, J. Wang, Y. Wang, Y.X. Wang, W.C. Warger, S. Wei, S.A. Weinman, B.M. Wenig, S.C. Wentz, S. Werner, G. Wertheim, E.M. Whitley, W. Wooderchak-Donahue, K. Woods, E.F.M. Wouters, Y. Wu, W. Xing, P. Yachimski, P. Yan, J. Yang, L. Yang, S. Yoshizawa, J. Yuan, S.-H. Yun, A. Yvon, H. Zhang, P. Zhang, Z. Zhao, G. Zhu, R. Zhu, B.N. Zordoky, J. Zou, J.A. Zuccato, J. Zucman-Rossi
Externí odkaz:
https://explore.openaire.eu/search/publication?articleId=doi_________::9503e3dab053bc345e04354a10e1690d
https://doi.org/10.1016/b978-0-12-386456-7.09990-1
https://doi.org/10.1016/b978-0-12-386456-7.09990-1
Publikováno v:
Journal of the American Animal Hospital Association. 35:436-439
A nine-week-old saluki puppy was presented to Tufts University School of Veterinary Medicine for progressive, generalized weakness and bilateral forelimb deformities. Examination suggested a diffuse neuromuscular lesion. Cerebrospinal fluid (CSF) ana