Zobrazeno 1 - 10
of 19
pro vyhledávání: '"F S Furbish"'
Publikováno v:
Proceedings of the National Academy of Sciences of the United States of America. 87(5)
Enzyme replacement has been under consideration as a therapeutic strategy for patients with Gaucher disease for more than two decades. Previous studies indicated that single injections of purified glucocerebrosidase reduced the amount of storage mate
Externí odkaz:
https://explore.openaire.eu/search/publication?articleId=doi_dedup___::e2f1684140a5c7843a376175e6cb252f
https://pubmed.ncbi.nlm.nih.gov/2308952
https://pubmed.ncbi.nlm.nih.gov/2308952
Autor:
F S Furbish, M M Ponpipom, John A. Barranger, T Y Shen, Thomas W. Doebber, Roscoe O. Brady, Margaret Wu, R L Bugianesi
Publikováno v:
Journal of Biological Chemistry. 257:2193-2199
Externí odkaz:
https://explore.openaire.eu/search/publication?articleId=doi_________::974f71fcc04e68893d18d5f69e9590be
https://doi.org/10.1016/s0021-9258(18)34905-6
https://doi.org/10.1016/s0021-9258(18)34905-6
Publikováno v:
Proceedings of the National Academy of Sciences. 74:3560-3563
Enzyme replacement therapy for the alleviation of Gaucher's disease has been impeded because of the difficulty in preparing large amounts of glucocerebrosidase, the enzyme that is deficient in patients with this disorder. A large-scale procedure for
Externí odkaz:
https://explore.openaire.eu/search/publication?articleId=doi_dedup___::a2c51a8520b000c41f0ae1b7c3521356
https://doi.org/10.1073/pnas.74.8.3560
https://doi.org/10.1073/pnas.74.8.3560
Autor:
C Moore, Roscoe O. Brady, Edward I. Ginns, F S Furbish, John A. Barranger, Gary J. Murray, S Pirruccello, Joseph M. Tager, S. H. Sorrell
Publikováno v:
Proceedings of the National Academy of Sciences. 79:5607-5610
Multiple molecular forms of beta-glucocerebrosidase that permit discrimination between neurologic and non-neurologic phenotypes of Gaucher disease have been identified radioimmunologically in fibroblasts and human brain tissue. In normal human fibrob
Externí odkaz:
https://explore.openaire.eu/search/publication?articleId=doi_dedup___::62dc1ab9f673446e9b4e321343e9e7f9
https://doi.org/10.1073/pnas.79.18.5607
https://doi.org/10.1073/pnas.79.18.5607
Autor:
J. A. Barranger, G. E. Mook, Jane M. Quirk, F S Furbish, Michael Pagel, Edward A. Neuwelt, Eugene P. Frenkel, R. O. Brady
Publikováno v:
Proceedings of the National Academy of Sciences. 78:5838-5841
The present studies were undertaken to evaluate the possibility that hexosaminidase A, the enzyme deficient in Tay--Sachs disease, could be effectively delivered to brain. Previous studies from our laboratory have shown that hypertonic mannitol can b
Externí odkaz:
https://explore.openaire.eu/search/publication?articleId=doi_dedup___::5903690a0e5fe124b0d0cf9b2ea7718d
https://doi.org/10.1073/pnas.78.9.5838
https://doi.org/10.1073/pnas.78.9.5838
Publikováno v:
Journal of Biological Chemistry. 259:10112-10117
The N-asparagine-linked oligosaccharide chains of homogeneous human placental beta-glucocerebrosidase were released by hydrazinolysis, and their structures were analyzed. The sequence of sugars, linkage, and anomeric configuration of the glycosidic b
Externí odkaz:
https://explore.openaire.eu/search/publication?articleId=doi_________::cf28a4aad0d327a25308a6ccb513eb5b
https://doi.org/10.1016/s0021-9258(18)90936-1
https://doi.org/10.1016/s0021-9258(18)90936-1
Autor:
Edward L. Schneider, H Tanaka, Andrew E. Gal, Peter G. Pentchev, Roscoe O. Brady, Kinuko Suzuki, F S Furbish
Publikováno v:
Clinica Chimica Acta. 77:53-59
Krabbe's disease is caused by a deficiency of galactocerebrosidase in organs and tissues. Determinations of galactocerebrosidase activity had required the use of galactocerebroside labeled with radiocarbon or radiohydrogen. These materials are expens
Externí odkaz:
https://explore.openaire.eu/search/publication?articleId=doi_dedup___::be533aae8f29d9331555c6866b4598bd
https://doi.org/10.1016/0009-8981(77)90401-6
https://doi.org/10.1016/0009-8981(77)90401-6
Publikováno v:
Inborn Errors of Metabolism in Humans ISBN: 9789400973275
Since the discovery that insufficient activity of lipid catabolizing enzymes formed the basis of heritable lipid-storage disorders, a number of therapeutic strategies have been suggested1. Approaches to the treatment of such disorders included: (1) e
Externí odkaz:
https://explore.openaire.eu/search/publication?articleId=doi_________::e7021dc502a74632b68dce5d6add2051
https://doi.org/10.1007/978-94-009-7325-1_10
https://doi.org/10.1007/978-94-009-7325-1_10
Autor:
P G, Pentchev, J W, Kusiak, J A, Barranger, F S, Furbish, S I, Rapoport, J M, Massey, R O, Brady
Publikováno v:
Advances in experimental medicine and biology. 101
Externí odkaz:
https://explore.openaire.eu/search/publication?articleId=pmid________::1208cfce9f5a0401605b7c88a7a365e8
https://pubmed.ncbi.nlm.nih.gov/208371
https://pubmed.ncbi.nlm.nih.gov/208371
Publikováno v:
Progress in clinical and biological research. 95
Externí odkaz:
https://explore.openaire.eu/search/publication?articleId=pmid________::ed874d122ac84e939aee6abda9a49938
https://pubmed.ncbi.nlm.nih.gov/7122639
https://pubmed.ncbi.nlm.nih.gov/7122639