Zobrazeno 1 - 10
of 940
pro vyhledávání: '"K, von Figura"'
Publikováno v:
Journal of Molecular Biology. 305:269-277
Arylsulfatase A (ASA) belongs to the sulfatase family whose members carry a C-alpha-formylglycine that is post-translationally generated by oxidation of a conserved cysteine or serine residue. The crystal structures of two arylsulfatases, ASA and ASB
Externí odkaz:
https://explore.openaire.eu/search/publication?articleId=doi_dedup___::9d519d177ade55bfeeeceea9626e308d
https://doi.org/10.1006/jmbi.2000.4297
https://doi.org/10.1006/jmbi.2000.4297
Publikováno v:
Proceedings of the National Academy of Sciences. 95:13200-13205
Deficiency of dolichyl- P -Glc:Man 9 GlcNAc 2 - PP -dolichyl glucosyltransferase is the cause of an additional type of carbohydrate-deficient glycoprotein syndrome (CDGS type V). Clinically this type resembles the classical type Ia of CDGS caused by
Externí odkaz:
https://explore.openaire.eu/search/publication?articleId=doi_________::7250e4c384afae4617cadc8fe80731e3
https://doi.org/10.1073/pnas.95.22.13200
https://doi.org/10.1073/pnas.95.22.13200
Publikováno v:
The EMBO Journal. 17:1304-1314
Among the various coats involved in vesicular transport, the clathrin associated coats that contain the adaptor complexes AP-1 and AP-2 are the most extensively characterized. The function of the recently described adaptor complex AP-3, which is simi
Externí odkaz:
https://explore.openaire.eu/search/publication?articleId=doi_dedup___::d978e2e89c52f635bdcb3dd01aac08f6
https://doi.org/10.1093/emboj/17.5.1304
https://doi.org/10.1093/emboj/17.5.1304
Autor:
K. Theis, Volkmar Gieselmann, K von Figura, G Lukatela, Norbert Krauss, Thorsten Selmer, W. Saenger
Publikováno v:
Biochemistry. 37:3654-3664
Human lysosomal arylsulfatase A (ASA) is a prototype member of the sulfatase family. These enzymes require the posttranslational oxidation of the -CH2SH group of a conserved cysteine to an aldehyde, yielding a formylglycine. Without this modification
Externí odkaz:
https://explore.openaire.eu/search/publication?articleId=doi_dedup___::f997adabc2e70b9c80cd35675cf3f68d
https://doi.org/10.1021/bi9714924
https://doi.org/10.1021/bi9714924
Publikováno v:
Glycoconjugate Journal. 15:499-505
In the carbohydrate deficient glycoprotein syndrome (CDGS) type 1 glycoproteins with less and shorter N-linked oligosaccharides are synthesized due to a deficiency of phosphomannomutase. Glucose deprivation or mannose addition are shown to partially
Externí odkaz:
https://explore.openaire.eu/search/publication?articleId=doi_________::a8dc63f2c7798dd992e987c7d949f20f
https://doi.org/10.1023/a:1006939104442
https://doi.org/10.1023/a:1006939104442
Publikováno v:
Biochemical Journal. 327:811-818
The cytosolic domain of the 46 kDa mannose-6-phosphate receptor (MPR 46) contains a signal that mediates sorting of the receptor and of a reporter protein to the basolateral surface domain of Madin-Darby canine kidney cells. Progressive truncation of
Externí odkaz:
https://explore.openaire.eu/search/publication?articleId=doi_dedup___::e6db85ee855cbf67f7c2e9d186ad5350
https://doi.org/10.1042/bj3270811
https://doi.org/10.1042/bj3270811
Publikováno v:
Journal of Biological Chemistry. 272:19884-19890
The two known mannose 6-phosphate receptors (MPR46 and MPR300) both mediate the transport of Man-6-P-containing lysosomal proteins to lysosomes. However, the MPRs cannot be detected in lysosomes, instead they recycle between the plasma membrane and e
Externí odkaz:
https://explore.openaire.eu/search/publication?articleId=doi_dedup___::1092ff1848122db91548997f0911c33f
https://doi.org/10.1074/jbc.272.32.19884
https://doi.org/10.1074/jbc.272.32.19884
Publikováno v:
Journal of Cell Science. 110:1023-1032
Recycling of mannose 6-phosphate receptors was investigated by microinjection of F(ab) fragments against their carboxy-terminal peptides (residues 54–67 or 150–164 of the cytoplasmic domain of 46 kDa and 300 kDa mannose 6-phosphate receptor, resp
Externí odkaz:
https://explore.openaire.eu/search/publication?articleId=doi_dedup___::fa186b3d32ffa652874de4a3838cf237
https://doi.org/10.1242/jcs.110.8.1023
https://doi.org/10.1242/jcs.110.8.1023
Autor:
Peter J. Schmidt, Paul Saftig, Christoph Peters, A. Hafner, Wolfgang W. Schmahl, K von Figura, D B McLoghlin, M Evers, Barbara Hess
Publikováno v:
Proceedings of the National Academy of Sciences. 93:8214-8219
Mucopolysaccharidosis VI (MPS VI) is a lysosomal storage disease with autosomal recessive inheritance caused by a deficiency of the enzyme arylsulfatase B (ASB), which is involved in degradation of dermatan sulfate and chondroitin 4-sulfate. A MPS VI
Externí odkaz:
https://explore.openaire.eu/search/publication?articleId=doi_dedup___::4a9ed041d76949ae4b0467d3e8208ec0
https://doi.org/10.1073/pnas.93.16.8214
https://doi.org/10.1073/pnas.93.16.8214
Autor:
R. Gitzelmann, Nils U. Bosshard, H J Sommerlade, Max A. Spycher, K von Figura, J. Briner, M Hubler, S. Arnold
Publikováno v:
Veterinary Pathology. 33:1-13
A 7-month-old female cat was seen for abnormal facial features and abnormality of gait. Facial dysmorphism, large paws in relation to body size, dysostosis multiplex, and poor growth were noted, and mucopolysaccharidosis was suspected. A negative uri
Externí odkaz:
https://explore.openaire.eu/search/publication?articleId=doi_dedup___::018e8a969515fa6299e575cd61bdda87
https://doi.org/10.1177/030098589603300101
https://doi.org/10.1177/030098589603300101