Crystal structures of complexes of N-butyl- and N-nonyl-deoxynojirimycin bound to acid beta-glucosidase: insights into the mechanism of chemical chaperone action in Gaucher disease
about
Molecular basis of reduced glucosylceramidase activity in the most common Gaucher disease mutant, N370SLess Is More: Substrate Reduction Therapy for Lysosomal Storage DisordersAcid beta-glucosidase: insights from structural analysis and relevance to Gaucher disease therapyCrystal Structure of the Salmonella enterica Serovar Typhimurium Virulence Factor SrfJ, a Glycoside Hydrolase Family EnzymeCyclodextrin-mediated crystallization of acid β-glucosidase in complex with amphiphilic bicyclic nojirimycin analoguesPharmacological chaperones for human -N-acetylgalactosaminidaseBinding of 3,4,5,6-Tetrahydroxyazepanes to the Acid-β-glucosidase Active Site: Implications for Pharmacological Chaperone Design for Gaucher DiseaseStructural snapshots illustrate the catalytic cycle of β-galactocerebrosidase, the defective enzyme in Krabbe disease.New therapeutic approaches for Krabbe disease: The potential of pharmacological chaperonesMolecular pharming's foot in the FDA's door: Protalix's trailblazing storyIdentification of pharmacological chaperones for Gaucher disease and characterization of their effects on beta-glucocerebrosidase by hydrogen/deuterium exchange mass spectrometry.Comparative therapeutic effects of velaglucerase alfa and imiglucerase in a Gaucher disease mouse model.The development and use of small molecule inhibitors of glycosphingolipid metabolism for lysosomal storage diseases.ELIGLUSTAT TARTRATE: Glucosylceramide Synthase Inhibitor Treatment of Type 1 Gaucher Disease.X-ray and biochemical analysis of N370S mutant human acid β-glucosidase.Pharmacological chaperone therapy for lysosomal storage diseases.Azasugar inhibitors as pharmacological chaperones for Krabbe diseaseIn vitro studies of novel PRKAR1A mutants that extend the predicted RIα protein sequence into the 3'-untranslated open reading frame: proteasomal degradation leads to RIα haploinsufficiency and Carney complexCovalent inhibitors of glycosidases and their applications in biochemistry and biology.Glycosylation and functionality of recombinant β-glucocerebrosidase from various production systems.Glycosphingolipids--nature, function, and pharmacological modulation.A Guided Tour of the Structural Biology of Gaucher Disease: Acid-β-Glucosidase and Saposin C.Glycomimetic-based pharmacological chaperones for lysosomal storage disorders: lessons from Gaucher, GM1-gangliosidosis and Fabry diseases.Design, synthesis, and biological evaluation of N-alkylated deoxynojirimycin (DNJ) derivatives for the treatment of dengue virus infection.Stabilization of Glucocerebrosidase by Active Site Occupancy.Participation of asparagine 370 and glutamine 235 in the catalysis by acid beta-glucosidase: the enzyme deficient in Gaucher disease.β-Glucuronidase from Lactobacillus brevis useful for baicalin hydrolysis belongs to glycoside hydrolase family 30.New insights into the pharmacological chaperone activity of c2-substituted glucoimidazoles for the treatment of Gaucher disease.Ethylenedioxy-PIP2 oxalate reduces ganglioside storage in juvenile Sandhoff disease mice.Accessing 2-substituted piperidine iminosugars by organometallic addition/intramolecular reductive amination: aldehyde vs. nitrone route.Synthetic deoxynojirimycin derivatives bearing a thiolated, fluorinated or unsaturated N-alkyl chain: identification of potent α-glucosidase and trehalase inhibitors as well as F508del-CFTR correctors.Mass spectrometric study of gas-phase ions of acid β-glucosidase (Cerezyme) and iminosugar pharmacological chaperones.Tuning protein folding in lysosomal storage diseases: the chemistry behind pharmacological chaperones.
P2860
Q24621867-B875793F-B102-4846-90AF-656D4F928CC0Q26744230-D6AE177E-6F4C-40F5-A30A-D5A2128BA380Q27652072-717C62E9-C0CE-4985-8918-C4D4D05BCAA2Q27657233-E1A39974-CCCF-4658-A1FC-47DCD8819E21Q27667205-4CDEECF3-F002-4563-AA11-BAFD816155DFQ27674346-A08621B0-3CBF-4960-BA95-A67C9DB32FAEQ27675348-E27E04CA-0407-47D1-BF1E-6BC483B56405Q27680741-F0D26657-9F9C-4D67-926A-2148A746AC1AQ28068142-EF72F256-7918-4412-9D1D-8402290FE2C5Q28086959-E6C8F946-F422-42FB-8CA6-CE99261298DAQ33381275-051671B5-5727-4F6E-8301-B7522017A384Q33588593-5E5FE085-5A2A-4261-8C8F-881C22AC2D63Q33825097-623555C6-998A-4239-9098-1B5B872E9EF9Q34273177-C55C18A7-183F-401B-A5E9-4E2C9FB5F79DQ34452544-7C6659E8-3ECE-4FA7-9A25-E619FD00B544Q35214286-DBD9CA38-216E-4903-A5F7-F5415366A1EBQ35652295-12F37CEB-E559-43EE-BC39-7E531D32063DQ35869641-BB8DF5DB-9744-4A3E-AD46-D0157C1BC5D3Q37171562-AD4DC0AD-EB11-4553-AAB0-6201BFB245FCQ37196619-D239C66E-17EA-4BB0-B5C3-C9F0F2CD4AA9Q37622816-57EB3E1F-DCD2-4BC1-A0D8-857517D9F7C6Q37965168-EEE70987-6D84-4247-9A82-2FC3A4AB5D92Q38797648-6252FF64-E6E7-48BA-BA49-37976989B67CQ39328622-95973D9F-31F0-4D02-AAC6-4419BB6B3232Q41110570-7AF4529C-8219-49ED-9E9C-640F4C3EFAF5Q41296611-62077E76-5A82-4D3D-88FC-1691B431907FQ43926238-E8FB764F-AB08-41C5-B404-CD9736276485Q45931929-6F4A9A6C-8B1D-4B69-95E9-170ECFFFD16AQ48159574-9092ECEF-8057-450A-BED1-2103C424F3DBQ48625712-4C34BF99-1B16-456D-80D6-01032358F03EQ48739210-0F565220-9234-4218-83F9-3122DB4995D6Q52838684-ACDE5CA0-C231-4611-8807-F3E29B3705B7Q54945677-6644B068-4A44-4B6F-AD0E-ACC64419BF24
P2860
Crystal structures of complexes of N-butyl- and N-nonyl-deoxynojirimycin bound to acid beta-glucosidase: insights into the mechanism of chemical chaperone action in Gaucher disease
description
2007 nî lūn-bûn
@nan
2007 թուականի Սեպտեմբերին հրատարակուած գիտական յօդուած
@hyw
2007 թվականի սեպտեմբերին հրատարակված գիտական հոդված
@hy
2007年の論文
@ja
2007年論文
@yue
2007年論文
@zh-hant
2007年論文
@zh-hk
2007年論文
@zh-mo
2007年論文
@zh-tw
2007年论文
@wuu
name
Crystal structures of complexe ...... rone action in Gaucher disease
@ast
Crystal structures of complexe ...... rone action in Gaucher disease
@en
Crystal structures of complexe ...... rone action in Gaucher disease
@nl
type
label
Crystal structures of complexe ...... rone action in Gaucher disease
@ast
Crystal structures of complexe ...... rone action in Gaucher disease
@en
Crystal structures of complexe ...... rone action in Gaucher disease
@nl
prefLabel
Crystal structures of complexe ...... rone action in Gaucher disease
@ast
Crystal structures of complexe ...... rone action in Gaucher disease
@en
Crystal structures of complexe ...... rone action in Gaucher disease
@nl
P2093
P2860
P50
P3181
P356
P1476
Crystal structures of complexe ...... rone action in Gaucher disease
@en
P2093
Boris Brumshtein
David Aviezer
Harry M Greenblatt
Terry D Butters
Yoseph Shaaltiel
P2860
P304
P3181
P356
10.1074/JBC.M705005200
P407
P577
2007-09-28T00:00:00Z