1-Deoxynojirimycin: An alpha-glucosidase inhibitor with antiviral action. Derivatives of deoxynojirimycin may have anti-HIV activity.alpha-Glucosidases: Enzymes that catalyze the exohydrolysis of 1,4-alpha-glucosidic linkages with release of alpha-glucose. Deficiency of alpha-1,4-glucosidase may cause GLYCOGEN STORAGE DISEASE TYPE II.GlucosamineIndolizinesMorus: A plant genus of the family MORACEAE that is widely planted for shade.Swainsonine: An indolizidine alkaloid from the plant Swainsona canescens that is a potent alpha-mannosidase inhibitor. Swainsonine also exhibits antimetastatic, antiproliferative, and immunomodulatory activity.Glucosidases: Enzymes that hydrolyze O-glucosyl-compounds. (Enzyme Nomenclature, 1992) EC 3.2.1.-.Mannosidases: Glycoside hydrolases that catalyze the hydrolysis of alpha or beta linked MANNOSE.alpha-Mannosidase: An enzyme that catalyzes the HYDROLYSIS of terminal, non-reducing alpha-D-mannose residues in alpha-D-mannosides. The enzyme plays a role in the processing of newly formed N-glycans and in degradation of mature GLYCOPROTEINS. There are multiple isoforms of alpha-mannosidase, each having its own specific cellular location and pH optimum. Defects in the lysosomal form of the enzyme results in a buildup of mannoside intermediate metabolites and the disease ALPHA-MANNOSIDOSIS.Oligosaccharides: Carbohydrates consisting of between two (DISACCHARIDES) and ten MONOSACCHARIDES connected by either an alpha- or beta-glycosidic link. They are found throughout nature in both the free and bound form.Imino Furanoses: Five-carbon furanose sugars in which the OXYGEN is replaced by a NITROGEN atom.Imino Pyranoses: Six-carbon pyranose sugars in which the OXYGEN is replaced by a NITROGEN atom.Glucan 1,4-alpha-Glucosidase: An enzyme that catalyzes the hydrolysis of terminal 1,4-linked alpha-D-glucose residues successively from non-reducing ends of polysaccharide chains with the release of beta-glucose. It is also able to hydrolyze 1,6-alpha-glucosidic bonds when the next bond in sequence is 1,4.Imino Sugars: Sugars in which the OXYGEN is replaced by a NITROGEN atom. This substitution prevents normal METABOLISM resulting in inhibition of GLYCOSIDASES and GLYCOSYLTRANSFERASES.Acarbose: An inhibitor of ALPHA-GLUCOSIDASES that retards the digestion and absorption of DIETARY CARBOHYDRATES in the SMALL INTESTINE.Sodium-Glucose Transport Proteins: Monosaccharide transport proteins that function as active symporters. They utilize SODIUM or HYDROGEN IONS to transport GLUCOSE across CELL MEMBRANES.Mannosyl-Glycoprotein Endo-beta-N-Acetylglucosaminidase: A group of related enzymes responsible for the endohydrolysis of the di-N-acetylchitobiosyl unit in high-mannose-content glycopeptides and GLYCOPROTEINS.Alkaloids: Organic nitrogenous bases. Many alkaloids of medical importance occur in the animal and vegetable kingdoms, and some have been synthesized. (Grant & Hackh's Chemical Dictionary, 5th ed)Hexosaminidases: Enzymes that catalyze the hydrolysis of N-acylhexosamine residues in N-acylhexosamides. Hexosaminidases also act on GLUCOSIDES; GALACTOSIDES; and several OLIGOSACCHARIDES.Mannose: A hexose or fermentable monosaccharide and isomer of glucose from manna, the ash Fraxinus ornus and related plants. (From Grant & Hackh's Chemical Dictionary, 5th ed & Random House Unabridged Dictionary, 2d ed)Glycosylation: The chemical or biochemical addition of carbohydrate or glycosyl groups to other chemicals, especially peptides or proteins. Glycosyl transferases are used in this biochemical reaction.Pituitary Hormones, Anterior: Hormones secreted by the adenohypophysis (PITUITARY GLAND, ANTERIOR). Structurally, they include polypeptide, protein, and glycoprotein molecules.Glucosyltransferases: Enzymes that catalyze the transfer of glucose from a nucleoside diphosphate glucose to an acceptor molecule which is frequently another carbohydrate. EC 2.4.1.-.beta-Glucosidase: An exocellulase with specificity for a variety of beta-D-glycoside substrates. It catalyzes the hydrolysis of terminal non-reducing residues in beta-D-glucosides with release of GLUCOSE.alpha 1-Antitrypsin: Plasma glycoprotein member of the serpin superfamily which inhibits TRYPSIN; NEUTROPHIL ELASTASE; and other PROTEOLYTIC ENZYMES.Enzyme Inhibitors: Compounds or agents that combine with an enzyme in such a manner as to prevent the normal substrate-enzyme combination and the catalytic reaction.Carbohydrate Sequence: The sequence of carbohydrates within POLYSACCHARIDES; GLYCOPROTEINS; and GLYCOLIPIDS.Glycoside HydrolasesKinetics: The rate dynamics in chemical or physical systems.
(1/366) Trypanosoma cruzi calreticulin is a lectin that binds monoglucosylated oligosaccharides but not protein moieties of glycoproteins.

Trypanosoma cruzi is a protozoan parasite that belongs to an early branch in evolution. Although it lacks several features of the pathway of protein N-glycosylation and oligosaccharide processing present in the endoplasmic reticulum of higher eukaryotes, it displays UDP-Glc:glycoprotein glucosyltransferase and glucosidase II activities. It is herewith reported that this protozoan also expresses a calreticulin-like molecule, the third component of the quality control of glycoprotein folding. No calnexin-encoding gene was detected. Recombinant T. cruzi calreticulin specifically recognized free monoglucosylated high-mannose-type oligosaccharides. Addition of anti-calreticulin serum to extracts obtained from cells pulse-chased with [35S]Met plus [35S]Cys immunoprecipitated two proteins that were identified as calreticulin and the lysosomal proteinase cruzipain (a major soluble glycoprotein). The latter but not the former protein disappeared from immunoprecipitates upon chasing cells. Contrary to what happens in mammalian cells, addition of the glucosidase II inhibitor 1-deoxynojirimycin promoted calreticulin-cruzipain interaction. This result is consistent with the known pathway of protein N-glycosylation and oligosaccharide processing occurring in T. cruzi. A treatment of the calreticulin-cruzipain complexes with endo-beta-N-acetylglucosaminidase H either before or after addition of anti-calreticulin serum completely disrupted calreticulin-cruzipain interaction. In addition, mature monoglucosylated but not unglucosylated cruzipain isolated from lysosomes was found to interact with recombinant calreticulin. It was concluded that the quality control of glycoprotein folding appeared early in evolution, and that T. cruzi calreticulin binds monoglucosylated oligosaccharides but not the protein moiety of cruzipain. Furthermore, evidence is presented indicating that glucosyltransferase glucosylated cruzipain at its last folding stages.  (+info)

(2/366) Delayed symptom onset and increased life expectancy in Sandhoff disease mice treated with N-butyldeoxynojirimycin.

Sandhoff disease is a neurodegenerative disorder resulting from the autosomal recessive inheritance of mutations in the HEXB gene, which encodes the beta-subunit of beta-hexosaminidase. GM2 ganglioside fails to be degraded and accumulates within lysosomes in cells of the periphery and the central nervous system (CNS). There are currently no therapies for the glycosphingolipid lysosomal storage diseases that involve CNS pathology, including the GM2 gangliosidoses. One strategy for treating this and related diseases is substrate deprivation. This would utilize an inhibitor of glycosphingolipid biosynthesis to balance synthesis with the impaired rate of catabolism, thus preventing storage. One such inhibitor is N-butyldeoxynojirimycin, which currently is in clinical trials for the potential treatment of type 1 Gaucher disease, a related disease that involves glycosphingolipid storage in peripheral tissues, but not in the CNS. In this study, we have evaluated whether this drug also could be applied to the treatment of diseases with CNS storage and pathology. We therefore have treated a mouse model of Sandhoff disease with the inhibitor N-butyldeoxynojirimycin. The treated mice have delayed symptom onset, reduced storage in the brain and peripheral tissues, and increased life expectancy. Substrate deprivation therefore offers a potentially general therapy for this family of lysosomal storage diseases, including those with CNS disease.  (+info)

(3/366) Cellobiose transport by mixed ruminal bacteria from a Cow.

The transport of cellobiose in mixed ruminal bacteria harvested from a holstein cow fed an Italian ryegrass hay was determined in the presence of nojirimycin-1-sulfate, which almost inhibited cellobiase activity. The kinetic parameters of cellobiose uptake were 14 microM for the Km and 10 nmol/min/mg of protein for the Vmax. Extracellular and cell-associated cellobiases were detected in the rumen, with both showing higher Vmax values and lower affinities than those determined for cellobiose transport. The proportion of cellobiose that was directly transported before it was extracellularly degraded into glucose increased as the cellobiose concentration decreased, reaching more than 20% at the actually observed levels of cellobiose in the rumen, which were less than 0.02 mM. The inhibitor experiment showed that cellobiose was incorporated into the cells mainly by the phosphoenolpyruvate phosphotransferase system and partially by an ATP-dependent and proton-motive-force-independent active transport system. This finding was also supported by determinations of phosphoenolpyruvate phosphotransferase-dependent NADH oxidation with cellobiose and the effects of artificial potentials on cellobiose transport. Cellobiose uptake was sensitive to a decrease in pH (especially below 6.0), and it was weakly but significantly inhibited in the presence of glucose.  (+info)

(4/366) Molecular chaperones stimulate the functional expression of the cocaine-sensitive serotonin transporter.

The serotonin transporter (SERT) is an N-glycosylated integral membrane protein that is predicted to contain 12 transmembrane regions. SERT is the major binding site in the brain for antidepressant drugs, and it also binds amphetamines and cocaine. The ability of various molecular chaperones to interact with a tagged version of SERT (Myc-SERT) was investigated using the baculovirus expression system. Overexpression of Myc-SERT using the baculovirus system led to substantial quantities of inactive transporter, together with small amounts of fully active and, therefore, correctly folded molecules. The high levels of inactive Myc-SERT probably arose because folding was rate-limiting due, perhaps, to insufficient molecular chaperones. Therefore, Myc-SERT was co-expressed with the endoplasmic reticulum (ER) molecular chaperones calnexin, calreticulin and immunoglobulin heavy chain binding protein (BiP), and the foldase, ERp57. The expression of functional Myc-SERT, as determined by an inhibitor binding assay, was enhanced nearly 3-fold by co-expressing calnexin, and to a lesser degree on co-expression of calreticulin and BiP. Co-expression of ERp57 did not increase the functional expression of Myc-SERT. A physical interaction between Myc-SERT-calnexin and Myc-SERT-calreticulin was demonstrated by co-immunoprecipitation. These associations were inhibited in vivo by deoxynojirimycin, an inhibitor of N-glycan precusor trimming that is known to prevent the calnexin/calreticulin-N-glycan interaction. Functional expression of the unglycosylated SERT mutant, SERT-QQ, was also increased on co-expression of calnexin, suggesting that the interaction between calnexin and SERT is not entirely dictated by the N-glycan. SERT is the first member of the neurotransmitter transporter family whose folding has been shown to be assisted by the molecular chaperones calnexin, calreticulin, and BiP.  (+info)

(5/366) Temporal association of the N- and O-linked glycosylation events and their implication in the polarized sorting of intestinal brush border sucrase-isomaltase, aminopeptidase N, and dipeptidyl peptidase IV.

The temporal association between O-glycosylation and processing of N-linked glycans in the Golgi apparatus as well as the implication of these events in the polarized sorting of three brush border proteins has been the subject of the current investigation. O-Glycosylation of pro-sucrase-isomaltase (pro-SI), aminopeptidase N (ApN), and dipeptidyl peptidase IV (DPPIV) is drastically reduced when processing of the mannose-rich N-linked glycans is blocked by deoxymannojirimycin, an inhibitor of the Golgi-located mannosidase I. By contrast, O-glycosylation is not affected in the presence of swainsonine, an inhibitor of Golgi mannosidase II. The results indicate that removal of the outermost mannose residues by mannosidase I from the mannose-rich N-linked glycans is required before O-glycosylation can ensue. On the other hand, subsequent mannose residues in the core chain impose no sterical constraints on the progression of O-glycosylation. Reduction or modification of N- and O-glycosylation do not affect the transport of pro-SI, ApN, or DPPIV to the cell surface per se. However, the polarized sorting of two of these proteins, pro-SI and DPPIV, to the apical membrane is substantially altered when O-glycans are not completely processed, while the sorting of ApN is not affected. The processing of N-linked glycans, on the other hand, has no influence on sorting of all three proteins. The results indicate that O-linked carbohydrates are at least a part of the sorting mechanism of pro-SI and DPPIV. The sorting of ApN implicates neither O-linked nor N-linked glycans and is driven most likely by carbohydrate-independent mechanisms.  (+info)

(6/366) VIP36 localisation to the early secretory pathway.

VIP36, an integral membrane protein previously isolated from epithelial MDCK cells, is an intracellular lectin of the secretory pathway. Overexpressed VIP36 had been localised to the Golgi complex, plasma membrane and endocytic structures suggesting post-Golgi trafficking of this molecule (Fiedler et al., 1994). Here we provide evidence that endogenous VIP36 is localised to the Golgi apparatus and the early secretory pathway of MDCK and Vero cells and propose that retention is easily saturated. High resolution confocal microscopy shows partial overlap of VIP36 with Golgi marker proteins. Punctate cytoplasmic structures colocalise with coatomer and ERGIC-53, labeling ER-Golgi intermediate membrane structures. Cycling of VIP36 is suggested by colocalisation with anterograde cargo trapped in pre-Golgi structures and modification of its N-linked carbohydrate by glycosylation enzymes of medial Golgi cisternae. Furthermore, after brefeldin A treatment VIP36 is segregated from resident Golgi proteins and codistributes with ER-Golgi recycling proteins.  (+info)

(7/366) Effects of N-butyldeoxynojirimycin and the Lec3.2.8.1 mutant phenotype on N-glycan processing in Chinese hamster ovary cells: application to glycoprotein crystallization.

Heterologous gene expression in either (1) the glycosylation-defective, mutant Chinese hamster ovary cell line, Lec3.2.8.1, or (2) the presence of the alpha-glucosidase inhibitor, N-butyldeoxynojirimycin facilitates the trimming of N-linked glycans of glycoproteins to single N-acetylglucosamine (GlcNAc) residues with endoglycosidase H (endo H). Both approaches are somewhat inefficient, however, with as little as 12% of the total protein being rendered fully endo H-sensitive under these conditions. It is shown here that the combined effects of these approaches on the restriction of oligosaccharide processing are essentially additive, thereby allowing the production of glycoproteins that are essentially completely endo H-sensitive. The preparation of a soluble chimeric form of CD58, the ligand of the human T-cell surface recognition molecule CD2, illustrates the usefulness of the combined approach when expression levels are low or the deglycosylated protein is unstable at low pH. The endo H-treated chimera produced crystals of space group P3(1)21 or P3(2)21, and unit cell dimensions a = b = 116.4 A, c = 51.4 A alpha = beta = 90 degrees , gamma = 120 degrees , that diffract to a maximum resolution of 1.8 A.  (+info)

(8/366) High-mannose type oligosaccharide-dependent apoptosis in U937 cells induced by pradimicin, a mannose-binding antibiotic.

Cell surface oligosaccharides play a role in a variety of biological events such as cell adhesion and signal transduction. We have shown that BMY-28864, a semi-synthetic analog of pradimicin, induced apoptosis of U937 cells which had been incubated with 1-deoxymannojirimycin, an inhibitor of mannosidase I. BMY-28864 was not cytotoxic to the cells which had been cultivated with other glycosidase inhibitors such as castanospermine and swainsonine. We thus propose that BMY-28864 induces apoptosis by acting on a specific mannose-rich oligosaccharide, presumably (Man)9(GlcNAc)2+.  (+info)

*  Miglustat
... (OGT 918, N-butyl-deoxynojirimycin) is a drug developed by Oxford GlycoSciences and marketed by Actelion and is used ... Yukhananov, Anna (1 May 2012). "U.S. FDA approves Pfizer/Protalix drug for Gaucher". Chicago Tribune. Reuters. Retrieved 2 May ... European Medicines Agency 1 April 2003 Scientific discussion related to approval of Zavesca. Clinicaltrials.gov ... Zavesca approved -- first oral treatment option for type 1 Gaucher disease UK Medicines Information. New Drugs Online Report ...
*  Alpha-glucosidase
Retrieved 1 March 2012. Wu XQ, Xu H, Yue H, Liu KQ, Wang XY (December 2009). "Inhibition kinetics and the aggregation of alpha- ... Retrieved 1 March 2012. Yoshimizu, M.; Tajima, Y; Matsuzawa, F; Aikawa, S; Iwamoto, K; Kobayashi, T; Edmunds, T; Fujishima, K; ... 306 (1): 188-94. doi:10.1006/abbi.1993.1499. PMID 8215402. Tadera K, Minami Y, Takamatsu K, Matsuoka T (April 2006). " ... It was shown that 1-deoxynojirimycin (DNJ) would bind the strongest of the sugars tested and blocked the active site of the ...
*  1-Deoxynojirimycin
Deoxynojirimycin. Chaluntorn Vichasilp; et al. (2012). "Development of high 1-deoxynojirimycin (DNJ) content mulberry tea and ... 1-Deoxynojirimycin (DNJ), also called duvoglustat or moranolin, is an alpha-glucosidase inhibitor, most commonly found in ... "Production of the α-glycosidase inhibitor 1-deoxynojirimycin from Bacillus species". Food chemistry. 138 (1): 516-23. doi: ... Nojirimycin 1-Deoxygalactonojirimycin, a stereoisomer of 1-deoxynojirimycin KEGG: ...
*  GANC
187 (1): 377-82. doi:10.1016/0042-6822(92)90331-I. PMID 1736542. Murphy CI, Lennick M, Lehar SM, Beltz GA, Young E (Oct 1990 ... 141 (1): 33-8. doi:10.1016/S0006-291X(86)80330-8. PMID 3099781. Usuki F, Ishiura S, Nonaka I, Sugita H (Apr 1988). "alpha- ... 181 (1): 180-92. doi:10.1016/0042-6822(91)90483-R. PMID 1704656. Dedera DA, Gu RL, Ratner L (Mar 1992). "Role of asparagine- ... 1 (1): 17-23. doi:10.1093/glycob/1.1.17. PMID 2136376. Land A, Braakman I (Aug 2001). "Folding of the human immunodeficiency ...
*  Cathepsin D
ISBN 978-1-4051-1922-1. Baechle D, Flad T, Cansier A, Steffen H, Schittek B, Tolson J, Herrmann T, Dihazi H, Beck A, Mueller GA ... 44 (1): 73-80. doi:10.1007/s00726-011-1089-6. PMID 21960143. Devosse T, Dutoit R, Migeotte I, De Nadai P, Imbault V, Communi D ... 9 (1): 1-9. doi:10.1089/dna.1990.9.1. PMID 2180427. Capony F, Rougeot C, Montcourrier P, Cavailles V, Salazar G, Rochefort H ( ... 46 (1): 23-38. doi:10.2478/v10042-008-0003-x. PMID 18296260. Briozzo P, Morisset M, Capony F, Rougeot C, Rochefort H (July 1988 ...
*  MAN2A1
122 (1): 39-51. doi:10.1083/jcb.122.1.39. PMC 2119607 . PMID 8314846. Misago M, Liao YF, Kudo S, Eto S, Mattei MG, Moremen KW, ... 67 (1): 150-60. PMC 237347 . PMID 8093218. Yeh JC, Seals JR, Murphy CI, van Halbeek H, Cummings RD (Oct 1993). "Site-specific N ... 141 (1): 33-8. doi:10.1016/S0006-291X(86)80330-8. PMID 3099781. Montefiori DC, Robinson WE, Mitchell WM (Dec 1988). "Role of ... 187 (1): 377-82. doi:10.1016/0042-6822(92)90331-I. PMID 1736542. Moremen KW, Touster O, Robbins PW (Sep 1991). "Novel ...
*  GANAB
181 (1): 180-92. doi:10.1016/0042-6822(91)90483-R. PMID 1704656. Dedera DA, Gu RL, Ratner L (1992). "Role of asparagine-linked ... 187 (1): 377-82. doi:10.1016/0042-6822(92)90331-I. PMID 1736542. Murphy CI, Lennick M, Lehar SM, Beltz GA, Young E (1991). " ... 1 (1): 17-23. doi:10.1093/glycob/1.1.17. PMID 2136376. Land A, Braakman I (2001). "Folding of the human immunodeficiency virus ... 141 (1): 33-8. doi:10.1016/S0006-291X(86)80330-8. PMID 3099781. Montefiori DC, Robinson WE, Mitchell WM (1988). "Role of ...
*  Acid alpha-glucosidase
1 (1): 17-23. doi:10.1093/glycob/1.1.17. PMID 2136376. Reuser AJ, Kroos MA, Hermans MM, et al. (1995). "Glycogenosis type II ( ... 181 (1): 180-92. doi:10.1016/0042-6822(91)90483-R. PMID 1704656. Dedera DA, Gu RL, Ratner L (1992). "Role of asparagine-linked ... 187 (1): 377-82. doi:10.1016/0042-6822(92)90331-I. PMID 1736542. Hermans MM, Kroos MA, van Beeumen J, et al. (1991). "Human ... Lysosomal alpha-glucosidase, also called α-1,4-glucosidase and acid maltase, is an enzyme (EC 3.2.1.20) that in humans is ...
*  Nojirimycin
1-deoxynojirimycin or duvoglustat 1-deoxygalactonojirimycin or migalastat, a drug for the treatment of Fabry disease Inouye, S ...
*  GCS1
181 (1): 180-92. doi:10.1016/0042-6822(91)90483-R. PMID 1704656. Dedera DA, Gu RL, Ratner L (1992). "Role of asparagine-linked ... 1 (1): 17-23. doi:10.1093/glycob/1.1.17. PMID 2136376. Land A, Braakman I (2001). "Folding of the human immunodeficiency virus ... 187 (1): 377-82. doi:10.1016/0042-6822(92)90331-I. PMID 1736542. Murphy CI, Lennick M, Lehar SM, et al. (1991). "Temporal ... 141 (1): 33-8. doi:10.1016/S0006-291X(86)80330-8. PMID 3099781. Montefiori DC, Robinson WE, Mitchell WM (1988). "Role of ...
*  ALG13
93 (1): 35-41. doi:10.1007/BF00218910. PMID 7505766. Venter JC, Adams MD, Myers EW, et al. (2001). "The sequence of the human ... 36 (1): 40-5. doi:10.1038/ng1285. PMID 14702039. Oh JH, Yang JO, Hahn Y, et al. (2005). "Transcriptome analysis of human ... 200 (1-2): 149-56. doi:10.1016/S0378-1119(97)00411-3. PMID 9373149. Gerhard DS, Wagner L, Feingold EA, et al. (2004). "The ... 16 (1): 55-65. doi:10.1101/gr.4039406. PMC 1356129 . PMID 16344560. Suzuki Y, Yoshitomo-Nakagawa K, Maruyama K, et al. (1997 ...
*  MGAT2
Alpha-1,6-mannosyl-glycoprotein 2-beta-N-acetylglucosaminyltransferase is an enzyme that in humans is encoded by the MGAT2 gene ... 67 (1): 150-60. PMC 237347 . PMID 8093218. Yeh JC, Seals JR, Murphy CI, et al. (1993). "Site-specific N-glycosylation and ... 406 (1-2): 191-5. doi:10.1016/S0014-5793(97)00273-1. PMID 9109416. GeneReviews/NCBI/NIH/UW entry on Congenital Disorders of ... 141 (1): 33-8. doi:10.1016/S0006-291X(86)80330-8. PMID 3099781. Montefiori DC, Robinson WE, Mitchell WM (1988). "Role of ...
*  MAN2B2
6 (1): 63-70. doi:10.1093/dnares/6.1.63. PMID 10231032. Park C, Meng L, Stanton LH, Collins RE, Mast SW, Yi X, Strachan H, ... 67 (1): 150-60. PMC 237347 . PMID 8093218. Yeh JC, Seals JR, Murphy CI, et al. (1993). "Site-specific N-glycosylation and ... 406 (1-2): 191-5. doi:10.1016/S0014-5793(97)00273-1. PMID 9109416. Hiramoto S, Tamba M, Kiuchi S, et al. (1998). "Stage- ... 141 (1): 33-8. doi:10.1016/S0006-291X(86)80330-8. PMID 3099781. Montefiori DC, Robinson WE, Mitchell WM (1988). "Role of ...
*  MAN1B1
67 (1): 150-60. PMC 237347 . PMID 8093218. Yeh JC, Seals JR, Murphy CI, et al. (1993). "Site-specific N-glycosylation and ... 141 (1): 33-8. doi:10.1016/S0006-291X(86)80330-8. PMID 3099781. Montefiori DC, Robinson WE, Mitchell WM (1988). "Role of ... 187 (1): 377-82. doi:10.1016/0042-6822(92)90331-I. PMID 1736542. Kalyanaraman VS, Rodriguez V, Veronese F, et al. (1990). " ... 406 (1-2): 191-5. doi:10.1016/S0014-5793(97)00273-1. PMID 9109416. Tremblay LO, Campbell Dyke N, Herscovics A (1998). " ...
*  MAN1A1
Mannosyl-oligosaccharide 1,2-alpha-mannosidase IA is an enzyme that in humans is encoded by the MAN1A1 gene. This gene encodes ... 67 (1): 150-60. PMC 237347 . PMID 8093218. Yeh JC, Seals JR, Murphy CI, et al. (1993). "Site-specific N-glycosylation and ... 406 (1-2): 191-5. doi:10.1016/S0014-5793(97)00273-1. PMID 9109416. Bieberich E, Treml K, Völker C, et al. (1997). "Man9- ... 141 (1): 33-8. doi:10.1016/S0006-291X(86)80330-8. PMID 3099781. Montefiori DC, Robinson WE, Mitchell WM (1988). "Role of ...
*  Migalastat
Depending on the mutation, the EC50 is between 0.8 µM and over 1 mM in cellular models. The enzyme alpha-galactosidase A (α- ... Migalastat is used in form of the hydrochloride, which is a white crystalline solid and is soluble in water (≥1 mg/mL). The ... 1 April 2016. "Public summary of opinion on orphan designation". European Medicines Agency. 29 April 2014. Clinical trial ... The most common side effect in clinical trials was headache (in about 10% of patients). Less common side effects (between 1 and ...
*  Iminosugar
The 1-deoxy analogs of iminosugars are C-glycosides, with the nitrogen as part of an ordinary amine linkage. Their piperidine, ... The first iminosugar to be isolated from a natural source, 1-deoxynojirimycin (DNJ), found in Mulberry, was reported in 1976, ... In terms of biochemical activity for medicinal applications, DNJ and 1,4-dideoxy-1,4-imino-D-arabinitol (DAB, another early ...
*  List of MeSH codes (D03)
2-ethyl-1,3,4,6,7,11b-hexahydro-3-isobutyl-9,10-dimethoxy- MeSH D03.438.834.775 --- sparteine MeSH D03.438.834.850 --- ... 5-dihydro-1-(3-(trifluoromethyl)phenyl)-1h-pyrazol-3-amine MeSH D03.383.129.539.200 --- epirizole MeSH D03.383.129.539.487 --- ... 1,4-dihydro-2,6-dimethyl-5-nitro-4-(2-(trifluoromethyl)phenyl)-, methyl ester MeSH D03.383.725.547.950 --- xanthinol niacinate ... 1,4-dihydro-2,6-dimethyl-5-nitro-4-(2-(trifluoromethyl)phenyl)-, methyl ester MeSH D03.383.725.210 --- dimethindene MeSH ...
*  Streptomyces subrutilus
... produces deoxynojirimycin, deoxymannonojirimycin and hydroxystreptomycin. So, NW; Rho, JY; Lee, SY; ... "The biosynthesis of deoxynojirimycin and deoxymannonojirimycin in Streptomyces subrutilus". Journal of the Chemical Society, ... 194 (1): 93-8. doi:10.1016/s0378-1097(00)00514-0. PMID 11150672. Nemr, El Sayed H. El Ashry, Ahmed El (2007). Synthesis of ... ISBN 1-4051-4479-3. al.], Senior reporter, R.J. Ferrier ; reporters, R. Blattner-- [et (1993). Carbohydrate chemistry. a review ...
*  Glycoside hydrolase
Nitrogen-containing, 'sugar-shaped' heterocycles have been found in nature, including deoxynojirimycin, swainsonine, australine ... 3 (1): 26-44. doi:10.2174/22115501113026660041. Fleming, Derek; Rumbaugh, Kendra P. (2017-04-01). "Approaches to Dispersing ... Glycoside hydrolases are classified into EC 3.2.1 as enzymes catalyzing the hydrolysis of O- or S-glycosides. Glycoside ... 2006, 1, 294-298. Naumoff, D.G. (2011). "Hierarchical classification of glycoside hydrolases". Biochemistry (Moscow). 76 (6): ...
*  Sandhoff disease
Currently the government is testing several treatments including N-butyl-deoxynojirimycin in mice, as well as stem cell ... 318 (1-2): 133-7. doi:10.1016/S0009-8981(02)00002-5. PMID 11880123. Kuliev A, Rechitsky S, Laziuk K, Verlinsky O, Tur-Kaspa I, ... 3 (1): 139-145. doi:10.1093/hmg/3.1.139. PMID 8162015. "From a parents perspective: Parents view of Sandhoff". sandhoffdisease. ... 107 (1): 12-17. doi:10.1007/s004390050003. PMID 10982028. Cantor RM, Kaback MM (1985). "Sandhoff disease (SHD) heterozygote ...
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EDIBLE FAT-CONTINUOUS EMULSION COMPRISING 1-DEOXYNOJIRIMYCIN AND LECITHIN - UNILEVER N.V.  EDIBLE FAT-CONTINUOUS EMULSION COMPRISING 1-DEOXYNOJIRIMYCIN AND LECITHIN - UNILEVER N.V.
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RCSB PDB - 1DOG: REFINED STRUCTURE FOR THE COMPLEX OF 1-DEOXYNOJIRIMYCIN WITH GLUCOAMYLASE FROM (ASPERGILLUS AWAMORI) VAR. X100...  RCSB PDB - 1DOG: REFINED STRUCTURE FOR THE COMPLEX OF 1-DEOXYNOJIRIMYCIN WITH GLUCOAMYLASE FROM (ASPERGILLUS AWAMORI) VAR. X100...
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1-deoxynojirimycin-1-sulfonic acid | Ligand page | IUPHAR/BPS Guide to PHARMACOLOGY  1-deoxynojirimycin-1-sulfonic acid | Ligand page | IUPHAR/BPS Guide to PHARMACOLOGY
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Generation of specific deoxynojirimycin-type inhibitors of the non-lysosomal glucosylceramidase. J Biol Chem 273: 26522-26527. ... deoxynojirimycin (AMP-DNM) that inhibits glucosylceramide synthase. Here, we treated the liver hepatoma cell line HepG2 with ... deoxynojirimycin Induces Sterol Regulatory Element-Binding Protein-Regulated Gene Expression and Cholesterol Synthesis in HepG2 ... deoxynojirimycin; SREBP, sterol regulatory element-binding protein; DMEM/HAMF-12, Dulbecco's modified Eagle's medium/Ham's ...
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1-Deoxynojirimycin. Antiviral Agents. Anti-Infective Agents. Enzyme Inhibitors. Molecular Mechanisms of Pharmacological Action ... or myocardial infarction within the 3 months before Visit 1 ... Visit 1), which is ,30 mL/min/1.73m2 ...
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Stage 1 includes a screening period of up to 2 months followed by a 6-month treatment period which will involve 4 visits to the ... Subjects who complete both Stage 1 and Stage 2 of the study as scheduled may be offered the opportunity to participate in an ... 1-Deoxynojirimycin. Antiviral Agents. Anti-Infective Agents. Enzyme Inhibitors. Molecular Mechanisms of Pharmacological Action ... A follow-up visit will be undertaken 1 month following completion or discontinuation from the open-label treatment extension. ...
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  • One inhibitor molecule is associated with strong electron density and represents the principal site of interaction of 1-deoxynojirimycin with the enzyme. (rcsb.org)
  • Interactions of 1-deoxynojirimycin with the enzyme at its principal site involve Arg 45, Asp 55, Arg 305, and carbonyl 177. (rcsb.org)
  • For the treatment of adult patients with mild to moderate type 1 (nonneuropathic) Gaucher's disease for whom enzyme replacement therapy is not a therapeutic option (e.g. due to constraints such as allergy, hypersensitivity, or poor venous access). (drugbank.ca)
  • This enzyme hydrolyses terminal, non-reducing 1,4-linked alpha-D-glucose residues and releases alpha-D-glucose. (wikipedia.org)
  • In addition, a water molecule (water 500) hydrogen bonds to Glu 400 and the 6-hydroxyl of 1-deoxynojirimycin and is at an approximate distance of 3.3 A from the "anomeric" carbon of the inhibitor. (rcsb.org)
  • The distinct effect exerted by various glycosidase inhibitors (e.g. 1-deoxynojirimycin, N-methyl-dNM, 1-deoxymannojirimycin) on the electrophoretic mobility of the pp-α-F 3 polypeptide indicates that its oligosaccharide chains are processed to presumbly Man 9 -GlcNAc 2 structures under the in vitro conditions of translation. (bioscirep.org)
  • The method (1) comprises the steps of disposing (S1) flour in a first container, providing (S2) an electrolyte solution (S) in a second container, circulating (S3) an elec. (sumobrain.com)
  • The model includes residues corresponding to residues 1-471 of glucoamylase I from Aspergillus niger, two molecules of bound 1-deoxynojirimycin and 605 sites for water molecules. (rcsb.org)
  • As a consequence of this improved function of fat tissue we observed less inflammation, which was characterized by reduced numbers of adipose tissue macrophages (crown-like structures) and reduced levels of the macrophage chemo attractants monocyte-chemoattractant protein-1 (Mcp-1/Ccl2) and osteopontin (OPN). (nih.gov)
  • They hydrolyze terminal (1,4)alpha-glucosidic linkages and (1,6)beta-glucosidic linkages, liberating alpha-glucose and beta-glucose. (genecards.org)
  • For PC-12 cells pretreated with 1-deoxymannojirimycin at 1mM for 72h, thawed cell viability after more than 8-w cryopreservation at -80 degrees C in 10% (v/v) dimethyl sulfoxide was much higher than that for cells without pretreatment. (biomedsearch.com)
  • Of the 56 up-regulated genes, 17 were direct target genes for transcription factors sterol regulatory element-binding protein (SREBP) 1 or SREBP2, which activate genes in the sterol biosynthesis pathway. (aspetjournals.org)
  • MAN2B1 (Mannosidase Alpha Class 2B Member 1) is a Protein Coding gene. (genecards.org)
  • Subjects who complete both Stage 1 and Stage 2 of the study as scheduled may be offered the opportunity to participate in an open-label treatment extension phase with AT1001. (clinicaltrials.gov)
  • An edible fat-continuous emulsion comprising 1-deoxynojirimycin (DNJ) and lecithin, wherein the lecithin comprises phosphatidylcholine (PC), phosphatidylinositol (PI), and phosphatidylethanolamine (PE) wherein the weight ratio (PC+PI)/PE is at least 2.5. (sumobrain.com)
  • Emulsion according to claim 1 or 2, wherein the emulsion comprises from 0.002 to 0.05 % by weight, preferably from 0.003 to 0.04 % by weight on the emulsion of 1-deoxynojirimycin (DNJ). (sumobrain.com)
  • PKC-epsilon activity was well preserved after 16-h cryopreservation at -20 degrees C in the presence of mannose 9-N-acetylglucosamine 2 (Man9-GlcNAc2) (1 mM), an HMOS, while the activity was reduced to 15% without Man9-GlcNAc2. (biomedsearch.com)
  • GO annotations related to this gene include carbohydrate binding and alpha-1,4-glucosidase activity . (genecards.org)
  • Package: 1-20kg by Aluminum foil bag, 25kg/drum, or according to customer requirement. (made-in-china.com)
  • Stage 1 includes a screening period of up to 2 months followed by a 6-month treatment period which will involve 4 visits to the clinic. (clinicaltrials.gov)