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Yukari Fujita et al.
Internal medicine (Tokyo, Japan), 51(18), 2581-2585 (2012-09-20)
We herein describe a 59-year-old woman who had undergone a total gastrectomy for gastric carcinoma and suffered from postprandial hypoglycemia characterized by a loss of consciousness and spasms. She was diagnosed with reactive hypoglycemia and treated with nutrition therapy, but
I Neverova et al.
Analytical biochemistry, 222(1), 190-195 (1994-10-01)
A spectrophotometric assay for glucosidase I using the synthetic trisaccharide alpha-D-Glc 1-->2 alpha-D-Glc 1-->3 alpha-D-Glc-O(CH2)8COOCH3 is reported. The terminal glucose is released from the substrate by the enzyme and quantitated using glucose oxidase, peroxidase, and o-dianisidine. The trisaccharide is specific
Gustavo Tiscornia et al.
Human molecular genetics, 22(4), 633-645 (2012-11-03)
Gaucher's disease (GD) is caused by mutations in the GBA1 gene, which encodes acid-β-glucosidase, an enzyme involved in the degradation of complex sphingolipids. While the non-neuronopathic aspects of the disease can be treated with enzyme replacement therapy (ERT), the early-onset
Timothy M Cox et al.
Orphanet journal of rare diseases, 7, 102-102 (2012-12-29)
Previous studies have provided equivocal data on the use of miglustat as maintenance therapy in Gaucher disease type 1. We report findings from a clinical trial evaluating the effects of miglustat treatment in patients with stable type 1 Gaucher disease
Prescrire international, 21(130), 212-213 (2012-09-29)
Detailed imaging studies of gas cysts in the intestinal mucosa (pneumatosis cystoides intestinalis) have been reported in around 20 patients taking acarbose, miglitolor voglibose, i.e. intestinal alpha-glucosidase inhibitors used as hypoglycaemic agents. Almost all the patients had nonspecific abdominal symptoms
Joanna L Miller et al.
Antimicrobial agents and chemotherapy, 56(12), 6379-6386 (2012-10-17)
A key challenge faced by promising antiviral drugs, such as iminosugars, is in vivo delivery to achieve effective levels of drug without toxicity. Four iminosugars, all deoxynojirimycin (DNJ) derivatives-N-butyl DNJ (NB-DNJ), N-nonyl DNJ, N-(9-methoxynonyl) DNJ, and N-(6'-[4″-azido-2″-nitrophenylamino]hexyl)-1-DNJ (NAP-DNJ)-potently inhibited both
Manabu Nakashita et al.
Digestive diseases and sciences, 58(2), 354-362 (2012-08-25)
Ceramide has been suggested to play a role in apoptosis during gastric ulcerogenesis. The present study is designed to investigate whether accumulated ceramide could serve as the effector molecules of ulcer formation in a rat model of acetic acid-induced gastric
Stephen G Davies et al.
Organic letters, 15(8), 2042-2045 (2013-04-11)
The asymmetric syntheses of (-)-1-deoxymannojirimycin and (+)-1-deoxyallonojirimycin are described herein. The ring-closing iodoamination of two epimeric bishomoallylic amines to give the corresponding 5-iodomethylpyrrolidines was followed by in situ ring-expansion to give two diastereoisomerically pure (>99:1 dr) cyclic carbonates. Subsequent deprotection
Phedias Diamandis et al.
Nature chemical biology, 3(5), 268-273 (2007-04-10)
The identification of self-renewing and multipotent neural stem cells (NSCs) in the mammalian brain holds promise for the treatment of neurological diseases and has yielded new insight into brain cancer. However, the complete repertoire of signaling pathways that governs the
Chaluntorn Vichasilp et al.
Food chemistry, 134(4), 1823-1830 (2013-02-28)
Mulberry 1-deoxynojirimycin (DNJ), a potent α-glycosidase inhibitor, has therapeutic potency in the suppression of postprandial blood glucose levels thereby possibly preventing diabetes mellitus. However, DNJ has a relatively short half-life in vivo (about 2 h). Therefore, several doses of mulberry
Myung-Ji Seo et al.
Bioscience, biotechnology, and biochemistry, 77(2), 398-401 (2013-02-09)
The 1-deoxynojirimycin (DNJ) biosynthetic gene cluster of Bacillus amyloliquefaciens 140N isolated from traditional Korean fermented food was isolated by PCR screening. It showed 78.9% inhibitory activity against α-glucosidase and produced 0.8 g/L of DNJ in an optimized medium containing 2%
Chie Aoki et al.
Journal of pharmacological sciences, 120(2), 121-128 (2012-09-29)
Endothelial dysfunction caused by oxidative stress plays a key role in atherogenesis. This study investigated whether the anti-diabetic drug miglitol, an α-glucosidase inhibitor, which is currently available in clinical practice, can prevent endothelial cell apoptosis and whether it might restore
Keita Ishibashi et al.
Biochemical and biophysical research communications, 440(4), 570-575 (2013-10-10)
Dipeptidyl peptidase-4 (DPP-4) inhibitors improve glycemic control in patients with type 2 diabetes primarily by increasing plasma active glucagon-like peptide-1 (GLP-1) levels. While various combination therapies based on DPP-4 inhibitors have been proposed for treatment of type 2 diabetes, the
Mahdi Amiri et al.
Journal of inherited metabolic disease, 35(6), 949-954 (2012-09-15)
Miglustat is an oral medication that has approved indication for type I Gaucher disease and Niemann pick disease type C. Usually treatment with Miglustat is associated with occurrence of gastrointestinal side effects similar to carbohydrate maldigestion symptoms. Here, we studied
You-gui Li et al.
Chemico-biological interactions, 225, 70-79 (2014-12-03)
Type 2 diabetes mellitus (T2DM) is currently considered a worldwide epidemic and finding effective therapeutic strategies against this disease is highly important. A deoxynojirimycin-polysaccharide mixture (DPM) has previously been shown to exert hypoglycemic effects on alloxan- or streptozotocin (STZ)-induced diabetic
Mark Walterfang et al.
Orphanet journal of rare diseases, 7, 76-76 (2012-10-09)
Niemann-Pick disease type C (NP-C) is a rare neurovisceral disease characterised by progressive neurological deterioration and premature death, and has an estimated birth incidence of 1:120,000. Mutations in the NPC1 gene (in 95% of cases) and the NPC2 gene (in
Shinji Onose et al.
Food chemistry, 138(1), 516-523 (2012-12-26)
1-Deoxynojirimycin (DNJ), a potent α-glycosidase inhibitor, has therapeutic applications in treatments of HIV, Gaucher's disease, and diabetes. DNJ has been extracted from natural sources (mulberry leaves) for therapeutic purposes; however, DNJ ingredients are in limited supply and are costly to
Makoto Ogata et al.
The Journal of biological chemistry, 288(9), 6072-6082 (2013-01-11)
4-O-β-Di-N-acetylchitobiosyl moranoline (2) and 4-O-β-tri-N-acetylchitotriosyl moranoline (3) were produced by lysozyme-mediated transglycosylation from the substrates tetra-N-acetylchitotetraose, (GlcNAc)4, and moranoline, and the binding modes of 2 and 3 to hen egg white lysozyme (HEWL) was examined by inhibition kinetics, isothermal titration
S V Kyosseva et al.
Archives of biochemistry and biophysics, 316(2), 821-826 (1995-02-01)
Trehazolin, a new trehalase inhibitor isolated from the culture broth of Micromonospora, was reported to be a highly specific inhibitor for porcine and silk worm trehalases with IC50 values of 5.5 x 10(-9) and 3.7 x 10(-9) M, respectively (O.
A V Timokhova et al.
Molekuliarnaia biologiia, 46(4), 644-653 (2012-11-02)
Viral hepatitis C is one of the wide-spread and dangerous human diseases. The choice of drugs for treatment of chronic hepatitis C virus (HCV) infection is limited and prophylactic vaccines do not exist. Thus, the development of new antiviral strategies
Mike Flint et al.
Antimicrobial agents and chemotherapy, 58(6), 3206-3216 (2014-03-26)
No antiviral therapies are available for the tick-borne flaviviruses associated with hemorrhagic fevers: Kyasanur Forest disease virus (KFDV), both classical and the Alkhurma hemorrhagic fever virus (AHFV) subtype, and Omsk hemorrhagic fever virus (OHFV). We tested compounds reported to have
Giorgio Catelani et al.
Bioorganic & medicinal chemistry letters, 20(15), 4645-4648 (2010-06-29)
Deoxynojirimycin (1) and two new related 4-O-hexosaminyl-containing disaccharide mimics, beta-d-TalNAc-(1-->4)-DNJ (4) and beta-d-ManNAc-(1-->4)-DNJ (5), have been studied as agonists of natural killer (NK) cell receptors. As a positive and unexpected result, DNJ (1) displayed a remarkable activation effect towards both
Elisa Lombardo et al.
PloS one, 7(10), e38520-e38520 (2012-10-12)
The iminosugar N-(5'-adamantane-1'-yl-methoxy)-pentyl-1-deoxynoijirimycin (AMP-DNM), an inhibitor of glycosphingolipid (GSL) biosynthesis is known to ameliorate diabetes, insulin sensitivity and to prevent liver steatosis in ob/ob mice. Thus far the effect of GSL synthesis inhibition on pre-existing NASH has not yet been
[Niemann-Pick type C disease. Rare but possible diagnosis in adult neurology and psychiatry].
Niklas Mattsson et al.
Lakartidningen, 110(19-20), 956-957 (2013-06-12)
Alexandra Lipski et al.
Acta crystallographica. Section D, Biological crystallography, 69(Pt 2), 298-307 (2013-02-07)
Sucrose isomerase is an enzyme that catalyzes the production of sucrose isomers of high biotechnological and pharmaceutical interest. Owing to the complexity of the chemical synthesis of these isomers, isomaltulose and trehalulose, enzymatic conversion remains the preferred method for obtaining
Zygmunt Jamrozik et al.
Neurologia i neurochirurgia polska, 47(1), 86-89 (2013-03-15)
Niemann-Pick disease type C is a rare hereditary disorder caused by mutation-disrupted metabolism of cholesterol and low-density lipoprotein (LDL). In most patients, symptoms begin in childhood with severe clinical progression. We present a patient with heterozygote mutations 3001A>G and 3019C>G
Marc C Patterson et al.
Orphanet journal of rare diseases, 8, 12-12 (2013-01-18)
Niemann-Pick disease type C (NP-C) is a rare neurovisceral disease characterized by progressive neurodegeneration and premature death. We report data recorded at enrolment in an ongoing international NP-C registry initiated in September 2009 to describe disease natural history, clinical course
Yoji Hamada et al.
Metabolism: clinical and experimental, 62(5), 734-742 (2012-12-01)
Alpha-glucosidase inhibitors (α-GIs) show various anti-diabetic or anti-obesity effects in addition to the suppression of postprandial hyperglycemia. Based on recent observations that bile acids (BAs) are involved in glucose and energy homeostasis, we examined the ability of miglitol, an α-GI
Alison S Thomas et al.
British journal of haematology, 165(4), 427-440 (2014-03-05)
Gaucher disease (GD) is an autosomal recessive lysosomal storage disease, caused by deficiency of the enzyme glucocerebrosidase, required for the degradation of glycosphingolipids. Clinical manifestations include hepatosplenomegaly, thrombocytopenia, bone disease and a bleeding diathesis, frequently resulting in presentation to haematologists.
Christina M Ridley et al.
The Journal of biological chemistry, 288(36), 26052-26066 (2013-07-25)
β-Glucosidase 2 (GBA2) is an enzyme that cleaves the membrane lipid glucosylceramide into glucose and ceramide. The GBA2 gene is mutated in genetic neurological diseases (hereditary spastic paraplegia and cerebellar ataxia). Pharmacologically, GBA2 is reversibly inhibited by alkylated imino sugars
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