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出典(authority):フリー百科事典『ウィキペディア(Wikipedia)』「2014/03/29 11:07:16」(JST)

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  • Glucocerebrosidase Enhancers for Selected Gaucher Disease Genotypes by Modification of α-1-C-Substituted Imino-D-xylitols (DIXs) by Click Chemistry.
  • Serra-Vinardell J1, Díaz L, Casas J, Grinberg D, Vilageliu L, Michelakakis H, Mavridou I, Aerts JM, Decroocq C, Compain P, Delgado A.
  • ChemMedChem.ChemMedChem.2014 Aug;9(8):1744-54. doi: 10.1002/cmdc.201402023. Epub 2014 Jun 27.
  • A series of hybrid analogues was designed by combination of the iminoxylitol scaffold of parent 1C9-DIX with triazolylalkyl side chains. The resulting compounds were considered potential pharmacological chaperones in Gaucher disease. The DIX analogues reported here were synthesized by CuAAC click ch
  • PMID 24976039
  • Modeling changes in biomarkers in Gaucher disease patients receiving enzyme replacement therapy using a pathophysiological model.
  • Vigan M1, Stirnemann J, Caillaud C, Froissart R, Boutten A, Fantin B, Belmatoug N, Mentré F.
  • Orphanet journal of rare diseases.Orphanet J Rare Dis.2014 Jun 30;9:95. doi: 10.1186/1750-1172-9-95.
  • BACKGROUND: Gaucher disease (GD) is a rare recessively inherited disorder caused by deficiency of a lysosomal enzyme, glucocerebrosidase. Accumulation of glucosylceramide or glucosylsphingosine in macrophages leads to increased production of ferritin and chitotriosidase and to decreases in hemoglobi
  • PMID 24980507
  • A Phase 3, multicenter, open-label, switchover trial to assess the safety and efficacy of taliglucerase alfa, a plant cell-expressed recombinant human glucocerebrosidase, in adult and pediatric patients with Gaucher disease previously treated with imiglucerase.
  • Pastores GM1, Petakov M2, Giraldo P3, Rosenbaum H4, Szer J5, Deegan PB6, Amato DJ7, Mengel E8, Tan ES9, Chertkoff R10, Brill-Almon E11, Zimran A12.
  • Blood cells, molecules & diseases.Blood Cells Mol Dis.2014 Jun 17. pii: S1079-9796(14)00055-2. doi: 10.1016/j.bcmd.2014.05.004. [Epub ahead of print]
  • Taliglucerase alfa is a β-glucosidase enzyme replacement therapy (ERT) approved in the US and other countries for the treatment of Gaucher disease (GD) in adults and is approved in pediatric and adult patients in Australia and Canada. It is the first approved plant cell-expressed recombinant human
  • PMID 24950666


  • Successful Pregnancy and Lactation Outcome in a Patient With Gaucher Disease Receiving Enzyme Replacement Therapy, and the Subsequent Distribution and Excretion of Imiglucerase in Human Breast Milk
  • Sekijima Yoshiki,Ohashi Toya,Kosho Tomoki,Fukushima Yoshimitsu
  • CLINICAL THERAPEUTICS 32(12), 2048-2052, 2010-11
  • … Background: Enzyme replacement therapy (ERT) with imiglucerase is a well-established, effective treatment for Gaucher disease. … However, there have been no published reports regarding the excretion of imiglucerase into human breast milk and its effects on the nursing infant. …
  • NAID 120005248172
  • Gaucher 病酵素補充療法の変更後, 多発骨壊死をきたした1例
  • 阿部 真美,蓑口 まどか,神保 絢子 [他],菊地 陽子,千葉 篤,柴田 好,高後 裕
  • 日本内科学会雑誌 93(11), 2421-2423, 2004-11-10
  • NAID 10014293632
  • Improvement of Splenomegaly and Pancytopenia by Enzyme Replacement Therapy Against Type 1 Gaucher Disease: A Report of Sibling Cases
  • TSUBOI Kazuya,IIDA Shinsuke,KATO Miyuki,HAYAMI Yoshihito,HANAMURA Ichiro,MIURA Kazuhisa,HARADA Shinsuke,KOMATSU Hirokazu,BANNO Shogo,WAKITA Atsushi,NITTA Masakazu,UEDA Ryuzo
  • International journal of hematology 73(3), 356-362, 2001-04
  • NAID 10007320711


Package Label - Principal Display Panel ImigluceraseSeveral structures of glucocerebrosidase Imiglucerase-Cerezyme-inj-200unit-990211  of velaglucerase alfa and imiglucerasePackage Label - Principal Display Panel




  • マクロファージに蓄積する糖脂質グルコセレブロシド分解酵素の改良型でゴーシェ病に適応