43659
グルコシルシフィンゴシン
≥98.0% (TLC)
別名:
1-β-D-グルコシルシフィンゴシン, グルコシル-C18-スフィンゴシン, （2S,3R,4E）-2-アミノ-3-ヒドロキシ-4-オクタデセン-1-イル β-D-グルコピラノシド
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この商品について
実験式（ヒル表記法）:
C24H47NO7
CAS番号:
分子量:
461.63
MDL number:
UNSPSC Code:
12352211
PubChem Substance ID:
NACRES:
NA.85
Beilstein/REAXYS Number:
4333674
製品名
グルコシルシフィンゴシン, ≥98.0% (TLC)
SMILES string
O[C@@H]1[C@@H](O)[C@H](OC[C@H](N)[C@H](O)/C=C/CCCCCCCCCCCCC)O[C@H](CO)[C@H]1O
InChI key
HHJTWTPUPVQKNA-JLRUQHRASA-N
InChI
1S/C24H47NO7/c1-2-3-4-5-6-7-8-9-10-11-12-13-14-15-19(27)18(25)17-31-24-23(30)22(29)21(28)20(16-26)32-24/h14-15,18-24,26-30H,2-13,16-17,25H2,1H3/b15-14+/t18-,19+,20?,21+,22+,23?,24+/m0/s1
assay
≥98.0% (TLC)
form
powder
lipid type
sphingolipids
storage temp.
−20°C
Quality Level
関連するカテゴリー
Biochem/physiol Actions
グルコシルスフィンゴシンは細胞毒性物質です。ゴーシェ病では、グルコシルスフィンゴシンが脳および他の組織内で蓄積します。この疾患では、リソソームのグルコシルスフィンゴシンをグルコースとスフィンゴシンに変換させる酵素グルコセレブロシダーゼが遺伝的に欠損しています。
保管分類
11 - Combustible Solids
wgk
WGK 3
flash_point_f
Not applicable
flash_point_c
Not applicable
N G Conradi et al.
Acta neuropathologica, 75(4), 385-390 (1988-01-01)
Splenectomy in children with the Norrbottnian type of Gaucher disease is followed by increased blood levels of glucosylceramide and impaired neurological and mental status. High blood levels are associated with an increased accumulation of glucosylceramide in perivascular Gaucher cells in
A Kaloterakis et al.
Journal of internal medicine, 246(6), 587-590 (2000-01-05)
Chronic Gaucher disease [GD] in association with systemic AL amyloidosis is extremely rare. We describe a 46-year-old Greek male with chronic GD confirmed by low glucocerebroside activity in fibroblasts and N370S/L444P mutations at the cerebrosidase gene, who also had systemic
Lulu Kang et al.
Journal of human genetics, 62(8), 763-768 (2017-03-31)
Gaucher disease (GD) is an inherited metabolic disorder that involves accumulation of glycolipid glucocerebroside in monocyte-macrophage cells, which can result in multiple organ damage. Enzyme replacement and substrate reduction therapies have improved the potential for early diagnosis and treatment. Determining
E Beutler
Blood reviews, 2(1), 59-70 (1988-03-01)
Gaucher disease is a glycolipid storage disorder characterized by accumulation of glucocerebroside in the liver, spleen, and bones, and caused by a deficiency of glucocerebrosidase. Glucocerebrosidase cDNA has been cloned and sequenced, and much has been learned about the synthesis
Shiny Nair et al.
Molecular genetics and metabolism, 129(4), 286-291 (2020-02-12)
In Gaucher disease type 1 (GD1), genetic deficiency of lysosomal glucocerebrosidase results in the accumulation of glucosylceramide and glucosylsphingosine (GlcSph), that underlie chronic lipid-mediated metabolic inflammation. An important age-related phenotype is high risk of monoclonal gammopathy (MG), including multiple myeloma.
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