おすすめの製品
品質水準
アッセイ
≥98.0% (TLC)
形状
powder
脂質タイプ
sphingolipids
保管温度
−20°C
SMILES記法
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
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
InChI Key
HHJTWTPUPVQKNA-JLRUQHRASA-N
生物化学的／生理学的作用
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。
保管分類コード
11 - Combustible Solids
WGK
WGK 3
引火点(°F)
Not applicable
引火点(℃)
Not applicable
試験成績書（COA）
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E M Kaye et al.
Annals of neurology, 20(2), 223-230 (1986-08-01)
Glucocerebroside levels were measured in the brains of patients with neuronopathic forms (types 2 and 3) of Gaucher disease and compared to those obtained from control brain. Nine separate brain regions (frontal, temporal, occipital, and cerebellar cortices; thalamus; corpus striatum;
A convenient approach to facilitate monitoring Gaucher disease progression and therapeutic response.
Wujuan Zhang et al.
The Analyst, 142(18), 3380-3387 (2017-08-16)
Gaucher disease (GD) is caused by mutations on the GBA1 gene leading to deficiency in acid β-glucosidase (GCase) and subsequent accumulation of its substrates, glucosylceramide (GlcC) and glucosylsphingosine (GlcS). GlcS in plasma has been proposed as a highly sensitive and
Chengfang Tang et al.
Clinical biochemistry, 87, 79-84 (2020-11-15)
Gaucher disease (GD) is caused by a deficiency of β-glucosidase (GCase), leading to accumulation of glucosylceramide (GlcC) and glucosylsphingosine (Lyso-Gb1). Lyso-Gb1 is a reliable biomarker for GD. This study aims to develop a simple, effective and accurate method for the
Ellen Sidransky
Molecular genetics and metabolism, 83(1-2), 6-15 (2004-10-07)
Gaucher disease, the recessively inherited deficiency of the enzyme glucocerebrosidase and the most common sphingolipidosis, has both non-neurological and neuronopathic forms and a continuum of diverse clinical manifestations. Studies of genotype-phenotype correlations reveal significant genotypic heterogeneity among clinically similar patients
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
ライフサイエンス、有機合成、材料科学、クロマトグラフィー、分析など、あらゆる分野の研究に経験のあるメンバーがおります。.
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