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Merck
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주요 문서

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43659

Sigma-Aldrich

Glucosylsphingosine

≥98.0% (TLC)

동의어(들):

(2S,3R,4E)-2-Amino-3-hydroxy-4-octadecen-1-yl β-D-glucopyranoside, 1-β-D-Glucosylsphingosine, Glucosyl-C18-sphingosine

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모든 사진(1)

About This Item

실험식(Hill 표기법):
C24H47NO7
CAS Number:
52050-17-6
Molecular Weight:
461.63
Beilstein:
4333674
MDL number:
MFCD07374303
UNSPSC 코드:
12352211
PubChem Substance ID:
329756364
NACRES:
NA.85

Quality Level

100

분석

≥98.0% (TLC)

양식

powder

지질 유형

sphingolipids

저장 온도

−20°C

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

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

생화학적/생리학적 작용

Glucosylsphingosine is a cytotoxic compound. Accumulation of glucosylsphingosine in brain and other tissues occurs in patients with Gaucher disease, which is an inherited deficiency of lysosomal glucocerebrosidase, which converts glucosylsphingosine to glucose and sphingosine.

Storage Class Code

11 - Combustible Solids

WGK

WGK 3

Flash Point (°F)

Not applicable

Flash Point (°C)

Not applicable

가장 최신 버전 중 하나를 선택하세요:

시험 성적서(COA)

Lot/Batch Number
BCCG2390
BCCD1062
BCCB9443
BCBZ6356
BCBW4402

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문서 라이브러리 방문

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;
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
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