神经鞘氨醇半乳糖苷

神经鞘氨醇半乳糖苷用途

精神碱(半乳糖基鞘氨醇)是半乳糖苷酶(GALC)的底物,是克拉贝病的潜在生物标志物[1]。精神碱是一种具有高度细胞毒性的脂质,能够诱导多种细胞类型的细胞死亡,包括与球细胞白质营养不良(GLD)最相关的少突胶质细胞。精神药物至少部分通过细胞凋亡导致细胞死亡。精神药物也是PKC的抑制剂[1]。

神经鞘氨醇半乳糖苷名称

[ CAS 号 ]:
2238-90-6

[ 中文名 ]:
神经鞘氨醇半乳糖苷

[ 英文名 ]:
Psychosine

[英文别名 ]:

Galactosylsphingosine
D-galactosyl-ß1-1'-D-erythro-sphingosine
1-B-D-GALACTOSYLSPHINOGOSINE
Psychosine from bovine brain
PSYCHOSIN
PSYCHOSINE
D-GALACTOSYL-B1-1' SPHINGOSN
LYSO-CERAMIDEGALACTOSIDE
MFCD00057505
O-Galactosylsphingosine

神经鞘氨醇半乳糖苷生物活性

[ 描述 ]:

[ 相关类别 ]:

信号通路 >> 表观遗传学 >> PKC

研究领域 >> 癌症

研究领域 >> 神经疾病

信号通路 >> TGF-beta/Smad 信号通路 >> PKC

[ 靶点 ]

PKC

[体外研究]

精神药物是GALC酶的底物,有望帮助诊断和随访通过新生儿筛查(NBS)确定的高危婴儿[1]。精神药物可诱导多效性效应,包括多种细胞途径的功能障碍[2]。细胞活力测定[2]细胞系：少突胶质细胞,M03。13浓度：10、20和40μM培养时间：24小时结果：至少部分通过凋亡导致细胞死亡。

[参考文献]

[1]. M L Escolar, et al. Psychosine, a marker of Krabbe phenotype and treatment effect. Mol Genet Metab. 2017 Jul;121(3):271-278.

[2]. Jacqueline A Hawkins-Salsbury,et al.Psychosine, the cytotoxic sphingolipid that accumulates in globoid cell leukodystrophy, alters membrane architecture. J Lipid Res.2013 Dec;54(12):3303-11.

神经鞘氨醇半乳糖苷物理化学性质

[ 密度 ]:
1.14 g/cm3

[ 沸点 ]:
664.3ºC

[ 分子式 ]:
C₂₄H₄₇NO₇

[ 分子量 ]:
461.63200

[ 闪点 ]:
355.6ºC

[ 精确质量 ]:
461.33500

[ PSA ]:
145.63000

[ LogP ]:
2.44870

[ 外观性状 ]:
白色粉末

[ 蒸汽压 ]:
1.77E-20mmHg at 25°C

[ 折射率 ]:
1.535

[ 储存条件 ]:
−20°C

神经鞘氨醇半乳糖苷安全信息

[ 符号 ]:

GHS07

[ 信号词 ]:
Warning

[ 危害声明 ]:
H302-H312-H332

[ 警示性声明 ]:
P280

[ 个人防护装备 ]:
dust mask type N95 (US);Eyeshields;Gloves

[ 危害码 (欧洲) ]:
Xn: Harmful;

[ 风险声明 (欧洲) ]:
R20/21/22

[ 安全声明 (欧洲) ]:
36

[ 危险品运输编码 ]:
NONH for all modes of transport

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神经鞘氨醇半乳糖苷文献

How well does urinary lyso-Gb3 function as a biomarker in Fabry disease?

Clin. Chim. Acta 411(23-24) , 1906-14, (2010)

Fabry disease is characterized by accumulation of glycosphingolipids, such as globotriaosylceramide (Gb(3)), in many tissues and body fluids. A novel plasma biomarker, globotriaosylsphingosine (lyso-G...

Aberrant production of tenascin-C in globoid cell leukodystrophy alters psychosine-induced microglial functions.

J. Neuropathol. Exp. Neurol. 73(10) , 964-74, (2014)

Globoid cell leukodystrophy (GLD), or Krabbe disease, is a rare and often fatal demyelinating disease caused by mutations in the galactocerebrosidase (galc) gene that result in accumulation of galacto...

Bone marrow transplantation augments the effect of brain- and spinal cord-directed adeno-associated virus 2/5 gene therapy by altering inflammation in the murine model of globoid-cell leukodystrophy.

J. Neurosci. 31(27) , 9945-57, (2011)

Globoid-cell leukodystrophy (GLD) is an inherited demyelinating disease caused by the deficiency of the lysosomal enzyme galactosylceramidase (GALC). A previous study in the murine model of GLD (twitc...

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