storage temp.
−20°C
SMILES string
CCCCCCCCCCCCC\C=C\C(O)C(N)COC1OC(CO)C(O)C(O)C1O
Preparation Note
Prepared from glucocerebrosides from human (Gaucher′s) spleen
存储类别
13 - Non Combustible Solids
wgk
WGK 3
flash_point_f
Not applicable
flash_point_c
Not applicable
法规信息
新产品
此项目有
N Tayebi et al.
Molecular genetics and metabolism, 79(2), 104-109 (2003-06-18)
Among the phenotypes associated with Gaucher disease, the deficiency of glucocerebrosidase, are rare patients with early onset, treatment-refractory parkinsonism. Sequencing of glucocerebrosidase in 17 such patients revealed 12 different genotypes. Fourteen patients had the common "non-neuronopathic" N370S mutation, including five
Genji Imokawa
Journal of dermatological science, 55(1), 1-9 (2009-05-16)
A deficiency of ordinary ceramides in the stratum corneum is an essential etiologic factor for the dry and barrier-disrupted skin of patients with atopic dermatitis (AD). We have proposed that the mechanism underlying that deficiency involves a novel sphingolipid metabolizing
U H Schueler et al.
Neurobiology of disease, 14(3), 595-601 (2003-12-18)
Patients with Gaucher disease have been classified as type 1 nonneuronopathic, type 2 acute neuronopathic, and type 3 chronic neuronopathic phenotypes. Increased quantities of glucocerebroside and glucosylsphingosine (glucopsychosine) are present in the brain of type 2 and type 3 Gaucher
Joseph K Park et al.
Pediatric research, 53(3), 387-395 (2003-02-22)
Gaucher disease, the inherited deficiency of lysosomal glucocerebrosidase, presents with a wide spectrum of manifestations. Although Gaucher disease has been divided into three clinical types, patients with atypical presentations continue to be recognized. A careful phenotypic and genotypic assessment of
Emyr Lloyd-Evans et al.
The Biochemical journal, 375(Pt 3), 561-565 (2003-08-15)
Recently, we demonstrated that the GSL (glycosphingolipid), GlcCer (glucosylceramide), modulates Ca2+ release from intracellular stores and from microsomes by sensitizing the RyaR (ryanodine receptor), a major Ca2+-release channel of the endoplasmic reticulum, whereas the lyso derivative of GlcCer, namely GlcSph
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