G1637
Glucopsychosine
别名:
1-β-D-Glucosylsphingosine
SMILES string
CCCCCCCCCCCCC\C=C\C(O)C(N)COC1OC(CO)C(O)C(O)C1O
storage temp.
−20°C
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
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
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
Mutsumi Ishibashi et al.
Laboratory investigation; a journal of technical methods and pathology, 83(3), 397-408 (2003-03-22)
To clarify mechanisms underlying acylceramide deficiency as an causative factor of the permeability barrier disruption seen in the skin of patients with atopic dermatitis (AD), we hypothesized and then demonstrated the presence of a novel epidermal enzyme, termed glucosylceramide (GC)
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