Acid Ceramidase Pure 23 50ug

产品编号：BD-612302 品牌：BD-Pharmingen 原厂货号：612302
产品分类：抗体 > 一抗 > 蛋白特异性一抗
应用分类：

包装：

50ug

运保温度:

Store undiluted at -20°C.

到货周期：

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描述：

Ceramide is a sphingolipid that exhibits a wide variety of functions, including monocyte differentiation, apoptosis, neurite outgrowth, and

Ca2+ transport. It also serves as the precursor of many sphingolipids and anchors these into the outer leaflet of the plasma membrane via

hydrophobic interactions. Acid ceramidase is a lysosomal enzyme that was purified from human urine. It is synthesized as a 55kDa precursor

protein, which is then processesed into the mature α-subunit (13kDa) and β-subunit (40kDa). Acid ceramidase catalyzes the hydrolysis of

ceramide into free fatty acid and sphingosine. Sphingosine, and its phosphorylated form, sphingosine-1-phosphate (SPP), have been shown to

inhibit PKC activity and act as a second messenger in cell proliferation and differentiation. Acid ceramidase is also the cause of a lysosomal

storage disorder known as Farber’s disease. This disease is characterized by an accumulation of ceramide in tissues, leading to swelling and

pain of the joints and extremities, pulmonary insufficiency, and death at an early age. Thus, acid ceramidase is necessary in the hydrolysis of

ceramide and is the cause of Farber’s disease.

注意事项：

1.Since applications vary, each investigator should titrate the reagent to obtain optimal results.
2.Caution: Sodium azide yields highly toxic hydrazoic acid under acidic conditions. Dilute azide compounds in running water before discarding to avoid accumulation of potentially explosive deposits in plumbing.
3.Store undiluted at -20°C.