酸性神经酰胺酶1抗体
产品名称: 酸性神经酰胺酶1抗体
英文名称: ASAH1
产品编号: bs-12976R
产品价格: null
产品产地: 北京
品牌商标: BIOSS
更新时间: 2025-01-08T10:30:41
使用范围: WB=1:100-500,ELISA=1:500-1000,IHC-P=1:100-500,IHC-F=1:100-500,ICC=1:100-500,IF=1:100-500,
北京索莱宝科技有限公司
- 联系人 : 索莱宝-龚思雨
- 地址 : 北京市通州区中关村科技园区通州园金桥科技产业基地景盛南四街15号85A三层
- 邮编 : 101102
- 所在区域 : 北京
- 电话 : 178****1073 点击查看
- 传真 : 点击查看
- 邮箱 : 3193328036@qq.com
- 二维码 : 点击查看
Acid ceramidase catalyzes the degradation of ceramide in normal tissues, and deficiency leads to accumulation of ceramide in tissues, a hallmark of Farber disease. Effected individuals experience early onset joint problems and neurological problems, owing to mutations in the acid ceramidase gene. Bioinformatic analysis of gene expression also reveals acid ceramidase to be among the 5 most important genes associated with melanoma. In addition to ceramide hydrolysis, purified acid ceramidase also exhibits the ability to catalyze ceramide synthesis, utilizing [14C]lauric acid and sphingosine as substrates. Interestingly, pH regulates which reaction is favored; for hydrolysis the pH optimum is 4.5, whereas for the reverse reaction favors a pH of 5.5, further supporting a complex and central role for acid ceramidase in sphingolipid metabolism.