验证数据展示
产品信息
67092-2-PBS targets ASAH1 in WB, IF/ICC, Indirect ELISA applications and shows reactivity with human, rat, pig, rabbit samples.
经测试应用 | WB, IF/ICC, Indirect ELISA Application Description |
经测试反应性 | human, rat, pig, rabbit |
免疫原 | ASAH1 fusion protein Ag28645 种属同源性预测 |
宿主/亚型 | Mouse / IgG1 |
抗体类别 | Monoclonal |
产品类型 | Antibody |
全称 | N-acylsphingosine amidohydrolase (acid ceramidase) 1 |
别名 | ASAH, Acid ceramidase subunit beta, Acid ceramidase subunit alpha, Acid ceramidase, AC |
计算分子量 | 44 kDa |
观测分子量 | 44-55 kDa |
GenBank蛋白编号 | BC016828 |
基因名称 | ASAH1 |
Gene ID (NCBI) | 427 |
偶联类型 | Unconjugated |
形式 | Liquid |
纯化方式 | Protein G purification |
UNIPROT ID | Q13510 |
储存缓冲液 | PBS only , pH 7.3 |
储存条件 | Store at -80°C. The product is shipped with ice packs. Upon receipt, store it immediately at -80°C |
背景介绍
ASAH1, also named as AC, ACDase, Acid CDase, PHP32 and ASAH, belongs to the acid ceramidase family. ASAH1 is a lipid hydrolase that catalyzes the conversion of ceramide (cer) into sphingosine (SPH) and a free fatty acid. Mutation of ASAH1 will cause the Farber lipogranulomatosis (FL) which also known as Farber disease (FD). ASAH1 is highly expressed in Heart. And it is first synthesized as an 55-60 kDa precursor and subsequently processed to the mature, heterodimeric enzyme (40 + 13 kDa) in the endosomes/lysosomes.