酸性神经酰胺酶1抗体
规格：1mg/1ml
英文名： ASAH1
别名： AC; ACDase; Acid CDase; Acid ceramidase; Acid ceramidase precursor; Acid ceramidase subunit beta; Acylsphingosine deacylase; ASAH 1; ASAH; ASAH1; ASAH1_HUMAN; FLJ21558; FLJ22079; N acylsphingosine ami
分子量： 29kDa
储存液：0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glyce
克隆类型：Polyclonal
亚型：IgG
纯化方法：affinity purified by Protein A
**原：KLH conjugated synthetic peptide derived from human Acid cer
交叉反应：Human, Mouse, Rat, Chicken, Dog, Pig, Cow, Horse, Sheep,
细胞定位：细胞浆
酸性神经酰胺酶1抗体产品介绍：background: 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 酸性神经酰胺酶1抗体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. Function: Hydrolyzes the sphingolipid ceramide into sphingosine and free fatty acid. Subunit: Heterodimer of one alpha and one beta subunit. Subcellular Location: Lysosome. Tissue Specificity: Broadly expressed with highest expression in heart. DISEASE: Defects in ASAH1 are the cause of Farber lipogranulomatosis (FL) [MIM:228000]; also known as Farber disease (FD). 酸性神经酰胺酶1抗体This sphingolipid disease is characterized by subcutaneous lipid-loaded nodules, excruciating pain in the joints and extremities, marked accumulation of ceramide in lysosomes, and death by three years of age. Similarity: Belongs to the acid ceramidase family. Database links: UniProtKB/Swiss-Prot: Q13510.5 Important Note: This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.
酸性神经酰胺酶1抗体产品应用：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 （石蜡切片需做抗原修复） not yet tested in other applications. optimal dilutions/concentrations should be determined by the end user.
研究领域：肿瘤 心血管 细胞生物 神经生物学 信号转导 新陈代谢
储存条件： Store at -20 °C for one year. Avoid repeated freeze/thaw cycles.
来源： Rabbit
外观： Lyophilized or Liquid