Quantity size:
0.2ml
Concentration:
1mg/ml 半乳糖神经酰胺酶抗体 Buffer = 0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol.
Background:
GALC is a lysosomal enzyme that hydrolyzes galactose ester bonds in various galactolipids, including galactosylceramide, galactosylsphingosine, lactosylceramide and monogalactosyldiglyceride. Galactolipids contain glucose and/or galactose, and are found in the brain and other nerve tissue, especially the myelin sheath. Galactosylceramide 半乳糖神经酰胺酶抗体is a major lipid in myelin, kidney, and epithelial cells of the small intestine and colon. Mutations in the GALC gene that compromise protein function correlate to Krabbe disease (globoid cell leukodystrophy, GLD). GLD is an autosomal recessive condition that affects approximately 1 in 150,000 infants and results in progressive destruction of the nervous system. The 半乳糖神经酰胺酶抗体“twitcher” mouse is a model system for GLD; the genotype is a premature stop codon (W339X) in the galactosylceramidase (GALC) gene that abolishes enzymatic activity. Alternate transcriptional splice variants, encoding different isoforms, have been characterized.
Also known as:
半乳糖神经酰胺酶抗体Gacy; Galactocerebrosidase; Galactocerebroside beta galactosidase; Galactosylceramide beta galactosidase; galactosylceraminidase; Galc; GALCERase; Twitcher; GALC_HUMAN.
Specificity:
●
Rabbit Polyclonal IgG, affinity purified by Protein A.
●
Reacts with: Human, Mouse, Rat, Chicken, Dog, Pig, Cow, Horse, Rabbit, Sheep, .
●
Immunogen: KLH conjugated synthetic peptide derived from human G protei.
●
半乳糖神经酰胺酶抗体Predicted Molecular Weight: 73kDa.
Storage:
Shipped at 4℃, Store at -20℃ (Avoid repeated freeze/thaw cycles).
Application:
ELISA=1:500-1000 IHC-P=1:100-500 IHC-F=1:100-500 ICC=1:100-500 IF=1:100-500
Not yet tested in other applications.
Optimal working dilutions must be determined by the end user.
kl426Hu01细胞球蛋白(CYGB)多克隆抗体Polyclonal Antibody to Cytoglobin (CYGB)
kl426Ra01细胞球蛋白(CYGB)多克隆抗体Polyclonal Antibody to Cytoglobin (CYGB)
kl429Hu01死亡关联蛋白激酶1(DAPK1)多克隆抗体Polyclonal Antibody to Death Associated Protein Kinase 1 (DAPK1)
kl429Mu01死亡关联蛋白激酶1(DAPK1)多克隆抗体Polyclonal Antibody to Death Associated Protein Kinase 1 (DAPK1)
kl431Hu01丝织蛋白1(DBN1)多克隆抗体Polyclonal Antibody to Drebrin 1 (DBN1)
kl432Hu01皮肤桥蛋白(DPT)多克隆抗体Polyclonal Antibody to Dermatopontin (DPT)
kl432Mu01皮肤桥蛋白(DPT)多克隆抗体Polyclonal Antibody to Dermatopontin (DPT)
kl436Hu01牙本质涎磷蛋白(DSPP)多克隆抗体Polyclonal Antibody to Dentin Sialophosphoprotein (DSPP)
kl438Hu01迪格弗梅尔基奥尔克劳森综合征蛋白(DYM)多克隆抗体Polyclonal Antibody to Dymeclin (DYM)
kl440Hu01脱氧胞苷激酶(DCK)多克隆抗体Polyclonal Antibody to Deoxycytidine Kinase (DCK)
kl442Hu01双肾上腺皮质激素(DCX)多克隆抗体Polyclonal Antibody to Doublecortin (DCX)
kl442Mu01双肾上腺皮质激素(DCX)多克隆抗体Polyclonal Antibody to Doublecortin (DCX)
kl442Ra01双肾上腺皮质激素(DCX)多克隆抗体Polyclonal Antibody to Doublecortin (DCX)
kl445Hu01奇异不良素(DYSF)多克隆抗体Polyclonal Antibody to Dysferlin (DYSF)
kl448Hu01肌营养不良蛋白关联糖蛋白1(DAG1)多克隆抗体Polyclonal Antibody to Dystrophin Associated Glycoprotein 1 (DAG1)
kl448Mu01肌营养不良蛋白关联糖蛋白1(DAG1)多克隆抗体Polyclonal Antibody to Dystrophin Associated Glycoprotein 1 (DAG1)
kl450Hu01细胞外基质蛋白1(ECM1)多克隆抗体Polyclonal Antibody to Extracellular Matrix Protein 1 (ECM1)
kl450Mu01细胞外基质蛋白1(ECM1)多克隆抗体Polyclonal Antibody to Extracellular Matrix Protein 1 (ECM1)
kl459Mu01内磺肽α(ENSα)多克隆抗体Polyclonal Antibody to Endosulfine Alpha (ENSa)
kl463Hu01内皮细胞特异分子1(ESM1)多克隆抗体Polyclonal Antibody to Endothelial Cell Specific Molecule 1 (ESM1)
kl466Hu01巢蛋白(NID)多克隆抗体Polyclonal Antibody to Nidogen (NID)
kl468Hu01脂肪酸去饱和酶1(FADS1)多克隆抗体Polyclonal Antibody to Fatty Acid Desaturase 1 (FADS1)
kl468Ra01脂肪酸去饱和酶1(FADS1)多克隆抗体Polyclonal Antibody to Fatty Acid Desaturase 1 (FADS1)
kl469Hu01成纤维细胞激活蛋白α(FAPα)多克隆抗体Polyclonal Antibody to Fibroblast Activation Protein Alpha (FAPa)
更多
企业名称
上海康朗生物科技有限公司
企业信息已认证
企业类型
信用代码
310112001510759
成立日期
2015-05-29
注册资本
100
经营范围
从事生物科技技术领域内技术开发、技术咨询、技术服务、技术转让,实验分析仪器、化工产品及原料(除危险化学品、监控化学品、烟花爆竹、民用爆竹物品、易制毒化学品)、仪器仪表、环保设备、酒店用品、皮革制品、床上用品、电子产品的销售。【依法须经批准的项目,经相关部门批准后方可开展经营活动】
上海康朗生物科技有限公司
公司地址
上海市闵行区闵北路88弄1-30号第22栋BV145室
客服电话