商品属性：
产品名称
半乳糖神经酰胺酶抗体
货号
BJ008387
英文名称
Galactosylceramidase
浓 度
1mg/ml
研究领域
细胞生物 神经生物学 信号转导 新陈代谢
细胞定位
细胞浆
抗体来源
Rabbit
性 状
Liquid
克隆类型
Polyclonal
亚 型
IgG
理论分子量
73kDa
用途
仅供科研研究实验
商品介绍：
别 名 Gacy; Galactocerebrosidase; Galactocerebroside beta galactosidase; Galactosylceramide beta galactosidase; galactosylceraminidase; Galc; GALCERase; Twitcher; GALC_HUMAN.
交叉反应； Human, Mouse, Rat, (predicted: Chicken, Dog, Pig, Cow, Horse, Rabbit, Sheep, )
产品应用；ELISA=1:5000-10000 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.
免 疫 原；KLH conjugated synthetic peptide derived from human G protein-regulated inducer of neurite outgrowth 2: 81-180/685
亚 型；IgG
纯化方法；affinity purified by Protein A
缓 冲 液；0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol.
注意事项；This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.
半乳糖神经酰胺酶抗体产品介绍：
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 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 “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.
Galactosylceramidase hydrolyzes the galactose ester bonds of galactosylceramide, galactosylsphingosine, lactosylceramide, and monogalactosyldiglyceride. It is an enzyme with very low activity responsible for the lysosomal catabolism of galactosylceramide, a major lipid in myelin, kidney and epithelial cells of small intestine and colon. It shows highest level of activity in testes compared to brain, kidney, placenta and liver. It can also be found in urine. Defects in Galactosylceramidase are the cause of globoid cell leukodystrophy (GLD); also known as Krabbe disease. This autosomal recessive disorder results in the insufficient catabolism of several galactolipids that are important in the production of normal myelin. Clinically, the most frequent form is the infantile form. Most patients (90%) present before six months of age with irritability, spasticity, arrest of motor and mental development, and bouts of temperature elevation without infection. This is followed by myoclonic jerks of arms and legs, oposthotonus, hypertonic fits, and mental regression, which progresses to a severe decerebrate condition with no voluntary movements and death from respiratory infections or cerebral hyperpyrexia before 2 years of age. However, a significant number of cases with later onset, presenting with unexplained blindness, weakness and/or progressive motor, and sensory neuropathy that can progress to severe mental incapacity and death, have been identified.
一抗是一种球蛋白，能特异性结合特定蛋白或重点研究的其他生物分子，目的是对其进行纯化、检测和定量。使用小鼠、大鼠、兔子、山羊和其他动物作为宿主，可获得多克隆或单克隆抗体。
一抗的生产和类型有多种形式，从抗血清粗品到抗原纯化制剂不等。
我们常说的抗体指的是第抗体。市售的一抗有多种不同的生产方式，这些不同导致抗体各具特性，适用于不同的应用。了解每种抗体的特性对选择合适的抗体至关重要。
单克隆抗体和多克隆抗体都是我们实验中常用的抗体产品。他们在识别的抗原表位、生产和应用等方面有一些区别。
✓多克隆抗体（polyclonal antibody, pAb）：用一种包含多种抗原决定簇的抗原动物，可刺激机体多个 B 细胞克隆产生针对多种抗原表位的抗体。
✓单克隆抗体（monoclonal antibodies, mAb）：由单一 B 细胞克隆产生的识别一种抗原表位的抗体。
双特异性抗体实验流程 ：
1. 目标选择：
确定目标：确定需要靶向的相关蛋白或细胞表面受体。
确定机制：确定的作用机制，例如靶向细胞表面受体促进细胞凋亡，或者阻断病原体的进入等。
2. 抗体设计：
设计抗体结构：设计能够同时结合两个不同抗原的抗体结构。
确定结构特征：确定双特异性抗体的结构特征，例如连接方式、抗原结合位点等。
3. 抗体工程：
克隆基因：通过分子生物学技术克隆出编码双特异性抗体的基因序列。
重组表达：将基因插入适当的表达系统（通常是哺乳动物细胞或大肠杆菌）进行表达和生产。
4. 抗体表征：
亲和度分析：使用生物物理学技术（如表面等离子共振、生物层析等）评估抗体与抗原的亲和度。
功能性分析：评估抗体的生物学活性，例如在细胞模型中测试其对靶标的影响。
5. 生产和纯化：
大规模生产：将优选的双特异性抗体基因转染至合适的细胞系，进行大规模生产。
纯化：使用柱层析、亲和层析等技术对抗体进行纯化。
6. 功能验证：
体外实验：在细胞系或体外模型中评估双特异性抗体的活性和毒性。
动物模型：在动物模型中评估双特异性抗体的药效学和性。
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