痉挛性截瘫蛋白7/基质细胞粘附调节蛋白抗体

产品名称：痉挛性截瘫蛋白7/基质细胞粘附调节蛋白抗体 
中文名称：痉挛性截瘫蛋白7/基质细胞粘附调节蛋白抗体 
英文名称：Anti-SPG7/Paraplegin 
产品编号：byk-11761R 
产品规格：0.1ml/0.2ml
产品用途：科研实验
亚 型： IgG
贮 存： 贮存于-20℃
抗体的异质性。抗体的组成极为复杂，是由成千上万、多种多样的免疫球蛋白（Ig）分子所组成。这些Ig分子在形状、大小、结构以及氨基酸的组成和排列上，既相似，又有差别。由于有差别，它们的电泳活性就有很大的变化。
痉挛性截瘫蛋白7/基质细胞粘附调节蛋白抗体 产品说明：
Paraplegin is a 795 amino acid metalloprotease that is a member of the AAA protein family. Localized to the mitochrondrial membrane and expressed throughout the body, Paraplegin is a multi-pass membrane protein that is thought to be involved in signal transduction and chaperone-like activities in the mitochrondria. Defects in the gene encoding Paraplegin are the cause of spastic paraplegia type 7 (SPG7), a form of autosomal recessive hereditary spastic paraplegia (AR-HSP). HSPs are degenerative spinal cord disorders that are characterized by muscle spasms, stiffness in the legs and, in some cases, incontinence. Recent studies suggest that SPG7 may be a mitochondrial-based disease, as mutations in the Paraplegin gene lead to ragged-red fibers, oxidase-negative fibers and intense succinate dehydrogenase-stained areas of the mitochrondria. These mitochondrial dysfunctions lead to axonal degeneration and impaired axonal transport, thus causing the neurodegeneration seen in HSPs.
保存条件：Store at -20 °C for one year. Avoid repeated freeze/thaw cycles. The lyophilized antibody is stable at room temperature for at least one month and for greater than a year when kept at -20°C. When reconstituted in sterile pH 7.4 0.01M PBS or diluent of antibody the antibody is stable for at least two weeks at 2-4 °C. 
痉挛性截瘫蛋白7/基质细胞粘附调节蛋白抗体 相关产品：
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Anti-Ki-67（Antigen identified by monoclonal antibody Ki 67）  Ki67抗体
Anti-Ki-67（mo monoclonal）  小鼠Ki-67单克隆抗体
Anti-Kif14 protein   驱动蛋白家族Member14蛋白抗体
Anti-Kiss-1 (Metastasis-suppressor KiSS-1 precursor)  肿瘤转移YZ基因抗体
Anti-Klf4(Kruppel like factor 4)  肠道内富含的Kruppel样因子/上皮锌指蛋白4抗体
抗体结构:
抗体是具有4条多肽链的对称结构，其中2条较长、相对分子量较大的相同的重链(H链)；2条较短、相对分子量较小的相同的轻链(L链)。链间由二硫键和非共价键联结形成一个由4条多肽链构成的单体分子。轻链有κ和λ两种，重链有μ、δ、γ、ε和α五种。 整个抗体分子可分为恒定区和可变区两部分。在给定的物种中，不同抗体分子的恒定区都具有相同的或几乎相同的氨基酸序列。可变区位于"Y"的两臂末端。在可变区内有一小部分氨基酸残基变化特别强烈，这些氨基酸的残基组成和排列顺序更易发生变异区域称高变区。高变区位于分子表面，多由17个氨基酸残基构成，少则只有2 ～ 3个。高变区氨基酸序列决定了该抗体结合抗原抗原的特异性。一个抗体分子上的两个抗原结合部位是相同的，位于两臂末端称抗原结合片段(antigen-binding fragment, Fab)。"Y"的柄部称结晶片段(crystalline fragment,FC)，糖结合在FC 上。