英文名称 Anti-SPG7/Paraplegin
中文名称 痉挛性截瘫蛋白7/基质细胞粘附调节蛋白抗体
痉挛性截瘫蛋白7/基质细胞粘附调节蛋白抗体别 名 CAR; Cell adhesion regulator; Cell matrix adhesion regulator; CMAR; Paraplegin; PGN; Spastic paraplegia 7 (pure and complicated autosomal recessive); Spastic paraplegia 7 homolog (human); Spastic paraplegia 7, paraplegin (pure and complicated autosomal recessive); Spastic paraplegia protein 7; SPG5C; SPG7 gene; SPG7_HUMAN.
浓 度 1mg/1ml
规 格 0.2ml/200μg
抗体来源 Rabbit
克隆类型 polyclonal
交叉反应 Human, Mouse, Rat
产品类型 一抗
公司全程供应Abcam、CST、Abnova、BD、GeneTex、Proteintech、SB、Abgent、AbFrontier、AnaSpec、Agrisera、Alomone、Epitomics、Active Motif、Assaybiotech、Biorbyt、BioVision、Bender、CaymanChemical、Covance、ChromoTek、Enzo、eBioscience、Glen Research、MBL、ProSci、PeproTech、Swant、Vector等品牌公司抗体,产品货期短、痉挛性截瘫蛋白7/基质细胞粘附调节蛋白抗体品质保证、全国免邮,并提供抗体相应实验技术指导。
研究领域 心血管 细胞生物 神经生物学 细胞粘附分子
蛋白分子量 predicted molecular weight: 88kDa
性 状 Lyophilized or Liquid
免 疫 原 KLH conjugated synthetic peptide derived from human Paraplegin (201-285aa)
亚 型 IgG
纯化方法 affinity purified by Protein A
储 存 液 Preservative: 15mM Sodium Azide, Constituents: 1% BSA, 0.01M PBS, pH 7.4
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.
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.
Important Note This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.
痉挛性截瘫蛋白7/基质细胞粘附调节蛋白抗体产品介绍 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.
Function : SPG7 is a metalloprotease protein that is a member of the AAA (ATPases associated with a variety of cellular activities) protein family. Members of this protein family share an ATPase domain and have roles in diverse cellular processes including membrane trafficking, intracellular motility, organelle biogenesis, protein folding, and proteolysis. Two transcript variants encoding distinct isoforms have been identified for this gene. Mutations associated with this gene cause autosomal recessive spastic paraplegia 7.
Subunit : Interacts with AFG3L2; the interaction is required forthe efficient assembly of mitochondrial complex I.
Subcellular Location : Mitochondrion membrane; Multi pass membrane protein.
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企业名称
上海研生生化试剂有限公司
企业信息已认证
企业类型
信用代码
310112001038197
成立日期
2011-01-12
注册资本
100
经营范围
上海研生实业有限公司
公司地址
上海市漕宝路66号20F
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