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货号： bs-11761R-Bio 基本售价： 2980.0 元 规格： 100ul
产品信息
- 产品编号
- bs-11761R-Bio
- 英文名称
- Anti-SPG7/Biotin
- 中文名称
- 生物素标记的痉挛性截瘫蛋白7/基质细胞粘附调节蛋白抗体
- 别 名
- CAR; Paraplegin; 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.
- 规格价格
- 100ul/2980元购买 大包装/询价
- 说 明 书
- 100ul
- 研究领域
- 心血管 细胞生物 神经生物学 细胞粘附分子
- 抗体来源
- Rabbit
- 克隆类型
- Polyclonal
- 交叉反应
- Human, Mouse, Rat,
- 产品应用
- WB=1:50-200 ELISA=1:100-1000 IHC-P=1:50-200 IHC-F=1:50-200 ICC=1:50-200 IF=1:50-200
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.
- 分 子 量
- 88kDa
- 性 状
- Lyophilized or Liquid
- 浓 度
- 1mg/ml
- 免 疫 原
- KLH conjugated synthetic peptide derived from human Paraplegin
- 亚 型
- IgG
- 纯化方法
- affinity purified by Protein A
- 储 存 液
- 0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol.
- 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.
- 产品介绍
- background:
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.
Tissue Specificity:
Ubiquitous.
DISEASE:
Defects in SPG7 are the cause of spastic paraplegiaautosomal recessive type 7 (SPG7) [MIM:607259]. Spastic paraplegiais a degenerative spinal cord disorder characterized by a slow,gradual, progressive weakness and spasticity of the lower limbs.SPG7 is a complex form. Additional clinical features are cerebellarsyndrome, supranuclear palsy, and cognitive impairment,particularly disturbance of attention and executive functions.
Note=Defects in SPG7 may cause autosomal recessiveosteogenesis imperfecta (OI). Osteogenesis imperfecta defines agroup of connective tissue disorders characterized by bonefragility and low bone mass. Clinical features of SPG7-relatedosteogenesis imperfecta include recurrent fractures, mild bonedeformities, delayed tooth eruption, normal hearing and whitesclera.
Similarity:
In the N-terminal section; belongs to the AAA ATPasefamily.
In the C-terminal section; belongs to the peptidaseM41 family.
Database links:Entrez Gene: 6687Human
Entrez Gene: 234847Mouse
Omim: 602783Human
SwissProt: Q9UQ90Human
SwissProt: Q3ULF4Mouse
Unigene: 185597Human
Important Note:
This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.