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标记类型
HRPBiotinGoldRBITCAPFITCCy3Cy5Cy5.5Cy7PEPE-Cy3PE-Cy5PE-Cy5.5PE-Cy7APCAlexa Fluor 350Alexa Fluor 488Alexa Fluor 555Alexa Fluor 594Alexa Fluor 647

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HRPBiotinGoldRBITCAPFITCCy3Cy5Cy5.5Cy7PEPE-Cy3PE-Cy5PE-Cy5.5PE-Cy7APCAlexa Fluor 350Alexa Fluor 488Alexa Fluor 555Alexa Fluor 594Alexa Fluor 647

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Anti-SPG3A/Biotin

货号: bs-11759R-Bio 基本售价: 2980.0 元 规格: 100ul

产品信息

产品编号
bs-11759R-Bio
英文名称
Anti-SPG3A/Biotin
中文名称
生物素标记的G蛋白结合蛋白3抗体
别    名
Atlastin; AD FSP; atl1; ATLA1_HUMAN; Atlastin GTPase 1; Atlastin-1; Atlastin1; Brain specific GTP binding protein; Brain-specific GTP-binding protein; FSP1; GBP-3; GBP3; GTP-binding protein 3; Guanine nucleotide-binding protein 3; Guanylate binding protein 3; hGBP3; HSN1D; Spastic paraplegia 3 protein A; SPG 3A; SPG3; SPG3A.  
规格价格
100ul/2980元购买        大包装/询价
说 明 书
100ul  
研究领域
细胞生物  神经生物学  结合蛋白  G蛋白偶联受体  G蛋白信号  
抗体来源
Rabbit
克隆类型
Polyclonal
交叉反应
Human, Mouse, Rat, Rabbit, 
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
64kDa
性    状
Lyophilized or Liquid
浓    度
1mg/ml
免 疫 原
KLH conjugated synthetic peptide derived from human SPG3A/Atlastin (201-300aa)
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:
Atlastins are Golgi-localized, integral membrane proteins that function as GTPases. The Atlastin proteins, also designated SPG3A and guanylate-binding protein 3, comprise a Dynamin superfamily that plays a role in axonal maintenance. Hereditary spastic paraplegia (HSP) is an inherited neurodegenerative disorder that is characterized by retrograde axonal degeneration. HSP primarily affects long corticospinal neurons and causes spastic lower extremity weakness. Spastin, a microtubule (MT)-severing AAA ATPase, is a binding partner of Atlastin that is involved in membrane dynamics. This Spastin/Atlastin binding may be involved in the biochemical pathway that leads to HSP development. Mutations in the Atlastin gene (SPG3A) account for approximately 10% of all autosomal dominant HSPs, while mutations in the Spastin gene (SPG4) account for almost 40%.

Function:
GTPase tethering membranes through formation of trans-homooligomer and mediating homotypic fusion of endoplasmic reticulum membranes. Functions in endoplasmic reticulum tubular network biogenesis. May also regulate Golgi biogenesis. May regulate axonal development.

Subunit:
Homooligomer. Interacts (via N-terminal region) withMAP4K4 (via CNH regulatory domain). Interacts with REEP5, RTN3 andRTN4 (via the transmembrane region). Interacts with SPAST;interaction is direct. May interact with TMED2. Interacts withREEP1.

Subcellular Location:
Endoplasmic reticulum membrane. Golgi apparatus membrane. Cell projection >axon.

Tissue Specificity:
Expressed predominantly in the adult and fetal central nervous system. Measurable expression in all tissues examined, although expression in adult brain is at least 50-fold higher than in other tissues. Detected predominantly in pyramidal neurons in the cerebral cortex and the hippocampus of the brain. Expressed in upper and lower motor neurons (at protein level).

DISEASE:
Defects in ATL1 are the cause of spastic paraplegia autosomal dominant type 3 (SPG3) [MIM:182600]; also known as Strumpell-Lorrain syndrome. Spastic paraplegia is a degenerative spinal cord disorder characterized by a slow, gradual, progressive weakness and spasticity of the lower limbs.

Similarity:
Belongs to the GBP family. Atlastin subfamily.

Database links:

Entrez Gene: 51062Human

Entrez Gene: 73991Mouse

Entrez Gene: 362750Rat

Entrez Gene: 535424Cow

Omim: 606439Human

SwissProt: Q58D72Cow

SwissProt: Q8WXF7Human

SwissProt: Q8BH66Mouse

SwissProt: Q6PST4Rat

Unigene: 584905Human

Unigene: 474462Mouse

Unigene: 135117Rat



Important Note:
This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.