轴周蛋白PRX抗体

英文名称    Periaxin    

中文名称    轴周蛋白PRX抗体    

别    名    PRX; CMT4F; KIAA1620; Periaxin; PRAX_HUMAN; Prx; L-Periaxin.    

轴周蛋白PRX抗体      

说 明 书    0.1ml  0.2ml      

研究领域    细胞生物      

抗体来源    Rabbit    

克隆类型    Polyclonal    

交叉反应    

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.    

分 子 量    161kDa    

细胞定位    细胞核 细胞浆 细胞膜     

性    状    Lyophilized or Liquid    

浓    度    1mg/1ml    

免 疫 原    KLH conjugated synthetic peptide derived from human Periaxin    

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    

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.    

轴周蛋白PRX抗体 产品介绍    background:
This gene encodes a protein involved in peripheral nerve myelin upkeep. The encoded protein contains 2 PDZ domains which were named after PSD95 (post synaptic density protein), DlgA (Drosophila disc large tumor suppressor), and ZO1 (a mammalian tight junction protein). Two alternatively spliced transcript variants have been described for this gene which encode different protein isoforms and which are targeted differently in the Schwann cell. Mutations in this gene cause Charcot-Marie-Tooth neuoropathy, type 4F and Dejerine-Sottas neuropathy. [provided by RefSeq, Jul 2008]

Function:
Seems to be required for maintenance of peripheral nerve myelin sheath. May have a role in axon-glial interactions, possibly by interacting with the cytoplasmic domains of integral membrane proteins such as myelin-associated glycoprotein in the periaxonal regions of the Schwann cell plasma membrane. May have a role in the early phases of myelin deposition.

Subunit:
Interacts with SCN10A. Found in a complex with SCN10A.

Subcellular Location:
Nucleus.Isoform 1: Cell membrane. Isoform 2: Cytoplasm.

Tissue Specificity:
Isoform 1 and isoform 2 are found in sciatic nerve and Schwann cells.

DISEASE:
Defects in PRX are a cause of Dejerine-Sottas syndrome (DSS) [MIM:145900]; also known as Dejerine-Sottas neuropathy (DSN) or hereditary motor and sensory neuropathy III (HMSN3). DSS is a severe degenerating neuropathy of the demyelinating Charcot-Marie-Tooth disease category, with onset by age 2 years. DSS is characterized by motor and sensory neuropathy with very slow nerve conduction velocities, increased cerebrospinal fluid protein concentrations, hypertrophic nerve changes, delayed age of walking as well as areflexia. There are both autosomal dominant and autosomal recessive forms of Dejerine-Sottas syndrome.

Similarity:
Belongs to the periaxin family. Contains 1 PDZ (DHR) domain.

Gene ID:
19153

Database links:
Entrez Gene: 57716 Human

Entrez Gene: 19153 Mouse

Entrez Gene: 78960 Rat

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

轴周蛋白PRX抗体 

hz-0336R-AP Rabbit Anti-Monkey IgM/AP  碱性磷酸酶（AP）标记的兔抗猴IgM

hz-0336R-Gold Rabbit Anti-Monkey IgM/Gold  胶体金标记的兔抗猴IgM

hz-0336R-HRP Rabbit Anti-Monkey IgM/HRP  辣根过氧化物酶标记的兔抗猴IgM

hz-0336R-PE-hzy3 Rabbit Anti-Monkey IgM/PE-Cy3  PE-Cy3标记的兔抗猴IgM

hz-0336R-PE-hzY5 Rabbit Anti-Monkey IgM/PE-CY5  PE-CY5标记的兔抗猴IgM

hz-0336R-FIThz Rabbit Anti-Monkey IgM/FITC  FITC标记的兔抗猴IgM

hz-0336R-RBIThz Rabbit Anti-Monkey IgM/RBITC  罗丹明标记的兔抗猴IgM

hz-0296R-RBIThz Rabbit Anti-mouse IgG/RBITC  罗丹明标记的兔抗小鼠IgG

hz-0296R-FIThz Rabbit Anti-mouse IgG/FITC  FITC标记的兔抗小鼠IgG

hz-0296R-Gold Rabbit Anti-mouse IgG/Gold  胶体金标记的兔抗小鼠IgG

hz-0296R-HRP Rabbit Anti-mouse IgG/HRP  辣根过氧化物酶标记的兔抗小鼠IgG

hz-0296R-AP Rabbit Anti-mouse IgG/AP  碱性磷酸酶（AP）标记的兔抗小鼠IgG

hz-0296R-Bio Rabbit Anti-mouse IgG/Bio  生物素标记的兔抗小鼠IgG

hz-0296R-hzy3 Rabbit Anti-mouse IgG/Cy3  Cy3标记的兔抗小鼠IgG

hz-0296R-hzy5.5 Rabbit Anti-mouse IgG/Cy5.5  Cy5.5标记的兔抗小鼠IgG

hz-0296R-hzy7 Rabbit Anti-mouse IgG/Cy7  Cy7标记的兔抗小鼠IgG

hz-0296R-PE Rabbit Anti-mouse IgG/PE  PE标记的兔抗小鼠IgG

hz-0296R-PE-hzy3 Rabbit Anti-mouse IgG/PE-Cy3  PE-Cy3标记的兔抗小鼠IgG

hz-0296R-PE-hzY5 Rabbit Anti-mouse IgG/PE-CY5  PE-CY5标记的兔抗小鼠IgG

hz-0296R-APhz Rabbit Anti-mouse IgG/APC  APC标记的兔抗小鼠IgG

hz-0296R-AF350 Rabbit Anti-mouse IgG/Alexa Fluor 350  Alexa Fluor 350标记的兔抗小鼠IgG

hz-0296R-AF488 Rabbit Anti-mouse IgG/Alexa Fluor 488  Alexa Fluor 488标记的兔抗小鼠IgG

hz-0296R-AF555 Rabbit Anti-mouse IgG/Alexa Fluor 555  Alexa Fluor 555标记的兔抗小鼠IgG

hz-0296R-AF647 Rabbit Anti-mouse IgG/Alexa Fluor 647  Alexa Fluor 647标记的兔抗小鼠IgG

hz-0296R-PE-hzy5.5 Rabbit Anti-mouse IgG/PE-Cy5.5  PE-Cy5.5标记的兔抗小鼠IgG

hz-0296R-PE-hzy7 Rabbit Anti-mouse IgG/PE-Cy7  PE-Cy7标记的兔抗小鼠IgG

hz-0368R-Bio Rabbit Anti-mouse IgM/Bio  生物素标记的兔抗小鼠IgM

hz-0368R-HRP Rabbit Anti-mouse IgM/HRP  辣根过氧化物酶标记的兔抗小鼠IgM

hz-0368R-Gold Rabbit Anti-mouse IgM/Gold  胶体金标记的兔抗小鼠IgM

hz-0368R-AP Rabbit Anti-mouse IgM/AP  碱性磷酸酶（AP）标记的兔抗小鼠IgM

hz-0368R-hzy5 Rabbit Anti-mouse IgM/Cy5  Cy5标记的兔抗小鼠IgM

hz-0368R-hzy5.5 Rabbit Anti-mouse IgM/Cy5.5  Cy5.5标记的兔抗小鼠IgM

hz-0368R-hzy7 Rabbit Anti-mouse IgM/Cy7  Cy7标记的兔抗小鼠IgM

hz-0368R-PE Rabbit Anti-mouse IgM/PE  PE标记的兔抗小鼠IgM

hz-0368R-PE-hzy3 Rabbit Anti-mouse IgM/PE-Cy3  PE-Cy3标记的兔抗小鼠IgM

hz-0368R-PE-hzY5 Rabbit Anti-mouse IgM/PE-CY5  PE-CY5标记的兔抗小鼠IgM

hz-0368R-APhz Rabbit Anti-mouse IgM/APC  APC标记的兔抗小鼠IgM

hz-0368R-AF555 Rabbit Anti-mouse IgM/Alexa Fluor 555  Alexa Fluor 555标记的兔抗小鼠IgM

hz-0368R-AF647 Rabbit Anti-mouse IgM/Alexa Fluor 647  Alexa Fluor 647标记的兔抗小鼠IgM

hz-0368R-PE-hzy5.5 Rabbit Anti-mouse IgM/PE-Cy5.5  PE-Cy5.5标记的兔抗小鼠IgM

hz-0368R-PE-hzy7 Rabbit Anti-mouse IgM/PE-Cy7  PE-Cy7标记的兔抗小鼠IgM

hz-0368R-AF350 Rabbit Anti-mouse IgM/Alexa Fluor 350  Alexa Fluor 350标记的兔抗小鼠IgM

hz-0368R-AF488 Rabbit Anti-mouse IgM/Alexa Fluor 488  Alexa Fluor 488标记的兔抗小鼠IgM

hz-0368R-hzy3 Rabbit Anti-mouse IgM/Cy3  Cy3标记的兔抗小鼠IgM

hz-0368R-RBIThz Rabbit Anti-mouse IgM/RBITC  罗丹明标记的兔抗小鼠IgM