Rabbit Anti-Periaxin/FITC Conjugated antibody
|别 名||PRX; CMT4F; KIAA1620; Periaxin; PRAX_HUMAN; Prx; L-Periaxin.|
|规格价格||100ul/2980元 购买 大包装/询价|
|说 明 书||100ul|
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.
|分 子 量||161kDa|
|性 状||Lyophilized or Liquid|
|免 疫 原||KLH conjugated synthetic peptide derived from human PRX/periaxin|
|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.|
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]
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.
Interacts with SCN10A. Found in a complex with SCN10A.
Nucleus.Isoform 1: Cell membrane. Isoform 2: Cytoplasm.
Isoform 1 and isoform 2 are found in sciatic nerve and Schwann cells.
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.
Belongs to the periaxin family. Contains 1 PDZ (DHR) domain.
Entrez Gene: 57716 HumanEntrez Gene: 19153 Mouse
Entrez Gene: 78960 Rat
This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications
该基因编码一个参与周围神经髓鞘维持的蛋白质。编码的蛋白质包含2个PDZ结构域，其命名为PSD95（突触后密度蛋白）、DlgA（果蝇盘大肿瘤抑制子）和ZO1（哺乳动物紧密连接蛋白）。已经描述了两种交替剪接的转录变体，该基因编码不同的蛋白质亚型，并且在雪旺细胞中靶向不同。该基因的突变导致夏科玛丽牙神经病变，4F型和Dejerine Sottas神经病变。[ RefSeq，JUL 2008 ]