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BN41320R-100ul
100ul
￥2360.00
交叉反應：Human,Mouse(predicted:Rat) 推薦應用：WB,IHC-P,IHC-F,IF,ELISA
BN41320R-200ul
200ul
￥3490.00
交叉反應：Human,Mouse(predicted:Rat) 推薦應用：WB,IHC-P,IHC-F,IF,ELISA
產品描述
| 英文名稱 | EGR2 |
| 中文名稱 | 早期生長反應蛋白2抗體 |
| 別 名 | CMT1D; CMT4E; DKFZp686J1957; Early growth response 2; Early growth response protein 2; EGR-2; egr2; EGR2_HUMAN; FLJ14547; KROX 20 Drosophila homolog; Krox 20 homolog Drosophila; KROX20; Krox20 protein; Zinc finger protein Krox-20; AT591. |
| 研究領域 | 細胞生物 免疫學 神經生物學 信號轉導 表觀遺傳學 |
| 抗體來源 | Rabbit |
| 克隆類型 | Polyclonal |
| 交叉反應 | Human, Mouse, (predicted: Rat, ) |
| 產品應用 | WB=1:500-2000 ELISA=1:5000-10000 IHC-P=1:100-500 IHC-F=1:100-500 IF=1:50-200 （石蠟切片需做抗原修復） not yet tested in other applications. optimal dilutions/concentrations should be determined by the end user. |
| 分 子 量 | 50kDa |
| 細胞定位 | 細胞核 |
| 性 狀 | Liquid |
| 濃 度 | 1mg/ml |
| 免 疫 原 | KLH conjugated synthetic peptide derived from human EGR2:351-450/476 |
| 亞 型 | IgG |
| 純化方法 | affinity purified by Protein A |
| 儲 存 液 | 0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol. |
| 保存條件 | Shipped at 4℃. Store at -20 °C for one year. Avoid repeated freeze/thaw cycles. |
| PubMed | PubMed |
| 產品介紹 | Egr proteins function in transcription regulatory activities surrounding cellular growth, differentiation and function. The deduced amino acid sequences of human Egr-2 and mouse Egr-1 are 92% identical in the zinc finger region but show no homology elsewhere. Egr-2 is a sequence-specific DNA-binding transcription factor that binds two specific DNA sites located in the promoter region of HoxA4 and localizes to the nucleus. Defects in the Egr-2 protein are a cause of congenital hypomyelination neuropathy (CHN). CHN is characterized clinically by early onset of hypotonia, areflexia, distal muscle weakness and very slow nerve conduction velocities. Mutations in the gene that encodes Egr-2 (EGR2) also cause Dejerine-Sottas syndrome (DSS), which is also known as Dejerine-Sottas neuropathy (DSN) or hereditary motor and sensory neuropathy III (HMSN3). DSS patients exhibit severe early onset motor and sensory neuropathy with very slow nerve conduction velocities and elevated cerebrospinal fluid protein concentrations. Function: Sequence-specific DNA-binding transcription factor. Binds to two specific DNA sites located in the promoter region of HOXA4. E3 SUMO-protein ligase helping SUMO1 conjugation to its coregulators NAB1 and NAB2, whose sumoylation down-regulates EGR2 own transcriptional activity. Subunit: Interacts with HCFC1. Interacts with WWP2. Interacts with UBC9. Subcellular Location: Nucleus. Post-translational modifications: Ubiquitinated by WWP2 leading to proteasomal degradation (By similarity). DISEASE: Defects in EGR2 are a cause of congenital hypomyelination neuropathy (CHN) [MIM:605253]. Inheritance can be autosomal dominant or recessive. Recessive CHN is also known as Charcot-Marie-Tooth disease type 4E (CMT4E). CHN is characterized clinically by early onset of hypotonia, areflexia, distal muscle weakness, and very slow nerve conduction velocities. Defects in EGR2 are a cause of Charcot-Marie-Tooth disease type 1D (CMT1D) [MIM:607678]. CMT1D is a form of Charcot-Marie-Tooth disease, the most common inherited disorder of the peripheral nervous system. Charcot-Marie-Tooth disease is classified in two main groups on the basis of electrophysiologic properties and histopathology: primary peripheral demyelinating neuropathy or CMT1, and primary peripheral axonal neuropathy or CMT2. Neuropathies of the CMT1 group are characterized by severely reduced nerve conduction velocities (less than 38 m/sec), segmental demyelination and remyelination with onion bulb formations on nerve biopsy, slowly progressive distal muscle atrophy and weakness, absent deep tendon reflexes, and hollow feet. Defects in EGR2 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 EGR C2H2-type zinc-finger protein family. Contains 3 C2H2-type zinc fingers. SWISS: P11161 Gene ID: 1959 Database links: Entrez Gene: 1959 Human Entrez Gene: 13654 Mouse GenBank: BC035625 Human Omim: 129010 Human SwissProt: P11161 Human SwissProt: P08152 Mouse Unigene: 1395 Human Unigene: 290421 Mouse Unigene: 89235 Rat Important Note: This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications. |
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