| 產(chǎn)品編號 |
bs-8368R-BF594 |
| 英文名稱 |
Rabbit Anti-EGR2/BF594 Conjugated antibody
|
| 中文名稱 |
BF594標記的早期生長反應蛋白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. |
| 規(guī)格價格 |
100ul/2980元
購買 大包裝/詢價 |
| 說 明 書 |
100ul
|
| 研究領域 |
細胞生物 免疫學 神經(jīng)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 |
|
| 產(chǎn)品應用 |
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 |
50kDa |
| 性 狀 |
Lyophilized or Liquid |
| 濃 度 |
1mg/ml |
| 免 疫 原 |
KLH conjugated synthetic peptide derived from human EGR2 |
| 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 |
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. |
| 產(chǎn)品介紹 |
background:
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.
Database links:
UniProtKB/Swiss-Prot: P11161.3
Important Note:
This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.
|
|
|
