FITC标记的早老素蛋白1+2抗体
产品名称: FITC标记的早老素蛋白1+2抗体
英文名称: Anti-Presenilin 1 + 2/FITC
产品编号: HZ-11681R-FITC
产品价格: null
产品产地: 中国/上海
品牌商标: HZbscience
更新时间: 2023-08-17T10:24:20
使用范围: ICC=1:50-200 IF=1:50-200
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Rabbit Anti-Presenilin 1 + 2/FITC Conjugated antibody
FITC标记的早老素蛋白1+2抗体
产品编号 | bs-11681R-FITC |
英文名称 | Anti-Presenilin 1 + 2/FITC |
中文名称 | FITC标记的早老素蛋白1+2抗体 |
别 名 | AD 3; AD4; AD5; Alzheimer disease 3; Alzheimer disease 4; FAD; Presenilin 1; Presenilin 2; Presenilin-1; Presenilin-2; Presenilin1; Presenilin1; PS1; PS2; PS-1; PS-2; PSEN1; PSEN2; STM2; PSN1_HUMAN; PSN2_HUMAN. |
规格价格 | 100ul/2980元 购买 大包装/询价 |
说 明 书 | 100ul |
研究领域 | 肿瘤 神经生物学 信号转导 细胞凋亡 细胞自噬 Alzheimer's |
抗体来源 | Rabbit |
克隆类型 | Polyclonal |
交叉反应 | Human, Mouse, Rat, Chicken, |
产品应用 | 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 | 19/52kDa |
细胞定位 | 细胞膜 |
性 状 | Lyophilized or Liquid |
浓 度 | 1mg/ml |
免 疫 原 | KLH conjugated synthetic peptide derived from human Presenilin-1 NTF subunit |
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: Alzheimer's disease (AD) patients with an inherited form of the disease carry mutations in the presenilin proteins (PSEN1; PSEN2) or the amyloid precursor protein (APP). These disease-linked mutations result in increased production of the longer form of amyloid-beta (main component of amyloid deposits found in AD brains). Presenilins are postulated to regulate APP processing through their effects on gamma-secretase, an endoprotease complex that catalyzes the intramembrane cleavage of integral membrane proteins such as Notch receptors and APP. Function: Probable catalytic subunit of the gamma-secretase complex, an endoprotease complex that catalyzes the intramembrane cleavage of integral membrane proteins such as Notch receptors and APP (beta-amyloid precursor protein). Requires the other members of the gamma-secretase complex to have a protease activity. May play a role in intracellular signaling and gene expression or in linking chromatin to the nuclear membrane. Stimulates cell-cell adhesion though its association with the E-cadherin/catenin complex. Under conditions of apoptosis or calcium influx, cleaves E-cadherin promoting the disassembly of the E-cadherin/catenin complex and increasing the pool of cytoplasmic beta-catenin, thus negatively regulating Wnt signaling. May also play a role in hematopoiesis. Subunit: Homodimer. Component of the gamma-secretase complex, a complex composed of a presenilin homodimer (PSEN1 or PSEN2), nicastrin (NCSTN), APH1 (APH1A or APH1B) and PEN2. Such minimal complex is sufficient for secretase activity. Other components which are associated with the complex include SLC25A64, SLC5A7, PHB and PSEN1 isoform 3. Predominantly heterodimer of a N-terminal (NTF) and a C-terminal (CTF) endoproteolytical fragment. Associates with proteolytic processed C-terminal fragments C83 and C99 of the amyloid precursor protein (APP). Associates with NOTCH1. Associates with cadherin/catenin adhesion complexes through direct binding to CDH1 or CDH2. Interaction with CDH1 stabilizes the complex and stimulates cell-cell aggregation. Interaction with CDH2 is essential for trafficking of CDH2 from the endoplasmic reticulum to the plasma membrane. Interacts with CTNND2, CTNNB1, HERPUD1, FLNA, FLNB, MTCH1, PKP4 and PARL. Interacts through its N-terminus with isoform 3 of GFAP. Interacts with DOCK3. Subcellular Location: Endoplasmic reticulum membrane; Multi-pass membrane protein. Golgi apparatus membrane; Multi-pass membrane protein. Cell surface. Note=Bound to NOTCH1 also at the cell surface. Colocalizes with CDH1/2 at sites of cell-cell contact. Colocalizes with CTNNB1 in the endoplasmic reticulum and the proximity of the plasma membrane. Also present in azurophil granules of neutrophils. Tissue Specificity: Expressed in a wide range of tissues including various regions of the brain, liver, spleen and lymph nodes. Post-translational modifications: Heterogeneous proteolytic processing generates N-terminal (NTF) and C-terminal (CTF) fragments of approximately 35 and 20 kDa, respectively. During apoptosis, the C-terminal fragment (CTF) is further cleaved by caspase-3 to produce the fragment, PS1-CTF12. After endoproteolysis, the C-terminal fragment (CTF) is phosphorylated on serine residues by PKA and/or PKC. Phosphorylation on Ser-346 inhibits endoproteolysis. DISEASE: Defects in PSEN1 are a cause of Alzheimer disease type 3 (AD3) [MIM:607822]. AD3 is a familial early-onset form of Alzheimer disease. Alzheimer disease is a neurodegenerative disorder characterized by progressive dementia, loss of cognitive abilities, and deposition of fibrillar amyloid proteins as intraneuronal neurofibrillary tangles, extracellular amyloid plaques and vascular amyloid deposits. The major constituent of these plaques is the neurotoxic amyloid-beta-APP 40-42 peptide (s), derived proteolytically from the transmembrane precursor protein APP by sequential secretase processing. The cytotoxic C-terminal fragments (CTFs) and the caspase-cleaved products such as C31 derived from APP, are also implicated in neuronal death. Defects in PSEN1 are a cause of frontotemporal dementia (FTD) [MIM:600274]. [DISEASE] Defects in PSEN1 are the cause of cardiomyopathy dilated type 1U (CMD1U) [MIM:613694]. It is a disorder characterized by ventricular dilation and impaired systolic function, resulting in congestive heart failure and arrhythmia. Patients are at risk of premature death. Defects in PSEN1 are the cause of familial acne inversa type 3 (ACNINV3) [MIM:613737]. A chronic relapsing inflammatory disease of the hair follicles characterized by recurrent draining sinuses, painful skin abscesses, and disfiguring scars. Manifestations typically appear after puberty. Similarity: Belongs to the peptidase A22A family. Database links: Entrez Gene: 5663 Human Entrez Gene: 19164 Mouse Entrez Gene: 29192 Rat Omim: 104311 Human SwissProt: P49768 Human SwissProt: P49769 Mouse SwissProt: P97887 Rat Unigene: 3260 Human Unigene: 998 Mouse Unigene: 44440 Rat Important Note: This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications. |
阿尔茨海默病(AD)患者的遗传形式的疾病携带突变的早老蛋白(PSEN1,PSEN2)或淀粉样前体蛋白(APP)。这些与疾病相关的突变导致长形淀粉样β(AD脑中淀粉样蛋白沉积的主要成分)的产生增加。早老素是通过调节其对γ分泌酶的调节来调节APP处理的,这是一种内切酶复合物,其催化膜膜蛋白如Notch受体和APP的膜内切割。