FITC标记的早老素蛋白-1抗体-抗体-抗体-生物在线
上海沪震实业有限公司
FITC标记的早老素蛋白-1抗体

FITC标记的早老素蛋白-1抗体

商家询价

产品名称: FITC标记的早老素蛋白-1抗体

英文名称: Anti-presenilin 1/FITC

产品编号: HZ-0024R-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: Flow-Cyt=1:50-200 IF=1:50-200

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 Rabbit Anti-presenilin 1/FITC Conjugated antibody 

FITC标记的早老素蛋白-1抗体

 

英文名称 Anti-presenilin 1/FITC
中文名称 FITC标记的早老素蛋白-1抗体
别    名 Presenilin-1 NTF subunit; AD 3; AD3; Ad3h; Alzheimer Disease 3; EC 3.4.23.; FAD; Homo Sapiens Clone CC44 Senilin 1; Presenilin 1 Alzheimer disease 3; Presenilin 1; Presenilin1; Protein S182; PS 1; PS-1; PS1; PSEN 1; PSEN1; PSN 1; PSN1; PSN1_HUMAN; PSNL 1; PSNL1; S182; S182 Protein; Senilin 1; Senilin1.  
规格价格 100ul/2980元 购买        大包装/询价
说 明 书 100ul  
研究领域 神经生物学  细胞凋亡  
抗体来源 Rabbit
克隆类型 Polyclonal
交叉反应 Human, Mouse, Rat, Dog, Pig, Cow, Rabbit, 
产品应用 Flow-Cyt=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 34/52kDa
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 in 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 enzyme that cleaves APP. Also, it is thought that the presenilins are involved in the cleavage of the Notch receptor, such that they either directly regulate gamma-secretase activity or themselves are protease enzymes. Several alternatively spliced transcript variants encoding different isoforms have been identified for this gene, the full-length nature of only some have been determined. [provided by RefSeq, Aug 2008]

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]. 

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. 

此抗体识别分子量为45-50kDa早老素蛋白-1。PS-1主要在神经细胞中表达,早老蛋白集中于体细胞和树突状细胞中。相反,再早发家族AD(FAD)中和散发AD病人中,PS1免疫反应出现在老年斑和神经纤维缠结的神经炎中
   
   

遗传性阿尔茨海默病(AD)患者携带早老素蛋白(PSEN1;PSEN2)或淀粉样前体蛋白(APP)的突变。这些与疾病相关的突变导致更长形式的淀粉样β的产生(AD大脑中发现的淀粉样沉积物的主要成分)。早老素被假定为通过对γ分泌酶的调节来调节APP处理,γ分泌酶是裂解APP的酶。此外,人们认为早老素参与Notch受体的裂解,从而它们要么直接调节γ-分泌酶活性,要么本身就是蛋白酶。已经为这个基因鉴定了几个编码不同异构体的选择性剪接的转录体变体,仅确定了一些的全长性质。[ RefSeq,Aug 2008提供]