- 当前位置：首页 > >Merck Millipore > 生命科学研究
- 生命科学研究
- 产品介绍
重要规格表
品种反应性 主要应用 宿主 格式 抗体类型 Mk, H ICC, WB Rb Serum Polyclonal Antibody 描述 产品目录编号 AB3598 品牌系列 Chemicon®
商名 - Chemicon
描述 Anti-Mitochondria Antibody 背景信息 Presenilin 1 is a protein found in brain cells. Presenilin 1 has a major influence on the risk of contracting alzheimer's disease. Its function is unknown. However, it may be involved in protein production and trafficking, especially during early development. More than 40 different mutations have been found in the presenilin 1 gene, and these mutations associate with early-onset familial alzheimer's disease. Mutations in presenilin 1 tend to elevate levels of amyloid beta in the blood, cerebrospinal fluid, and brains of those affected by mutations 产品信息 格式 Serum 控制 - HeLa whole cell extract, human neuroblastoma SH-SY5Y cells
演示 Rabbit serum preabsorbed against immunogen peptide. Liquid. Contains 0.01% sodium azide. 应用 应用 Anti-Mitochondria Antibody is an antibody against Mitochondria for use in WB & IC. 主要应用 - Immunocytochemistry
- Western Blotting
应用说明 Immunoblotting: 1:500-1:1000
Immunocytochemistry: 1:100-1:300 on COS7, HeLa, 293 and SK-N-MC cell lines.
Optimal working dilutions must be determined by the end user.生物信息 免疫原品种 Presenilin 1 peptide conjugated to KLH. 宿主 Rabbit 特异性 Mitochondria. Recognizes a 62 kD protein of immunoblots of subcellular fractions enriched in mitochondrial fractions.
The antibody shows a moderate titer by ELISA to the immunogen peptide but does not immunoblot any expected presenilin 1 bands. Immunocytochemistry with the serum revealed a mitochondria-like staining that was still present after exhaustive preabsorption with the peptide. The staining co-localizes with CHEMICON's anti-mitochondria antibody MAB1273.品种反应性 MonkeyHuman 抗体类型 Polyclonal Antibody Entrez基因编号 - NM_000021.2
Entrez基因汇总 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. Multiple alternatively spliced transcript variants have been identified for this gene, the full-length natures of only some have been determined. 基因符号 - Presenilin-1 CTF12 (PS1-CTF12)].
- PSEN1
- PS1
- PSNL1
- PS-1
- AD3
- FAD
- S182
- Presenilin-1
- EC 3.4.23.- [Contains: Presenilin-1 NTF subunit
- Presenilin-1 CTF subunit
UniProt编号 - P49768
UniProt汇总 FUNCTION: SwissProt: P49768 # 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.
SIZE: 467 amino acids; 52668 Da
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. Component of 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 (By similarity).
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.
DOMAIN: SwissProt: P49768 The PAL motif is required for normal active site conformation.
PTM: 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: SwissProt: P49768 # Defects in PSEN1 are a cause of familial early-onset Alzheimer disease type 3 (AD3) [MIM:607822]. AD3 is the most severe form of the disease, with complete penetrance and an onset occurring as early as 30 years of age. The second form is late- onset AD (LOAD), with mean age of onset greater than 58 years. AD is an autosomal dominant neurodegenerative disorder characterized by progressive dementia, parkinsonism, and deposition of fibrillar amyloid proteins as intraneuronal neurofibrillary tangles, extracellular amyloid plaques and vascular amyloid deposits. The major protein found within these deposits is a small, insoluble and highly aggregating polypeptide, beta-amyloid protein (beta- APP42). Defects in PSEN1 result in an overproduction of beta- APP42. Variant Pro-166, a very aggressive mutation that causes onset of AD3 in adolescence, not only induces an exceptionally high increase of beta-APP42 production, but also impairs Notch intracellular domain production and Notch signaling, as well as beta-APP intracellular domain generation. & Defects in PSEN1 are a cause of frontotemporal dementia [MIM:600274].
SIMILARITY: Belongs to the peptidase A22A family.产品使用声明 使用声明 - Unless otherwise stated in our catalog or other company documentation accompanying the product(s), our products are intended for research use only and are not to be used for any other purpose, which includes but is not limited to, unauthorized commercial uses, in vitro diagnostic uses, ex vivo or in vivo therapeutic uses or any type of consumption or application to humans or animals.
储存和货运信息 存储条件 Maintain at -20°C in undiluted aliquots for up to 12 months. Avoid repeated freeze-thaw cycles. 包装信息 数量 100 µL
上一件merck millipore产品：merck millipore,默克密理博,489479,LSD1 Inhibitor IV, RN-1,HCl - Calbiochem
下一件merck millipore产品：merck millipore,默克密理博,AX90010TC,AXIS™ Axon Isolation Device, 900 µm
相关产品
merck millipore,默克密理博,PSMT010R1,Millicell-24 插入式细胞培养板, 聚碳酸酯, 5.0 µm
merck millipore,默克密理博,368975,Guanidine Thiocyanate - CAS 593-84-0 - Calbiochem
merck millipore,默克密理博,524718,Phosphodiesterase Inhibitor Set I - Calbiochem
merck millipore,默克密理博,SF-131,RNA Pol II; Human, Cyanine 3, RNA Detection Probe,SmartFlare
merck millipore,默克密理博,489479,LSD1 Inhibitor IV, RN-1,HCl - Calbiochem
merck millipore,默克密理博,AX90010TC,AXIS™ Axon Isolation Device, 900 µm
merck millipore,默克密理博,S25-100ML,Mouse Serum
merck millipore,默克密理博,MAB2256,Anti-Tropomyosin-5 Antibody, clone 2G10.2
