U-Blot® Presenilin 2 Rabbit mAb
Ucallm_WR6025-50
Price range: $268.00 through $328.00
Product details
Background：
Alzheimer's disease (AD) patients with an inherited form of the disease carry mutations in the presenilin proteins (PSEN1 or 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 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 act are protease enzymes. Two alternatively spliced transcript variants encoding different isoforms of PSEN2 have been identified. [provided by RefSeq , Jul 2008] ,
Specifications
| Size | 50μL/100μL |
| Target | Presenilin 2 |
| Reactivity | Human, Mouse, Rat |
| Applications | WB, IHC, IF, IP, ELISA |
| MW(Calculated) | 50kD |
| MW(Observed) | 23 (C-terminal fragment),54 (full-length)kD |
| Host Species | Rabbit |
| Isotype | IgG, Kappa |
| Conjugate/Modification | Unmodified |
| Recommended Dilution Ratio | IHC 1:200-1:1000; WB 1:2000-1:10000; IF 1:200-1:1000; ELISA 1:5000-1:20000; IP 1:50-1:200 / Note: For IHC, we suggest antigen retrieval with TE buffer pH 9.0 (Cat#WT1004) |
| Formulation | PBS, 50% glycerol, 0.05% Proclin 300, 0.05%BSA |
| Purification | Recombinant Antibody expressed in animal component-free (ACF) media, purified via Protein A affinity chromatography. |
| Storage | -15°C to -25°C/1 year (Do not lower than -25°C) |
| Concentration | |
| Clonality | Monoclonal |
| Clone Number | RM1980R |
| Immunogen | The specific immunogen used to produce this antibody is proprietary information. |
| Specificity | Endogenous |
| Gene Name | PSEN2 AD4 PS2 PSNL2 STM2 |
| Protein Name | Presenilin-2 |
| Other Name | PSEN2; / AD4; / PS2; / PSNL2; / STM2; / Presenilin-2; / PS-2; / AD3LP; / AD5; / E5-1; / STM-2; / [Cleaved into: Presenilin-2 NTF subunit; / Presenilin-2 CTF subunit] |
| Species | Human |
| Gene ID | 5664 |
| Uniprot | P49810, |
| Species.1 | Mouse |
| Gene ID.1 | 19165 |
| Uniprot.1 | Q61144, |
| Species.2 | Rat |
| Gene ID.2 | 81751 |
| Uniprot.2 | O88777 |
| Cellular Localization | Endoplasmic reticulum membrane; Multi-pass membrane protein . Golgi apparatus membrane; Multi-pass membrane protein . |
| Tissue Expression | Isoform 1 is seen in the placenta , skeletal muscle and heart while isoform 2 is seen in the heart , brain , placenta , liver , skeletal muscle and kidney. |
| Signaling_pathway | Organismal Systems >> Nervous system >> Neurotrophin signaling pathway;Human Diseases >> Neurodegenerative disease >> Alzheimer disease;Human Diseases >> Neurodegenerative disease >> Pathways of neurodegeneration - multiple diseases;Environmental Information Processing >> Signal transduction >> Notch signaling pathway |
| Research Areas | >>Notch signaling pathway; / >>Neurotrophin signaling pathway; / >>Alzheimer disease; / >>Pathways of neurodegeneration - multiple diseases |
| Function | Disease:Defects in PSEN2 are the cause of Alzheimer disease type 4 (AD4) [MIM:606889]. AD is an autosomal dominant 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. ,Disease:Three causative genes have been identified that when mutated lead to presenile Alzheimer Disease: APP (amyloid precursor protein gene) , PSEN1 and PSEN2. These three genes account for half of the families with autosomal dominant presenile AD , which represent approximately 10% of the whole AD population. In addition , apolipoprotein E has been identified as a risk-modifying locus. ,Domain:The PAL motif is required for normal active site conformation. ,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. May function in the cytoplasmic partitioning of proteins. ,online information:Presenilins mutations ,PTM:Heterogeneous proteolytic processing generates N-terminal and C-terminal fragments. ,PTM:Phosphorylated on serine residues. ,similarity:Belongs to the peptidase A22A family. ,subunit:Interacts with DOCK3 (By similarity) . 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 , although other components may exist. Interacts with HERPUD1 , FLNA , FLNB and PARL. ,tissue specificity:Isoform 1 is seen in the placenta , skeletal muscle and heart while isoform 2 is seen in the heart , brain , placenta , liver , skeletal muscle and kidney. , |
Please login to post questions
Related products
