跨膜蛋白BRI抗体
规格：1mg/1ml
英文名： ITM2B
别名： ABRI; ABri/ADan amyloid peptide; BRI 2; BRI; BRI2; BRICD 2B; BRICD2B; BRICHOS domain containing 2B; E25B; E3 16; E3-16; FBD; Integral membrane protein 2B; ITM 2B; ITM2B ; ITM2B_HUMAN; Protein E25B; Tr
分子量： 30kDa
储存液：0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glyce
克隆类型：Polyclonal
亚型：IgG
纯化方法：affinity purified by Protein A
**原：KLH conjugated synthetic peptide derived from human ITM2B
交叉反应：Human, Mouse, Rat, Dog,
细胞定位：细胞膜
跨膜蛋白BRI抗体产品介绍：background: The type II integral membrane (ITM2) protein family consists of three members: ITM2A (also designated E25), ITM2B and ITM2C. ITM2A expression is high in osteogenic and lymphoid tissues, while both ITM2B and ITM2C are expressed in brain. ITM2B is a 266 amino acid protein that contains a potential N-glycosylation site, a potential single transmembrane-spanning domain between amino acids 52 and 74 and an extracellular C-terminal domain. Mutations in the ITM2B gene can lead to familial British dementia (FBD), and autosomal dominant disease with an onset around the fifth decade of life that is characterized by progressive dementia, spasticity and cerebellar ataxia. Familial Danish dementia (FDD), also designated heredopathia ophthalmo-oto-encephalica, is also associated with mutations in the ITM2B gene. FDD is an autosomal dominant disorder characterized by cataracts, deafness, progressive ataxia and dementia. Function: Functions as a protease inhibitor. Plays a role in APP processing regulating the physiological production of the beta amyloid peptide. Restricts docking of gamma-secretase to APP and access of alpha- and beta-secretase to their cleavage APP sequence. Subunit: Homodimer; disulfide-linked. Interacts with SPPL2A and SPPL2B. Interacts with APP. Mature BRI2 (mBRI2) interacts with the APP amyloid beta A4 protein; the interaction occurs at the cell surface and in the endocytic compartments and enable alpha- and beta-secretase-induced APP cleavage inhibition. Mature BRI2 (mBRI2) interacts with the APP C99; the interaction occurs in the endocytic compartments and enable gamma-secretase-induced C99 cleavage inhibition. May form heterodimers with Bri23 peptide and跨膜蛋白BRI抗体 APP beta-amyloid protein 40. Subcellular Location: Golgi apparatus membrane. Cell membrane. Tissue Specificity: Expressed in brain and in other tissues. Post-translational modifications: The C-terminal part of the ectodomain is processed by furin and related proteases producing a secreted peptide of 4 to 5 kDa. For the ABRI and ADAN variants the C-terminal secreted peptide is larger and may produce amyloid fibrils responsible for neuronal dysfunction and dementia. The remaining part of the ectodomain containing the BRICHOS domain is cleaved by ADAM10 and is secreted as a peptide of 25 kDa. The membrane-bound N-terminal fragment (NTF) of 22 kDa is further proteolytically processed by SPPL2A and SPPL2B through regulated intramembrane proteolysis producing a secreted peptide (BRI2C) and an intracellular domain (ICD) released in the cytosol. DISEASE: Defects in ITM2B are a cause of cerebral amyloid angiopathy ITM2B-related type 1 (CAA-ITM2B1) [MIM:176500]. A disorder characterized by amyloid deposition in the walls of cerebral blood vessels and neurodegeneration in the central nervous system. Cerebral amyloid angiopathy, non-neuritic and perivascular plaques and neurofibrillary tangles are the predominant pathological lesions. Clinical features include progressive mental deterioration, spasticity and muscular rigidity. Defects in ITM2B are a cause of cerebral amyloid angiopathy ITM2B-related type 2 (CAA-ITM2B2) [MIM:117300]; also known as heredopathia ophthalmo-oto-encephalica. A disorder characterized by amyloid deposition in the walls of the blood vessels of the cerebrum, choroid plexus,跨膜蛋白BRI抗体 cerebellum, spinal cord and retina. Plaques and neurofibrillary tangles are observed in the hippocampus. Clinical features include progressive ataxia, dementia, cataracts and deafness. Similarity: Belongs to the ITM2 family. Contains 1 BRICHOS domain. Gene ID: 9445 Database links: Entrez Gene: 510575 Cow Entrez Gene: 476916 Dog Entrez Gene: 9445 Human Entrez Gene: 16432 Mouse Entrez Gene: 595120 Rabbit Entrez Gene: 290364 Rat Omim: 603904 Human SwissProt: Q3T0P7 Cow SwissProt: Q9Y287 Human SwissProt: O89051 Mouse SwissProt: Q52N47 Pig SwissProt: Q5XIE8 Rat Unigene: 23522 Cow Unigene: 4266 Human Unigene: 643683 Human Unigene: 107335 Rat Important Note: This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.
跨膜蛋白BRI抗体产品应用：WB=1:100-500 ELISA=1:500-1000 IHC-P=1:100-500 IHC-F=1:100-500 ICC=1:100-500 IF=1:100-500 （石蜡切片需做抗原修复） not yet tested in other applications. optimal dilutions/concentrations should be determined by the end user.
研究领域：细胞生物 **学 神经生物学 Alzheimer's
储存条件： Store at -20 °C for one year. Avoid repeated freeze/thaw cycles.
来源： Rabbit
外观： Lyophilized or Liquid