Anti-MFN2 Antibody (RACO0528)
- SKU:
- RACO0528
- Product type:
- Recombinant Antibody
- Reactivity:
- Human
- Host Species:
- Human
- Isotype:
- IgG
- Application:
- ELISA
- Application:
- IHC
- Conjugation:
- Unconjugated
Frequently bought together:
Description
| 商品名: | MFN2 Antibody |
| Product SKU: | RACO0528 |
| サイズ: | 50ul |
| 宿主種: | Homo sapiens (Human) |
| 申し込み: | ELISA, IHC |
| 推奨される希釈: | IHC:1:50-1:200 |
| 反応性: | Human |
| 免疫原: | A synthesized peptide derived from human Mitofusin 2 |
| 憲法: | Liquid |
| ストレージバッファ: | Rabbit IgG in phosphate buffered saline, pH 7.4, 150mM NaCl, 0.02% sodium azide and 50% glycerol. |
| 精製方法: | Affinity-chromatography |
| 抗体のクローン性: | Monoclonal |
| アイソタイプ: | Rabbit IgG |
| Conjugate: | Non-conjugated |
| バックグラウンド: | Mitochondrial outer membrane GTPase that mediates mitochondrial clustering and fusion (PubMed:11181170, PubMed:11950885, PubMed:28114303). Mitochondria are highly dynamic organelles, and their morphology is determined by the equilibrium between mitochondrial fusion and fission events (PubMed:28114303). Overexpression induces the formation of mitochondrial networks (PubMed:28114303). Membrane clustering requires GTPase activity and may involve a major rearrangement of the coiled coil domains (Probable). Plays a central role in mitochondrial metabolism and may be associated with obesity and/or apoptosis processes (By similarity). Plays an important role in the regulation of vascular smooth muscle cell proliferation (By similarity). Involved in the clearance of damaged mitochondria via selective autophagy (mitophagy) (PubMed:23620051). Is required for PRKN recruitment to dysfunctional mitochondria (PubMed:23620051). Involved in the control of unfolded protein response (UPR) upon ER stress including activation of apoptosis and autophagy during ER stress (By similarity). Acts as an upstream regulator of EIF2AK3 and suppresses EIF2AK3 activation under basal conditions (By similarity). |
| シノニム: | Mitofusin-2 (EC 3.6.5.-) (Transmembrane GTPase MFN2), MFN2, CPRP1 KIAA0214 |
 | IHC image of RACO0528 diluted at 1:100 and staining in paraffin-embedded human kidney tissue performed on a Leica BondTM system. After dewaxing and hydration, antigen retrieval was mediated by high pressure in a citrate buffer (pH 6.0). Section was blocked with 10% normal goat serum 30min at RT. Then primary antibody (1% BSA) was incubated at 4°C overnight. The primary is detected by a Goat anti-rabbit IgG polymer labeled by HRP and visualized using 0.05% DAB. |
| UniProt Protein Function: | MFN2: Essential transmembrane GTPase, which mediates mitochondrial fusion. Fusion of mitochondria occurs in many cell types and constitutes an important step in mitochondria morphology, which is balanced between fusion and fission. MFN2 acts independently of the cytoskeleton. It therefore plays a central role in mitochondrial metabolism and may be associated with obesity and/or apoptosis processes. Overexpression induces the formation of mitochondrial networks. Plays an important role in the regulation of vascular smooth muscle cell proliferation. Defects in MFN2 are the cause of Charcot-Marie-Tooth disease type 2A2 (CMT2A2). CMT2A2 is a form of Charcot-Marie-Tooth disease, the most common inherited disorder of the peripheral nervous system. Charcot-Marie-Tooth disease is classified in two main groups on the basis of electrophysiologic properties and histopathology: primary peripheral demyelinating neuropathy or CMT1, and primary peripheral axonal neuropathy or CMT2. Neuropathies of the CMT2 group are characterized by signs of axonal regeneration in the absence of obvious myelin alterations, normal or slightly reduced nerve conduction velocities, and progressive distal muscle weakness and atrophy. Defects in MFN2 are the cause of Charcot-Marie-Tooth disease type 6 (CMT6); also referred to as autosomal dominant hereditary motor and sensory neuropathy VI (HMSN6). CMT6 is an autosomal dominant form of axonal CMT associated with optic atrophy. Belongs to the mitofusin family. 2 isoforms of the human protein are produced by alternative splicing. |
| UniProt Protein Details: | Protein type:Cell cycle regulation; Cytoskeletal; EC 3.6.5.-; Hydrolase; Membrane protein, integral; Membrane protein, multi-pass; Mitochondrial Chromosomal Location of Human Ortholog: 1p36.22 Cellular Component: cytosol; integral to membrane; intrinsic to mitochondrial outer membrane; mitochondrial outer membrane; mitochondrion Molecular Function:GTPase activity; protein binding; ubiquitin protein ligase binding Biological Process: blood coagulation; cell cycle arrest; macroautophagy; mitochondrial fusion; mitochondrial membrane organization and biogenesis; mitochondrion localization; negative regulation of Ras protein signal transduction; negative regulation of smooth muscle cell proliferation; protein targeting to mitochondrion Disease: Charcot-marie-tooth Disease, Axonal, Autosomal Recessive, Type 2a2b; Charcot-marie-tooth Disease, Axonal, Type 2a2; Neuropathy, Hereditary Motor And Sensory, Type Vi |
| NCBI Summary: | This gene encodes a mitochondrial membrane protein that participates in mitochondrial fusion and contributes to the maintenance and operation of the mitochondrial network. This protein is involved in the regulation of vascular smooth muscle cell proliferation, and it may play a role in the pathophysiology of obesity. Mutations in this gene cause Charcot-Marie-Tooth disease type 2A2, and hereditary motor and sensory neuropathy VI, which are both disorders of the peripheral nervous system. Defects in this gene have also been associated with early-onset stroke. Two transcript variants encoding the same protein have been identified. [provided by RefSeq, Jul 2008] |
| UniProt Code: | O95140 |
| NCBI GenInfo Identifier: | 47605777 |
| NCBI Gene ID: | 9927 |
| NCBI Accession: | O95140.3 |
| UniProt Secondary Accession: | O95140,O95572, Q5JXC3, Q5JXC4, Q9H131, Q9NSX8, A8K1B3 |
| UniProt Related Accession: | O95140 |
| Molecular Weight: | 86kDa |
| NCBI Full Name: | Mitofusin-2 |
| NCBI Synonym Full Names: | mitofusin 2 |
| NCBI Official Symbol: | MFN2 |
| NCBI Official Synonym Symbols: | HSG; MARF; CMT2A; CPRP1; CMT2A2; HMSN6A; CMT2A2A; CMT2A2B |
| NCBI Protein Information: | mitofusin-2 |
| UniProt Protein Name: | Mitofusin-2 |
| UniProt Synonym Protein Names: | Transmembrane GTPase MFN2 |
| Protein Family: | Mitofusin |
| UniProt Gene Name: | MFN2 |