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Product Name Mitofusin 2 Chinese Name Mitochondrion融合蛋白Mfn2单克隆抗体 Alias CMT2A; CMT2A2; MARF; CPRP 1; CPRP1; Fzo; HSG; hyperplasia suppressor gene; Hypertension related protein 1; MFN 2; Mfn2; MFN2_HUMAN; Mitochondrial assembly regulatory factor; Mitofusin-2; Mitofusin2; Transmembrane GTPase MFN2. literatures Specific References (1) | SLM-60007M has been referenced in 1 publications.[IF=5.572] Miao Song. et al. Mitophagy alleviates AIF-mediated spleen apoptosis induced by AlCl3 through Parkin stabilization in mice. FOOD CHEM TOXICOL. 2023 Jun;176:113762 WB ; Mouse.Research Area Cardiovascular Cell biology immunology Neurobiology Immunogen Species Mouse Clonality Monoclonal React Species Human, Mouse, Applications WB=1:500-2000
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.Theoretical molecular weight 83kDa Cellular localization cytoplasmic The cell membrane Form Liquid Concentration 1mg/ml immunogen KLH conjugated synthetic peptide derived from human Mitofusin 2 Lsotype IgG2a Purification affinity purified by Protein A Buffer Solution 0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol. Storage Shipped at 4℃. Store at -20 °C for one year. Avoid repeated freeze/thaw cycles. Attention This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications. PubMed PubMed Product Detail 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].
Subunit:
Forms homomultimers and heteromultimers with MFN1.
Subcellular Location:
Mitochondrion outer membrane; Multi-pass membrane protein.
Tissue Specificity:
Ubiquitous; expressed at low level. Highly expressed in heart and kidney.
DISEASE:
Defects in MFN2 are the cause of Charcot-Marie-Tooth disease type 2A2 (CMT2A2) [MIM:609260]. 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) [MIM:601152]; 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.
Similarity:
Belongs to the mitofusin family.
SWISS:
O95140
Gene ID:
9927
Database links:Entrez Gene: 9927 Human
Entrez Gene: 170731 Mouse
SwissProt: O95140 Human
SwissProt: Q80U63 Mouse
Unigene: 376681 Human
Product Picture Sample:
Lane 1: Mouse NIH/3T3 cell lysates
Lane 2: Human HeLa cell lysates
Lane 3: Human HepG2 cell lysates
Primary: Anti-Mitofusin 2 (SLM-60007M) at 1/1000 dilution
Secondary: IRDye800CW Goat Anti- Mouse IgG at 1/20000 dilution
Predicted band size: 83 kDa
Observed band size: 76 kDa
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