84-1082-89　Merlin 2 (Schwannomin, CN, BANF, SCH, Neurofibromin 2, Schwannomerlin, Neurofibromatosis-2, NF2) 100ul　M2965-30

特徴

• Na+/ H+ exchanger regulatory factor (NHERF1) is a PDZ domain containing adaptor protein known to bind to various receptors, channels, cytoskeletal elements and cytoplasmic signaling proteins.
• A 358aa mainly expressed in kidney, liver and pancrease by NHERF gene (chrm 17).
• And is represented in another isoform, NHERF2.
• NHERF1 protein contains 2 tandem PDZ domains of approximately 90aa and a C-terminal sequence that binds several members of the ERM (ezrin-radixin-moesin) family of membrane cytoskeletal adapters.
• The protein plays an important role in regulation of NHE3, turnover of G-protein coupled receptors, platelet derived growth factor receptor and ion transporters such as CFTR, Na/Pi cotransporter, NaHCO3 cotransporter and Trp channels.
• NHERF1 overexpression in cancers and mutations in NHERF1 targets, Merlin, the product of tumor suppressor gene, also expressed in 2 isoforms, Merlin is involved in pathogenesis of benign tumors of the human nervous system, Merlin is named for its striking similarity with ERM family which thought to link cytoskeletal components with proteins in the cell membrane, It has a binding partner called Syntenin, which is an adapter protein that couples transmembrane proteoglycans to cytoskeletal components.
• Merlin, Isoforms 1 and 2 are predominant, isoforms 4, 5 and 6 are expressed moderately, and isoform 8 is found at low frequency.
• Isoforms 7, 9 and 10 are not expressed in adult tissues, isoforms 1& 2 are 595aa and 590aa each in human, from same gene locus on chromosome 22q12, with approximate mol.
• wt of 70kD, they are structurally related to moesin, ezrin and redixin but not functionally.
• The Merlin isoforms are widely expressed in heart, lungs, skeletal muscle and spleen.
• Since being a membrane stabilizing protein, defects may lead to neurofibromatosis 2 a genetic disorder characterized by bilateral vestibular schwannomas.
• Affected individuals generally develop symptoms of eighth-nerve dysfunction in early adulthood, including deafness and balance disorder.
• Applications:Suitable for use in ELISA.
• Western Blot, though not tested, may potentially be used as an application.
• Other applications not tested.
• Recommended Dilution:Western Blot: 1-10ug/ml for affinity pure antibody using ECL technique
• ELISA:  Control peptide can be used to coat ELISA plates at 1ug/ml and detected with antibodies (0.5-1ug/ml for affinity pure).
• Storage and Stability:Lyophilized powder may be stored at -20°C.
• Stable for 12 months at -20°C.
• Reconstitute with sterile ddH2O or PBS.
• Aliquot to avoid repeated freezing and thawing.
• Store at -20°C.
• Reconstituted product is stable for 12 months at -20°C.
• For maximum recovery of product, centrifuge the original vial after thawing and prior to removing the cap.
• Further dilutions can be made in assay buffer.