货号：AG318

品牌：Merck Millipore

规格：100Ug

目录价：￥3456.00

市场价格：￥2937.60

会员价格：￥2764.80

金山科研平台，产品价格货期咨询微信：jinshanbio Description: Acetylcholine Receptor-γ, control peptide for AB5936 View All» Trade Name: Chemicon (Millipore) View All» Qty/Pk: 100 µg View All» Product Overview: Peptide from mouse acetylcholine receptor gamma. View All» Key Applications: Peptide Inhibition Assay View All» Application Notes: For use in blocking the reactivity of AB5936. Optimal working dilution must be determined by the end user. View All» Usage Statement: Unless otherwise stated in our catalog or other company documentation accompanying the product(s), our products are intended for research use only and are not to be used for any other purpose, which includes but is not limited to, unauthorized commercial uses, in vitro diagnostic uses, ex vivo or in vivo therapeutic uses or any type of consumption or application to humans or animals. View All» Entrez Gene Summary: For background information on the acetylcholine receptor (AChR), see CHRNA1 (MIM 100690). Two forms of AChR are found in mammalian skeletal muscle cells. The mature form is predominant in innervated adult muscle and the embryonic form is present in fetal and denervated muscle. Embryonic and mature AChR differ by the replacement of the gamma subunit in the pentameric glycoprotein complex by its isoform, the epsilon subunit (MIM 100725), which is specific to the mature AChR subtype. This switch is mediated by ARIA (acetylcholine receptor-inducing activity; MIM 142445).[supplied by OMIM] View All» UniProt Summary: FUNCTION: SwissProt: P07510 # After binding acetylcholine, the AChR responds by an extensive change in conformation that affects all subunits and leads to opening of an ion-conducting channel across the plasma membrane.SIZE: 517 amino acids; 57883 Da SUBUNIT: Pentamer of two alpha chains, and one each of the beta, delta, and gamma (in immature muscle) or epsilon (in mature muscle) chains.SUBCELLULAR LOCATION: Cell junction, synapse, postsynaptic cell membrane; Multi-pass membrane protein.DISEASE: SwissProt: P07510 # Defects in CHRNG are a cause of lethal type multiple pterygium syndrome [MIM:253290]. Multiple pterygia are found infrequently in children with arthrogryposis and in fetuses with fetal akinesia syndrome. Inheritance can be autosomal dominant, autosomal recessive, or X linked, but autosomal recessive inheritance appears to be most common. Clinical expression is very variable, and, in the severest form, lethal multiple pterygium syndrome there is intrauterine growth retardation, multiple pterygia (e.g., chin to sternum, cervical, axillary, humero-ulnar, crural, popliteal, and ankles), and flexion contractures causing severe arthrogryposis and fetal akinesia. Subcutaneous edema can be severe, causing fetal hydrops with cystic hygroma and lung hypoplasia. Oligohydramnios and facial anomalies-in particular, cleft palate-are frequent. & Defects in CHRNG are a cause of Escobar syndrome [MIM:265000]; also called Escobar variant multiple pterygium syndrome or nonlethal type multiple pterygium syndrome. Escobar syndrome is a nonlethal form of arthrogryposis multiplex congenita. It is an autosomal recessive condition characterized by excessive webbing (pterygia), congenital contractures (arthrogryposis), and scoliosis. Variable other features include intrauterine death, congenital respiratory distress, short stature, faciocranial dysmorphism, ptosis, low-set ears, arachnodactyly and cryptorchism in males. Congenital contractures are common and may be caused by reduced fetal movements at sensitive times of development. Possible causes of decreased fetal mobility include space constraints such as oligohydramnion, drugs, metabolic conditions or neuromuscular disorders including myasthenia gravis.SIMILARITY: SwissProt: P07510 ## Belongs to the ligand-gated ionic channel (TC 1.A.9) family. View All» Species: Mouse View All» Brand Family: Chemicon View All» Presentation: Liquid in PBS. View All» Storage Conditions: Maintain at -20°C in undiluted aliquots for up to 6 months after date of receipt. Avoid repeated freeze/thaw cycles. View All» Gene Symbol:

• CHRNG

• MGC133376

• ACHRG
  View All» Product Name: Acetylcholine Receptor-γ, control peptide for AB5936 View All» Concentration: 1.0 mg/mL View All» UniProt Number: P07510 View All» Entrez Gene Number: NM_005199.4 View All»
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