Western Blot (WB)
(Western blot analysis of extracts from K562 cells, using SCN4A antibody.)
Rabbit anti-Human SCN4A Polyclonal Antibody | anti-SCN4A antibody
SCN4A Antibody
Western Blot (WB)
(Western blot analysis of extracts from K562 cells, using SCN4A antibody.)
Western Blot (WB)
(Western blot analysis of extracts from K562 cells, using SCN4A antibody.)
NCBI and Uniprot Product Information
NCBI Description
Voltage-gated sodium channels are transmembrane glycoprotein complexes composed of a large alpha subunit with 24 transmembrane domains and one or more regulatory beta subunits. They are responsible for the generation and propagation of action potentials in neurons and muscle. This gene encodes one member of the sodium channel alpha subunit gene family. It is expressed in skeletal muscle, and mutations in this gene have been linked to several myotonia and periodic paralysis disorders. [provided by RefSeq, Jul 2008]
Uniprot Description
SCN4A: This protein mediates the voltage-dependent sodium ion permeability of excitable membranes. Assuming opened or closed conformations in response to the voltage difference across the membrane, the protein forms a sodium-selective channel through which Na(+) ions may pass in accordance with their electrochemical gradient. This sodium channel may be present in both denervated and innervated skeletal muscle. Defects in SCN4A are the cause of paramyotonia congenita of von Eulenburg (PMC). PMC is an autosomal dominant channelopathy characterized by myotonia, increased by exposure to cold, intermittent flaccid paresis, not necessarily dependent on cold or myotonia, lability of serum potassium, nonprogressive nature and lack of atrophy or hypertrophy of muscles. In some patients, myotonia is not increased by cold exposure (paramyotonia without cold paralysis). Patients may have a combination phenotype of PMC and HYPP. Defects in SCN4A are a cause of periodic paralysis hypokalemic type 2 (HOKPP2). It is an autosomal dominant disorder manifested by episodic flaccid generalized muscle weakness associated with falls of serum potassium levels. Defects in SCN4A are the cause of periodic paralysis hyperkalemic (HYPP). HYPP is an autosomal dominant channelopathy characterized by episodic flaccid generalized muscle weakness associated with high levels of serum potassium. Concurrence of myotonia is found in HYPP patients. Defects in SCN4A are the cause of periodic paralysis normokalemic (NKPP). NKPP is a disorder closely related to hyperkalemic periodic paralysis, but marked by a lack of alterations in potassium levels during attacks of muscle weakness. Defects in SCN4A are the cause of myotonia SCN4A-related (MYOSCN4A). Myotonia is characterized by sustained muscle tensing that prevents muscles from relaxing normally. Myotonia causes muscle stiffness that can interfere with movement. In some people the stiffness is very mild, while in other cases it may be severe enough to interfere with walking, running, and other activities of daily life. MYOSCN4A is a phenotypically highly variable myotonia aggravated by potassium loading, and often by cold. MYOSCN4A includes myotonia permanens and myotonia fluctuans. In myotonia permanens, the myotonia is generalized and there is a hypertrophy of the muscle, particularly in the neck and the shoulder. Attacks of severe muscle stiffness of the thoracic muscles may be life threatening due to impaired ventilation. In myotonia fluctuans, the muscle stiffness may fluctuate from day to day, provoked by exercise. Defects in SCN4A are the cause of a congenital myasthenic syndrome acetazolamide-responsive (CMSAR). A congenital myasthenic syndrome associated with fatigable generalized weakness and recurrent attacks of respiratory and bulbar paralysis since birth. The fatigable weakness involves lid- elevator, external ocular, facial, limb and truncal muscles and an decremental response of the compound muscle action potential on repetitive stimulation. Belongs to the sodium channel (TC 1.A.1.10) family. Nav1.4/SCN4A subfamily.
Protein type: Membrane protein, multi-pass; Channel, sodium; Membrane protein, integral
Chromosomal Location of Human Ortholog: 17q23.3
Cellular Component: voltage-gated sodium channel complex; integral to plasma membrane; plasma membrane
Molecular Function: voltage-gated sodium channel activity
Biological Process: muscle contraction; sodium ion transport; generation of action potential
Disease: Hyperkalemic Periodic Paralysis; Myotonia, Potassium-aggravated; Paramyotonia Congenita Of Von Eulenburg; Myasthenic Syndrome, Congenital, Acetazolamide-responsive; Hypokalemic Periodic Paralysis, Type 2; Hypokalemic Periodic Paralysis, Type 1
Research Articles on SCN4A
Similar Products
Product Notes
The SCN4A scn4a (Catalog #AAA9401263) is an Antibody produced from Rabbit and is intended for research purposes only. The product is available for immediate purchase. The SCN4A Antibody reacts with Human and may cross-react with other species as described in the data sheet. AAA Biotech's SCN4A can be used in a range of immunoassay formats including, but not limited to, Western Blot (WB). Western blotting: 1:500~1:3000. Researchers should empirically determine the suitability of the SCN4A scn4a for an application not listed in the data sheet. Researchers commonly develop new applications and it is an integral, important part of the investigative research process. It is sometimes possible for the material contained within the vial of "SCN4A, Polyclonal Antibody" to become dispersed throughout the inside of the vial, particularly around the seal of said vial, during shipment and storage. We always suggest centrifuging these vials to consolidate all of the liquid away from the lid and to the bottom of the vial prior to opening. Please be advised that certain products may require dry ice for shipping and that, if this is the case, an additional dry ice fee may also be required.Precautions
All products in the AAA Biotech catalog are strictly for research-use only, and are absolutely not suitable for use in any sort of medical, therapeutic, prophylactic, in-vivo, or diagnostic capacity. By purchasing a product from AAA Biotech, you are explicitly certifying that said products will be properly tested and used in line with industry standard. AAA Biotech and its authorized distribution partners reserve the right to refuse to fulfill any order if we have any indication that a purchaser may be intending to use a product outside of our accepted criteria.Disclaimer
Though we do strive to guarantee the information represented in this datasheet, AAA Biotech cannot be held responsible for any oversights or imprecisions. AAA Biotech reserves the right to adjust any aspect of this datasheet at any time and without notice. It is the responsibility of the customer to inform AAA Biotech of any product performance issues observed or experienced within 30 days of receipt of said product. To see additional details on this or any of our other policies, please see our Terms & Conditions page.Item has been added to Shopping Cart
If you are ready to order, navigate to Shopping Cart and get ready to checkout.
