Rat Sodium channel protein type 4 subunit alpha (SCN4A) ELISA kit; HYKPP; HYPP; NAC1A; Na (V) 1.4; Nav1.4; SkM1; skeletal muscle voltage-dependent sodium channel type IV alpha subunit; voltage-gated sodium channel type 4 alpha; sodium channel; voltage-gated; type IV; alpha subunit

For Research Use Only. Not for use in diagnostic procedures.

Store all reagents at 2-8 degree C

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Small volumes of SCN4A elisa kit vial(s) may occasionally become entrapped in the seal of the product vial during shipment and storage. If necessary, briefly centrifuge the vial on a tabletop centrifuge to dislodge any liquid in the container`s cap. Certain products may require to ship with dry ice and additional dry ice fee may apply.

MBS9337981 is a ready-to-use microwell, strip plate ELISA (enzyme-linked immunosorbent assay) Kit for analyzing the presence of the sodium channel, voltage-gated, type IV, alpha subunit (SCN4A) ELISA Kit target analytes in biological samples. The concentration gradients of the kit standards or positive controls render a theoretical kit detection range in biological research samples containing SCN4A. The ELISA analytical biochemical technique of the MBS9337981 kit is based on SCN4A antibody-SCN4A antigen interactions (immunosorbency) and an HRP colorimetric detection system to detect SCN4A antigen targets in samples. The ELISA Kit is designed to detect native, not recombinant, SCN4A. Appropriate sample types may include undiluted body fluids and/or tissue homogenates, secretions. Quality control assays assessing reproducibility identified the intra-assay CV (%) and inter-assay CV(%).

208,867 Da

sodium channel, voltage-gated, type IV, alpha subunit

sodium channel protein type 4 subunit alpha; mu-1; skM1; sodium channel protein type IV subunit alpha; voltage-gated sodium channel subunit alpha Nav1.4; sodium channel protein skeletal muscle subunit alpha; sodium channel, voltage-gated, type 4, alpha subunit; sodium channel voltage-gated type 4 alpha polypeptide; Sodium channel voltage-gated type IV alpha polypeptide; sodium channel, voltage-gated, type 4, alpha polypeptide; sodium channel, voltage-gated, type IV, alpha polypeptide

Sodium channel protein type 4 subunit alpha

SCN4A_RAT

voltage-gated Na+ channel subunit [RGD, Feb 2006]

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

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