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PrecisION hNav1.1-HEK Recombinant Cell Line
Description:
PrecisION hNav1.1-HEK Recombinant Cell Line
Trade Name:
Upstate (Millipore)
Qty/Pk:
2 mL
Product Overview:
Recombinant HEK293 cell line expressing the human Nav 1.1 (type I voltage-gated sodium channel alpha subunit).
Format:
2 x 1 ml aliquots containing 1.94 x 106cells/ml in 10% DMSO at passage 11.
Format:
2 x 1 ml aliquots containing 1.94 x 106cells/ml in 10% DMSO at passage 11.
Background Information:
The voltage gated sodium channel isoform Nav1.1 is involved with axon potential initiation and repetitive neuronal firing as well as the contraction-excitation coupling of cardiac myocytes. Nav1.1 is the target of antiepileptic drugs and the probable target causing side effects of centrally active local anaesthetics. It is detected cardiac myocytes as well as in the cell bodies of central neurons.
Application Notes:
HEK293 cells stably expressing hNav1.1 were analysed by patch-clamp and IonWorks™. Typically, >80% of cells sealed with >90% of cells expressing giving mean peak currents of 3.18 nA (n=303). IC50 values obtained for the known hNav1.1 inhibitors were 5.9 nM for tetrodotoxin and for lamotrigine (Lamictal) were 950 μM, 377 μM, and 128 μM at VH of -120 mV, -90 mV & -70 mV respectively.
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.
Entrez Gene Summary:
The vertebrate sodium channel is a voltage-gated ion channel essential for the generation and propagation of action potentials, chiefly in nerve and muscle. Voltage-sensitive sodium channels are heteromeric complexes consisting of a large central pore-forming glycosylated alpha subunit and 2 smaller auxiliary beta subunits. Functional studies have indicated that the transmembrane alpha subunit of the brain sodium channels is sufficient for expression of functional sodium channels (Goldin et al., 1986 [PubMed 2429308]; Isom, 2002 [PubMed 11779698]).[supplied by OMIM]
UniProt Summary:
FUNCTION: SwissProt: P35498 # 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.
SIZE: 2009 amino acids; 228972 Da
SUBUNIT: The sodium channel consists of a large polypeptide and 2- 3 smaller ones. This sequence represents a large polypeptide.
SUBCELLULAR LOCATION: Membrane; Multi-pass membrane protein.
DOMAIN: SwissProt: P35498 The sequence contains 4 internal repeats, each with 5 hydrophobic segments (S1,S2,S3,S5,S6) and one positively charged segment (S4). Segments S4 are probably the voltage-sensors and are characterized by a series of positively charged amino acids at every third position.
DISEASE: SwissProt: P35498 # Defects in SCN1A are the cause of generalized epilepsy with febrile seizures plus type 2 (GEFS+2) [MIM:604233]. This autosomal dominant disorder is characterized by febrile seizures in children and afebrile seizures in adults. Penetrance is incomplete and a large intrafamilial variability of the phenotype is observed. & Defects in SCN1A are a cause of severe myoclonic epilepsy in infancy (SMEI) [MIM:607208]; also called Dravet syndrome. SMEI is a severe form of generalized epilepsy with febrile seizures. It is a rare disorder characterized by normal development before onset, seizures beginning in the first year of life in the form of generalized or unilateral febrile clonic seizures, secondary appearance of myoclonic seizures, and occasionally partial seizures. It is associated with ataxia, slowed psychomotor development, and mental decline. & Defects in SCN1A are the cause of familial hemiplegic migraine 3 (FHM3) [MIM:609634]. FHM3 is an autosomal dominant severe subtype of migraine with aura characterized by some degree of hemiparesis during the attacks. The episodes are associated with variable features of nausea, vomiting, photophobia and phonophobia. Age at onset ranges from 6 to 15 years. Some patients may manifest seizures during infancy. & Defects in SCN1A are the cause of familial febrile convulsions 3 (FEB3) [MIM:604403]; also known as familial febrile seizures 3. Febrile convulsions affect 5-12% of infants and children up to 6 years of age. There is epidemiological evidence that febrile seizures are associated with subsequent afebrile and unprovoked seizures in 2% to 7% of patients. Inheritance pattern is autosomal dominant.
SIMILARITY: Belongs to the sodium channel family. & Contains 1 IQ domain.
SIZE: 2009 amino acids; 228972 Da
SUBUNIT: The sodium channel consists of a large polypeptide and 2- 3 smaller ones. This sequence represents a large polypeptide.
SUBCELLULAR LOCATION: Membrane; Multi-pass membrane protein.
DOMAIN: SwissProt: P35498 The sequence contains 4 internal repeats, each with 5 hydrophobic segments (S1,S2,S3,S5,S6) and one positively charged segment (S4). Segments S4 are probably the voltage-sensors and are characterized by a series of positively charged amino acids at every third position.
DISEASE: SwissProt: P35498 # Defects in SCN1A are the cause of generalized epilepsy with febrile seizures plus type 2 (GEFS+2) [MIM:604233]. This autosomal dominant disorder is characterized by febrile seizures in children and afebrile seizures in adults. Penetrance is incomplete and a large intrafamilial variability of the phenotype is observed. & Defects in SCN1A are a cause of severe myoclonic epilepsy in infancy (SMEI) [MIM:607208]; also called Dravet syndrome. SMEI is a severe form of generalized epilepsy with febrile seizures. It is a rare disorder characterized by normal development before onset, seizures beginning in the first year of life in the form of generalized or unilateral febrile clonic seizures, secondary appearance of myoclonic seizures, and occasionally partial seizures. It is associated with ataxia, slowed psychomotor development, and mental decline. & Defects in SCN1A are the cause of familial hemiplegic migraine 3 (FHM3) [MIM:609634]. FHM3 is an autosomal dominant severe subtype of migraine with aura characterized by some degree of hemiparesis during the attacks. The episodes are associated with variable features of nausea, vomiting, photophobia and phonophobia. Age at onset ranges from 6 to 15 years. Some patients may manifest seizures during infancy. & Defects in SCN1A are the cause of familial febrile convulsions 3 (FEB3) [MIM:604403]; also known as familial febrile seizures 3. Febrile convulsions affect 5-12% of infants and children up to 6 years of age. There is epidemiological evidence that febrile seizures are associated with subsequent afebrile and unprovoked seizures in 2% to 7% of patients. Inheritance pattern is autosomal dominant.
SIMILARITY: Belongs to the sodium channel family. & Contains 1 IQ domain.
Species:
Human
Quality Assurance:
Mycoplasma Testing:
The cell line has been screened to confirm the absence of Mycoplasma species
The cell line has been screened to confirm the absence of Mycoplasma species
Cell Type:
HEK
Cell Line Type:
Ion Channel Cell Lines
Components:
Pack contains 2 vials of mycoplasma-free cells, 1 ml per vial
Brand Family:
Upstate
Host Cells:
293 cell line
Presentation:
2 x 1 ml aliquots
Protein Target:
hNav1.1
UniProt Number:
Target Sub-Family:
Sodium
Packaging:
2 x 1 ml aliquots
Gene Symbol:
- SCN1A
- SCN1
- HBSCI
- FEB3
- NAC1
- SMEI
- Nav1.1
- GEFSP2
Protein or Enzyme Type:
Ion Channels
Product Name:
PrecisION hNav1.1-HEK Recombinant Cell Line
Entrez Gene Number:
Alternate Names:
- SCN1A
- SCN1
- HBSCI
- FEB3
- NAC1
- SMEI
- Nav1.1
- GEFSP2