Code
OSP00113W
$240USD
ID Tag
Rb1352-140310-WS
Immunogen
A synthetic peptide from c-terminal region of human KCNC3 (Kv3.3) conjugated to an immunogenic carrier protein was used as the antigen.
Accession
Also known
Potassium voltage-gated channel subfamily C member 3, Voltage-gated potassium channel subunit Kv3.3, KSHIIID
Target
Function: This protein mediates the voltage-dependent potassium ion permeability of excitable membranes. Assuming opened or closed conformations in response to the voltage difference across the membrane, the protein forms a potassium-selective channel through which potassium ions may pass in accordance with their electrochemical gradient.
Defects in KCNC3 are the cause of spinocerebellar ataxia type 13 (SCA13). Spinocerebellar ataxia is a clinically and genetically heterogeneous group of cerebellar disorders. Patients show progressive incoordination of gait and often poor coordination of hands, speech and eye movements, due to degeneration of the cerebellum with variable involvement of the brainstem and spinal cord. SCA13 is an autosomal dominant cerebellar ataxia (ADCA) characterized by slow progression and variable age at onset, ranging from childhood to late adulthood. Mental retardation can be present in some patients.
Tissue specificity: The KV3.3B isoform is highly enriched in the brain, particularly in the cerebellum, where its expression is confined to Purkinje cells and deep cerebellar nuclei. Isoform KV3.3A is not expressed in cerebellum.
Subcellular location: Membrane; Multi-pass membrane protein
Storage
Maintain the lyophilised/reconstituted antibodies frozen at -20°C for long term storage and refrigerated at 2-8°C for a shorter term. When reconstituting, glycerol (1:1) may be added for an additional stability. Avoid freeze and thaw cycles.
Expiry Date
12 months after reconstitution
Shipping
This item will be shipped to you at ambient temperature in a lyophilised form.
References
1. Rae J.L, et al. Exp. Eye Res. 70:339-348(2000)
2. Grimwood J, et al. Nature 428:529-535(2004)
3. Waters M.F, et al. Nat. Genet. 38:447-451(2006)
Limitation
For research use only
Code
OSP00113W
$240USD
Unit size
100 µl
Conjugate
Unconjugated antibody
Host
NZ white rabbit
Purity
Whole serum
Clonality
Polyclonal
Isotype
Polyclonal, whole serum
Applications
IHC, WB. A dilution of 1 : 200 to 1 : 2000 is recommended. The optimal dilution should be determined by the end user. Not yet tested in other applications.
Specificity
Specific for KCNC3.
Spcs X-react.
Human, rat, mouse. Other species not yet tested.
Format
Lyophilised
Reconstitution
Reconstitute in 100 µl of sterile water. Centrifuge to remove any insoluble material.