KCNC1 Antibody

Code CSB-PA003120
Size US$100
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  • Western Blot analysis of K562 cells using KV3.1 Polyclonal Antibody
  • Western Blot analysis of K562 cells using KV3.1 Polyclonal Antibody
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Product Details

Uniprot No.
Target Names
KCNC1
Alternative Names
KCNC1; Potassium voltage-gated channel subfamily C member 1; NGK2; Voltage-gated potassium channel subunit Kv3.1; Voltage-gated potassium channel subunit Kv4
Raised in
Rabbit
Species Reactivity
Human,Mouse,Rat
Immunogen
Synthesized peptide derived from the Internal region of Human KV3.1.
Immunogen Species
Homo sapiens (Human)
Conjugate
Non-conjugated
Isotype
IgG
Purification Method
The antibody was affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogen.
Concentration
It differs from different batches. Please contact us to confirm it.
Buffer
Liquid in PBS containing 50% glycerol, 0.5% BSA and 0.02% sodium azide.
Form
Liquid
Tested Applications
WB, IHC, ELISA
Recommended Dilution
Application Recommended Dilution
WB 1:500-1:2000
IHC 1:100-1:300
ELISA 1:5000
Troubleshooting and FAQs
Storage
Upon receipt, store at -20°C or -80°C. Avoid repeated freeze.
Lead Time
Basically, we can dispatch the products out in 1-3 working days after receiving your orders. Delivery time maybe differs from different purchasing way or location, please kindly consult your local distributors for specific delivery time.

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Target Background

Function
Voltage-gated potassium channel that plays an important role in the rapid repolarization of fast-firing brain neurons. The channel opens in response to the voltage difference across the membrane, forming a potassium-selective channel through which potassium ions pass in accordance with their electrochemical gradient. Can form functional homotetrameric channels and heterotetrameric channels that contain variable proportions of KCNC2, and possibly other family members as well. Contributes to fire sustained trains of very brief action potentials at high frequency in pallidal neurons.
Gene References into Functions
  1. A recurrent de novo mutation in KCNC1 (c.959G>A, p.Arg320His) has been identified recently as one of the important genetic causes of progress myoclonic epilepsy. This recurrent mutation in KCNC1 was identified in the two brothers who showed characteristic features of myoclonus epilepsy and ataxia due to potassium channel mutation (MEAK). The asymptomatic mother was suspected as being mosaic for this mutation. PMID: 29428275
  2. KNCN1 p.R320H mutation causes MEAK syndrome. PMID: 28380698
  3. A nonsense variant in KCNC1 gene was identified in three family members with intellectual disability without seizures. PMID: 28145425
  4. reviews the phenotype/genotype of progressive myoclonus epilepsy and ataxia due to potassium channel mutation (MEAK)associated with KCNC1 mutations [review] PMID: 27629860
  5. KCNC1 produces a resurgent current during repolarization, ensuring enough repolarizing power to terminate each action potential. The current results from a combination of steep voltage-dependent gating kinetics and ultra-fast voltage-sensor relaxation. PMID: 26673941
  6. A recurrent KCNC1 de novo mutation, c.959G>A (p.Arg320His), is a new major cause for progressive myoclonus epilepsy. It has a dominant-negative loss-of-function effect. PMID: 25401298
  7. Findings show a decrease in Kv3.1b channel protein in schizophrenia neocortex, a deficit that is restored by antipsychotic drugs PMID: 23628987
  8. Describes localization in mouse brain of two isoforms - the longer is called b and the shorter is called a. PMID: 12091563
  9. Describes two rat isoforms of Kv3.1, alpha is the longer one and beta is the shorter one PMID: 1432046
  10. KChIP4a suppresses A-type Kv4 current via ER retention and enhancement of Kv4 closed-state inactivation. PMID: 23576435
  11. Although all KV3 subunit transcripts are significantly expressed at embryonic age in whole mouse brain extracts, only KV3.1, KV3.2 and KV3.4 subunit transgenic proteins are present. PMID: 21912965
  12. demonstrated that glycosylation was necessary for both DPP10 trafficking to the cell surface and functional interaction with Kv4 channels PMID: 22387313
  13. Kv3.1 channels are transported into axons by binding to kinesin I. PMID: 21106837

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Involvement in disease
Epilepsy, progressive myoclonic 7 (EPM7)
Subcellular Location
Cell membrane; Multi-pass membrane protein. Cell projection, axon. Cell junction, synapse, presynaptic cell membrane.
Protein Families
Potassium channel family, C (Shaw) (TC 1.A.1.2) subfamily, Kv3.1/KCNC1 sub-subfamily
Database Links

HGNC: 6233

OMIM: 176258

KEGG: hsa:3746

STRING: 9606.ENSP00000265969

UniGene: Hs.552896

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