Anti-Scn1a 抗体 (ab24820)
Key features and details
- Rabbit polyclonal to Scn1a
- Suitable for: ICC/IF, IHC-P
- Reacts with: Rat, Human
- Isotype: IgG
製品の概要
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製品名
Anti-Scn1a antibody
Scn1a 一次抗体 製品一覧 -
製品の詳細
Rabbit polyclonal to Scn1a -
由来種
Rabbit -
アプリケーション
適用あり: ICC/IF, IHC-Pmore details -
種交差性
交差種: Rat, Human
交差が予測される動物種: Mouse, Rabbit, Zebrafish -
免疫原
Synthetic peptide within Human Scn1a aa 1450-1550. The exact sequence is proprietary.
Database link: P35498 -
特記事項
Ab with sodium azide is stable for 24 months when stored at 2-8 °C.
This product is FOR RESEARCH USE ONLY. For commercial use, please contact partnerships@abcam.com.
The Life Science industry has been in the grips of a reproducibility crisis for a number of years. Abcam is leading the way in addressing this with our range of recombinant monoclonal antibodies and knockout edited cell lines for gold-standard validation. Please check that this product meets your needs before purchasing.
If you have any questions, special requirements or concerns, please send us an inquiry and/or contact our Support team ahead of purchase. Recommended alternatives for this product can be found below, along with publications, customer reviews and Q&As
製品の特性
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製品の状態
Liquid -
保存方法
Shipped at 4°C. Upon delivery aliquot. Store at -20°C. Avoid freeze / thaw cycle. Please see notes section. -
バッファー
pH: 7.60
Preservative: 0.1% Sodium azide
Constituents: PBS, 1% BSA -
Concentration information loading...
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精製度
Immunogen affinity purified -
ポリ/モノ
ポリクローナル -
アイソタイプ
IgG -
研究分野
関連製品
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Compatible Secondaries
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Isotype control
アプリケーション
The Abpromise guarantee
Abpromise保証は、 次のテスト済みアプリケーションにおけるab24820の使用に適用されます
アプリケーションノートには、推奨の開始希釈率がありますが、適切な希釈率につきましてはご検討ください。
アプリケーション | Abreviews | 特記事項 |
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ICC/IF |
Use a concentration of 5 µg/ml.
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IHC-P | (1) |
1/200. Perform heat mediated antigen retrieval with citrate buffer pH 6 before commencing with IHC staining protocol.
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特記事項 |
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ICC/IF
Use a concentration of 5 µg/ml. |
IHC-P
1/200. Perform heat mediated antigen retrieval with citrate buffer pH 6 before commencing with IHC staining protocol. |
ターゲット情報
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機能
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. -
関連疾患
Defects in SCN1A are the cause of generalized epilepsy with febrile seizures plus type 2 (GEFS+2) [MIM:604233]. Generalized epilepsy with febrile seizures-plus refers to a rare autosomal dominant, familial condition with incomplete penetrance and large intrafamilial variability. Patients display febrile seizures persisting sometimes beyond the age of 6 years and/or a variety of afebrile seizure types. GEFS+ is a disease combining febrile seizures, generalized seizures often precipitated by fever at age 6 years or more, and partial seizures, with a variable degree of severity.
Defects in SCN1A are a cause of severe myoclonic epilepsy in infancy (SMEI) [MIM:607208]; also called Dravet syndrome. SMEI is a rare disorder characterized by generalized tonic, clonic, and tonic-clonic seizures that are initially induced by fever and begin during the first year of life. Later, patients also manifest other seizure types, including absence, myoclonic, and simple and complex partial seizures. Psychomotor development delay is observed around the second year of life. SMEI is considered to be the most severe phenotype within the spectrum of generalized epilepsies with febrile seizures-plus.
Defects in SCN1A are a cause of intractable childhood epilepsy with generalized tonic-clonic seizures (ICEGTC) [MIM:607208]. ICEGTC is a disorder characterized by generalized tonic-clonic seizures beginning usually in infancy and induced by fever. Seizures are associated with subsequent mental decline, as well as ataxia or hypotonia. ICEGTC is similar to SMEI, except for the absence of myoclonic seizures.
Defects in SCN1A are the cause of migraine familial hemiplegic type 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. FHM is occasionally associated with other neurologic symptoms such as cerebellar ataxia or epileptic seizures. A unique eye phenotype of elicited repetitive daily blindness has also been reported to be cosegregating with FHM in a single Swiss family.
Defects in SCN1A are the cause of familial febrile convulsions type 3A (FEB3A) [MIM:604403]; also known as familial febrile seizures 3. Febrile convulsions are seizures associated with febrile episodes in childhood without any evidence of intracranial infection or defined pathologic or traumatic cause. It is a common condition, affecting 2-5% of children aged 3 months to 5 years. The majority are simple febrile seizures (generally defined as generalized onset, single seizures with a duration of less than 30 minutes). Complex febrile seizures are characterized by focal onset, duration greater than 30 minutes, and/or more than one seizure in a 24 hour period. The likelihood of developing epilepsy following simple febrile seizures is low. Complex febrile seizures are associated with a moderately increased incidence of epilepsy. -
配列類似性
Belongs to the sodium channel (TC 1.A.1.10) family. Nav1.1/SCN1A subfamily.
Contains 1 IQ domain. -
ドメイン
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. -
細胞内局在
Membrane. - Information by UniProt
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参照データベース
- Entrez Gene: 6323 Human
- Entrez Gene: 20265 Mouse
- Entrez Gene: 100009591 Rabbit
- Entrez Gene: 81574 Rat
- Entrez Gene: 393101 Zebrafish
- Omim: 182389 Human
- SwissProt: P35498 Human
- SwissProt: P04774 Rat
see all -
別名
- brain sodium channel type I antibody
- EIEE6 antibody
- FEB3 antibody
see all
画像
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Staining of human cerebellum with anti-sodium channel antibody. The tissue was boiled in 10mM citrate buffer, pH 6.0 for 10 mins followed by cooling at room temparture for 20 mins. The working dilution for the antibody is 1:200 for 30 min at room temparture.
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ICC/IF image of ab24820 stained PC12 cells. The cells were 100% methanol fixed (5 min) and then incubated in 1%BSA / 10% normal goat serum / 0.3M glycine in 0.1% PBS-Tween for 1h to permeabilise the cells and block non-specific protein-protein interactions. The cells were then incubated with the antibody (ab24820, 5µg/ml) overnight at +4°C. The secondary antibody (green) was Alexa Fluor® 488 goat anti-rabbit IgG (H+L) used at a 1/1000 dilution for 1h. Alexa Fluor® 594 WGA was used to label plasma membranes (red) at a 1/200 dilution for 1h. DAPI was used to stain the cell nuclei (blue) at a concentration of 1.43µM.
プロトコール
データシートおよび資料
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SDS download
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Datasheet download
参考文献 (9)
ab24820 は 9 報の論文で使用されています。
- Scalise S et al. Human iPSC Modeling of Genetic Febrile Seizure Reveals Aberrant Molecular and Physiological Features Underlying an Impaired Neuronal Activity. Biomedicines 10:N/A (2022). PubMed: 35625812
- Okada T et al. Pain induces stable, active microcircuits in the somatosensory cortex that provide a therapeutic target. Sci Adv 7:N/A (2021). PubMed: 33741588
- Wang Z et al. Voltage-Gated Sodium Channels Are Involved in Cognitive Impairments in Parkinson's Disease- like Rats. Neuroscience 418:231-243 (2019). PubMed: 31473280
- Hamilton KA et al. Mice lacking the transcriptional regulator Bhlhe40 have enhanced neuronal excitability and impaired synaptic plasticity in the hippocampus. PLoS One 13:e0196223 (2018). PubMed: 29715265
- Chen YH et al. MDH2 is an RNA binding protein involved in downregulation of sodium channel Scn1a expression under seizure condition. Biochim Biophys Acta 1863:1492-1499 (2017). WB . PubMed: 28433711
- Zhang Y et al. Pharmacological characterization of an antisense knockdown zebrafish model of Dravet syndrome: inhibition of epileptic seizures by the serotonin agonist fenfluramine. PLoS One 10:e0125898 (2015). PubMed: 25965391
- Biet M et al. Prolongation of action potential duration and QT interval during epilepsy linked to increased contribution of neuronal sodium channels to cardiac late Na+ current: potential mechanism for sudden death in epilepsy. Circ Arrhythm Electrophysiol 8:912-20 (2015). PubMed: 26067667
- Richner M et al. MicroRNA-based conversion of human fibroblasts into striatal medium spiny neurons. Nat Protoc 10:1543-55 (2015). PubMed: 26379228
- Sugiura Y et al. Different degrees of loss of function between GEFS+ and SMEI Nav 1.1 missense mutants at the same residue induced by rescuable folding defects. Epilepsia 53:e111-4 (2012). PubMed: 22525008