Anti-Nance-Horan Syndrome Protein 抗体 (ab122177)
Key features and details
- Rabbit polyclonal to Nance-Horan Syndrome Protein
- Suitable for: ICC/IF, IHC-P
- Reacts with: Human
- Isotype: IgG
製品の概要
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製品名
Anti-Nance-Horan Syndrome Protein antibody -
製品の詳細
Rabbit polyclonal to Nance-Horan Syndrome Protein -
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: Human -
免疫原
antigen sequence, corresponding to amino acids 1139-1281 of Human Nance-Horan Syndrome Protein.
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ポジティブ・コントロール
- Human liver tissue.
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特記事項
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.
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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 and store at -20°C. Avoid freeze / thaw cycles. -
バッファー
pH: 7.20
Preservative: 0.02% Sodium azide
Constituents: 40% Glycerol (glycerin, glycerine), 59% PBS -
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ab122177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 1 - 4 µg/ml.
Recommend PFA Fixation and Triton X-100 treatment |
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IHC-P |
1/20 - 1/50. 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 1 - 4 µg/ml. Recommend PFA Fixation and Triton X-100 treatment |
IHC-P
1/20 - 1/50. 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
Unknown. May have a key functions in the regulation of eye, tooth, brain and craniofacial development. -
組織特異性
Detected at low levels in all tissues analyzed. Detected in fetal and adult brain, lens, retina, retinal pigment epithelium, placenta, lymphocytes and fibroblasts. Levels in retinal pigment epithelium, placenta, lymphocytes, and fibroblasts are very low. Expressed also in kidney, lung and thymus. -
関連疾患
Defects in NHS are the cause of Nance-Horan syndrome (NHS) [MIM:302350]; also known as cataract-dental syndrome. NHS is a rare X-linked disorder characterized by congenital cataracts, dental anomalies, dysmorphic features, and, in some cases, mental retardation. Distinctive dental anomalies are seen in affected males, including supernumerary incisors and crown shaped permanent teeth. Characteristic facial features are anteverted pinnae, long face, and prominent nasal bridge and nose. Carrier females display milder variable symptoms of disease with lens opacities often involving the posterior Y sutures, and on occasion dental anomalies and the characteristic facial features described.
Defects in NHS are the cause of cataract congenital X-linked (CXN) [MIM:302200]. A X-linked form of cataract, manifesting as a congenital nuclear opacity with severe visual impairment in affected males. Heterozygous females have suture cataracts and only slight reduction in vision. Note=Caused by copy number variations predicted to result in altered transcriptional regulation of the NHS gene: a 0.8 Mb segmental duplication-triplication encompassing the NHS, SCML1 and RAI2 genes, and an 4.8 kb intragenic deletion in NHS intron 1. -
配列類似性
Belongs to the NHS family. -
細胞内局在
Nucleus. - Information by UniProt
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参照データベース
- Entrez Gene: 4810 Human
- Omim: 300457 Human
- SwissProt: Q6T4R5 Human
- Unigene: 201623 Human
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別名
- Congenital cataracts and dental anomalies protein antibody
- CXN antibody
- Nance-Horan syndrome protein antibody
see all
画像
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Immunofluorescent staining of Human cell line U-2 OS shows positivity in nucleus but not nucleoli. Recommended concentration of ab122177 1-4 µg/ml. Cells treated with PFA/Triton X-100.
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ab122177 at 1/35 dilution staining Nance-Horan Syndrome Protein in Paraffin-embedded Human Liver tissue by Immunohistochemistry.
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 (0)
ab122177 は論文での使用が確認できていません。