| 產品編號 | bs-7854R |
| 英文名稱 | AHI1 Rabbit pAb |
| 中文名稱 | 白血病相關蛋白AHI1抗體 |
| 別 名 | Abelson helper integration site 1 protein homolog; Abelson helper integration site 1; Abelson helper integration site; AHI 1; AHI-1; Ahi1; AHI1_HUMAN; Contatins SH3 and WD40 domains; JBTS3; Jouberin; ORF1. |
| 研究領域 | 細胞生物 細胞周期蛋白 細胞分化 |
| 抗體來源 | Rabbit |
| 克隆類型 | Polyclonal |
| 克 隆 號 | |
| 交叉反應 | (predicted: Human,Mouse,Rat,Rabbit,Pig,Sheep,Cow,Dog,Horse) |
| 產品應用 | WB=1:500-2000,IHC-P=1:100-500,IHC-F=1:100-500,IF=1:100-500,ELISA=1:5000-10000
not yet tested in other applications. optimal dilutions/concentrations should be determined by the end user. |
| 理論分子量 | 137 kDa |
| 檢測分子量 | |
| 細胞定位 | 細胞漿 細胞膜 |
| 性 狀 | Liquid |
| 濃 度 | 1mg/ml |
| 免 疫 原 | KLH conjugated synthetic peptide derived from human AHI1: 801-900/1196 |
| 亞 型 | IgG |
| 純化方法 | affinity purified by Protein A |
| 緩 沖 液 | 0.01M TBS (pH7.4) with 1% BSA, 0.02% Proclin300 and 50% Glycerol. |
| 保存條件 | Shipped at 4℃. Store at -20℃ for one year. Avoid repeated freeze/thaw cycles. |
| 注意事項 | This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications. |
| PubMed | PubMed |
| 產品介紹 |
Highly expressed in the most primitive normal hematopoietic cells. Expressed in brain, particularly in neurons that give rise to the crossing axons of the corticospinal tract and superior cerebellar peduncles. Expressed in kidney (renal collecting duct cells) (at protein level). Involvement in disease:Defects in AHI1 are the cause of Joubert syndrome type 3 (JBTS3) . JBTS is an autosomal recessive disorder presenting with cerebellar ataxia, oculomotor apraxia, hypotonia, neonatal breathing abnormalities and psychomotor delay. Neuroradiologically, it is characterized by cerebellar vermian hypoplasia/aplasia, thickened and reoriented superior cerebellar peduncles, and an abnormally large interpeduncular fossa, giving the appearance of a molar tooth on transaxial slices (molar tooth sign). Additional variable features include retinal dystrophy and renal disease. JBTS3 shows minimal extra central nervous system involvement and appears not to be associated with renal dysfunction. Function: Component of the tectonic-like complex, a complexlocalized at the transition zone of primary cilia and acting as abarrier that prevents diffusion of transmembrane proteins betweenthe cilia and plasma membranes (By similarity). Subunit: Part of the tectonic-like complex (also named B9complex). Interacts with MKS1 (By similarity). Interacts withNPHP1. Subcellular Location: Cytoplasm, cytoskeleton, cilium basal body(By similarity). Cell junction, adherens junction. Tissue Specificity: Highly expressed in the most primitive normalhematopoietic cells. Expressed in brain, particularly in neuronsthat give rise to the crossing axons of the corticospinal tract andsuperior cerebellar peduncles. Expressed in kidney (renalcollecting duct cells) (at protein level). DISEASE: Defects in AHI1 are the cause of Joubert syndrome type 3(JBTS3) [MIM:608629]. JBTS is an autosomal recessive disorderpresenting with cerebellar ataxia, oculomotor apraxia, hypotonia,neonatal breathing abnormalities and psychomotor delay.Neuroradiologically, it is characterized by cerebellar vermianhypoplasia/aplasia, thickened and reoriented superior cerebellarpeduncles, and an abnormally large interpeduncular fossa, givingthe appearance of a molar tooth on transaxial slices (molar toothsign). Additional variable features include retinal dystrophy andrenal disease. JBTS3 shows minimal extra central nervous systeminvolvement and appears not to be associated with renaldysfunction. Similarity: Contains 1 SH3 domain. Contains 7 WD repeats. SWISS: Q8N157 Gene ID: 54806 Database links: Entrez Gene: 54806 Human Entrez Gene: 52906 Mouse Omim: 608894 Human SwissProt: Q8N157 Human SwissProt: Q8K3E5 Mouse Unigene: 386684 Human Unigene: 253280 Mouse Unigene: 155144 Rat 腦組織表達 |
| 1、抗體溶解方法 | |
| 2、抗體修復方式 | |
| 3、常用試劑的配制 | |
| 4、免疫組化操作步驟 | |
| 5、免疫組化問題解答 | |
| 6、Western Blotting 操作步驟 | |
| 7、Western Blotting 問題解答 | |
| 8、關于肽鏈的設計 | |
| 9、多肽的溶解與保存 | |
| 10、酶標抗體效價測定程序 | |
