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- Bioss
- bs-7854P
- 2025年10月16日
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500ug
| 产品编号 | bs-7854P |
| 英文名称 | AHI1 Antibody Blocking Peptide |
| 中文名称 | 白血病相关蛋白AHI1封闭多肽 |
| 英文别名 | 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. |
| 纯化方法 | HPLC |
| 研究领域 | Cell Biology > Cell Cycle > Cell differentiation |
| 亚基 | Part of the tectonic-like complex (also named B9complex). Interacts with MKS1 (By similarity). Interacts withNPHP1. |
| 亚细胞定位 | Cytoplasm, cytoskeleton, cilium basal body(By similarity). Cell junction, adherens junction. |
| 组织特异性 | 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). |
| 相似性 | Contains 1 SH3 domain. Contains 7 WD repeats. |
| 功能 | 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). |
| 保存条件 | Shipped at 4℃. Stored at -20℃ for one year. Avoid repeated freeze/thaw cycles. |
| 注意事项 | This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications. |
| 背景资料 | 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. |
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AHI1 Antibody Blocking Peptide(bs-7854P)-500ug
￥880
