- ￥880
- Bioss
- bs-11512P
- 2025年10月16日
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- 详细信息
- 技术资料
- 规格：
500ug
| 产品编号 | bs-11512P |
| 英文名称 | BBS10 Antibody Blocking Peptide |
| 中文名称 | 巴尔得-别德尔综合征相关蛋白10封闭多肽 |
| 英文别名 | Bardet Biedl syndrome 10 protein; Bardet Biedl syndrome 10 protein homolog; C12orf58; FLJ23560; RGD1560748; BBS10_HUMAN. |
| 纯化方法 | HPLC |
| 亚基 | Component of the BBS/CCT complex composed at least of MKKS, BBS10, BBS12, TCP1, CCT2, CCT3, CCT4, CCT5 AND CCT8. |
| 亚细胞定位 | Cell projection, cilium. Note=Located within the basal body of the primary cilium of differentiating preadipocytes. |
| 相似性 | Belongs to the TCP-1 chaperonin family. |
| 功能 | BBS10 belongs to the TCP-1 chaperonin family. It is a probable molecular chaperone; assist the folding of proteins upon ATP hydrolysis. Defects in BBS10 are the cause of Bardet-Biedl syndrome type 10 (BBS10) [MIM:209900]. Bardet-Biedl syndrome (BBS) is a genetically heterogeneous, autosomal recessive disorder characterized by usually severe pigmentary retinopathy, early onset obesity, polydactyly, hypogenitalism, renal malformation and mental retardation. |
| 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 | Bardet-Biedl syndrome (BBS) is a pleiotropic genetic disorder characterized by obesity, photoreceptor degeneration, polydactyly, hypogenitalism, renal abnormalities, and developmental delay. Other associated clinical findings in BBS patients include diabetes, hypertension, and congenital heart defects. BBS genes map to multiple loci and encode fourteen proteins, BBS1-BBS14. Many BBS genes encode basal body or cilia proteins, suggesting that BBS is a ciliary dysfunction disorder. BBS10 (Bardet-Biedl syndrome 10), also known as chromosome 12 open reading fame 58, C12orf58 or FLJ23560, is a novel 723 amino acid protein belonging to the TCP-1 chaperonin family. BBS10 localizes to the basal body of primary cilium and assists in protein folding upon ATP hydrolysis. Inhibition of BBS10 has been found to impair ciliogenesis, activate the glycogen synthase kinase 3 pathway and cause peroxisome proliferator-activated receptor nuclear accumulation. The gene encoding BBS10 contains two exons and maps to human chromosome 12q21.2. |
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BBS10 Antibody Blocking Peptide(bs-11512P)-500ug
￥880
