验证数据展示
产品信息
66136-1-PBS targets BBS5 in WB, IHC, IF/ICC, Indirect ELISA applications and shows reactivity with human, mouse samples.
经测试应用 | WB, IHC, IF/ICC, Indirect ELISA Application Description |
经测试反应性 | human, mouse |
免疫原 | BBS5 fusion protein Ag6844 种属同源性预测 |
宿主/亚型 | Mouse / IgG1 |
抗体类别 | Monoclonal |
产品类型 | Antibody |
全称 | Bardet-Biedl syndrome 5 |
别名 | Bardet-Biedl syndrome 5 protein, Bardet Biedl syndrome 5 |
计算分子量 | 39 kDa |
观测分子量 | 39 kDa |
GenBank蛋白编号 | BC044593 |
基因名称 | BBS5 |
Gene ID (NCBI) | 129880 |
RRID | AB_2881535 |
偶联类型 | Unconjugated |
形式 | Liquid |
纯化方式 | Protein A purification |
UNIPROT ID | Q8N3I7 |
储存缓冲液 | PBS only , pH 7.3 |
储存条件 | Store at -80°C. The product is shipped with ice packs. Upon receipt, store it immediately at -80°C |
背景介绍
BBS5 encodes a protein that has been directly linked to Bardet-Biedl syndrome. Bardet-Biedl syndrome (BBS) is an autosomal recessive condition characterised by rod-cone dystrophy, postaxial polydactyly, central obesity, mental retardation, hypogonadism, and renal dysfunction. Other associated clinical findings in BBS patients include diabetes, hypertension and congenital heart defects. BBS expression varies both within and between families and diagnosis is often difficult. Experimentation in non-human eukaryotes suggests that BBS5 is expressed in ciliated cells and that it is required for the formation of cilia. Alternate transcriptional splice variants have been observed but have not been fully characterized.