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Monoclonal Anti-CTNS antibody produced in mouse
产品编号: | 3143738 |
规格: | clone 5G6, purified immunoglobulin, buffered aqueous solution |
包装规格: | 100 μG |
产品类别: | 进口试剂 |
品牌: | Sigma-Aldrich |
优惠价: | 立即咨询 |
产品别名
Monoclonal Anti-CTNS antibody produced in mouse
Anti-cystinosis, nephropathic
Anti-PQLC4
Anti-CTNSLSB
基本信息
NACRES | NA.41 |
General description【一般描述】 | The cystinosin, lysosomal cystine transporter (CTNS) gene with 12 exons, spanning 23kb on genomic DNA, is mapped to human chromosome 17p13.3. The cystinosin protein contains seven-transmembrane-domains and is mainly localized in lysosomes. This gene encodes a seven-transmembrane domain protein that functions to transport cystine out of lysosomes. Its activity is driven by the H+ electrochemical gradient of the lysosomal membrane. Mutations in this gene cause cystinosis, a lysosomal storage disorder. Alternative splicing results in multiple transcript variants. (provided by RefSeq) |
Immunogen【免疫原】 | CTNS (NP_004928, 1 a.a. ~ 100 a.a) partial recombinant protein with GST tag. MW of the GST tag alone is 26 KDa. Sequence MIRNWLTIFILFPLKLVEKCESSVSLTVPPVVKLENGSSTNVSLTLRPPLNATLVITFEITFRSKNITILELPDEVVVPPGVTNSSFQVTSQNVGQLTVY |
Biochem/physiol Actions【生化/生理作用】 | Cystinosin (CTNS) plays a vital role in migrating cystine out of lysosomes. Mutations in CTNS gene is associated with the development of cystinosis, an autosomal recessive disease characterized by intralysosomal accumulation of cystine. |
Physical form【外形】 | Solution in phosphate buffered saline, pH 7.4 |
Legal Information【法律信息】 | GenBank is a registered trademark of United States Department of Health and Human Services |
产品性质
Quality Level【质量水平】 | 100 |
biological source【生物来源】 | mouse |
conjugate【偶联物】 | unconjugated |
antibody form【抗体形式】 | purified immunoglobulin |
antibody product type | primary antibodies |
clone【克隆】 | 5G6, monoclonal |
form【形式】 | buffered aqueous solution |
species reactivity | human |
technique(s) | indirect ELISA: suitable western blot: 1-5 μg/mL |
isotype【同位素/亚型】 | IgG2aκ |
GenBank® accession no. | NM_004937 |
UniProt accession no.【UniProt登记号】 | O60931 |
shipped in【运输】 | dry ice |
storage temp.【储存温度】 | −20℃ |
Gene Information | human ... CTNS(1497) |
产品说明
Disclaimer【免责声明】 | Unless otherwise stated in our catalog or other company documentation accompanying the product(s), our products are intended for research use only and are not to be used for any other purpose, which includes but is not limited to, unauthorized commercial uses, in vitro diagnostic uses, ex vivo or in vivo therapeutic uses or any type of consumption or application to humans or animals. |
安全信息
Storage Class Code【储存分类代码】 | 12 - Non Combustible Liquids |
Personal Protective Equipment【个人防护装备】 | Eyeshields, Gloves, multi-purpose combination respirator cartridge (US) |