产品说明
一般描述
Dysferlin is a transmembrane protein, that belongs to the ferlin-1 family of proteins including myoferlin and otoferlin. It is homologous to the C. elegans fer-1 protein. Dysferlin is expressed in kidney cells, skeletal and cardiac muscle.
Dysferlin is encoded by the gene mapped to human chromosome 2p13.2
特异性
Anti-Dysferlin (N-terminal region) specifically recognizes human and mouse Dysferlin.
免疫原
synthetic peptide corresponding to a sequence in the N-terminal region of human dysferlin (GeneID: 8291), conjugated to KLH.
应用
Anti-Dysferlin (N-terminal region) antibody produced in rabbit has been used in:
- immunoblotting
- immunoprecipitation
- immunofluorescence
生化/生理作用
Dysferlin is implicated in membrane fusion events. It plays a role in membrane repair processes, such as the ability to reseal the sarcolemma upon muscle injury. Dysferlin localization in the membrane and trafficking is impaired by mutations in caveolin-1 and -3. Mutations in the dysferlin gene are associated with limb-girdle muscle dystrophy type 2B (LGMD2B), distal anterior compartment myopathy and related Miyoshi myopathy.
外形
solution in 0.01 M phosphate buffered saline, pH 7.4, containing 15 mM sodium azide.
储存及稳定性
For continuous use, store at 2-8 ℃ for up to one month. For extended storage, freeze in working aliquots. Repeated freezing and thawing, or storage in “frost-free” freezers,is not recommended. If slight turbidity occurs upon prolonged storage, clarify the solution by centrifugation before use. Working dilutions should be discarded if not used within 12 hours.
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.
基本信息
| NACRES | NA.41 |
产品性质
| 生物来源 | rabbit |
| 质量水平 | 200 |
| 偶联物 | unconjugated |
| 抗体形式 | affinity isolated antibody |
| antibody product type | primary antibodies |
| 克隆 | polyclonal |
| 形式 | buffered aqueous solution |
| 分子量 | ~250 kDa |
| species reactivity | human, mouse |
| 增强验证 | recombinant expression Learn more about Antibody Enhanced Validation |
| 浓度 | ~1.5 mg/mL |
| technique(s) | immunocytochemistry: 7-14 μg/mL using differentiated C2C12 myoblasts immunoprecipitation (IP): 5-10 μg using HEK-293T cells over-expressing human dysferlin western blot: 0.5-1.0 μg/mL using HEK-293T cells over-expressing human dysferlin |
| UniProt登记号 | O75923 |
| 运输 | dry ice |
| 储存温度 | −20℃ |
| Gene Information | human ... DYSF(8291) mouse ... Dysf(26903) |
安全信息
| 储存分类代码 | 12 - Non Combustible Liquids |
| 闪点(F) | Not applicable |
| 闪点(C) | Not applicable |
