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Anti-Dysferlin (N-terminal region) antibody produced in rabbit
产品别名
Anti-Dysferlin (N-terminal region) antibody produced in rabbit
Anti-Limb girdle muscular dystrophy 2B
Anti-DYSF
基本信息
NACRES | NA.41 |
General description【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 |
Specificity【特异性】 | Anti-Dysferlin (N-terminal region) specifically recognizes human and mouse Dysferlin. |
Immunogen【免疫原】 | synthetic peptide corresponding to a sequence in the N-terminal region of human dysferlin (GeneID: 8291), conjugated to KLH. |
Application【应用】 | Anti-Dysferlin (N-terminal region) antibody produced in rabbit has been used in:
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Biochem/physiol Actions【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. |
Physical form【外形】 | solution in 0.01 M phosphate buffered saline, pH 7.4, containing 15 mM sodium azide. |
产品性质
biological source【生物来源】 | rabbit |
Quality Level【质量水平】 | 200 |
conjugate【偶联物】 | unconjugated |
antibody form【抗体形式】 | affinity isolated antibody |
antibody product type | primary antibodies |
clone【克隆】 | polyclonal |
form【形式】 | buffered aqueous solution |
mol wt【分子量】 | ~250 kDa |
species reactivity | human, mouse |
enhanced validation【增强验证】 | recombinant expression Learn more about Antibody Enhanced Validation |
concentration【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 accession no.【UniProt登记号】 | O75923 |
shipped in【运输】 | dry ice |
storage temp.【储存温度】 | −20℃ |
Gene Information | human ... DYSF(8291) mouse ... Dysf(26903) |
产品说明
Storage and Stability【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. |
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 |