SAB4200388

Anti-Dysferlin (N-terminal region) antibody produced in rabbit

~1.5 mg/mL, affinity isolated antibody

• 別名: Anti-Dysferlin (N-terminal region) antibody produced in rabbit, Anti-DYSF, Anti-Limb girdle muscular dystrophy 2B
• UNSPSCコード: 12352203
• NACRES: NA.41

特性

• 由来生物: rabbit
• 結合体: unconjugated
• 抗体製品の状態: affinity isolated antibody
• 抗体製品タイプ: primary antibodies
• クローン: polyclonal
• 形状: buffered aqueous solution
• 分子量: ~250 kDa
• 化学種の反応性: human, mouse
• 強化検証: recombinant expression; Learn more about Antibody Enhanced Validation
• 濃度: ~1.5 mg/mL
• テクニック: 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アクセッション番号: O75923
• 輸送温度: dry ice
• 保管温度: −20°C
• ターゲットの翻訳後修飾: unmodified
• 遺伝子情報: human ... DYSF(8291) ; mouse ... Dysf(26903)

詳細

詳細

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

特異性

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.

アプリケーション

Anti-Dysferlin (N-terminal region) antibody produced in rabbit has been used in:

• immunoblotting
• immunoprecipitation
• immunofluorescence

生物化学的／生理学的作用

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.

物理的形状

solution in 0.01 M phos­phate buffered saline, pH 7.4, containing 15 mM sodium azide.

保管および安定性

For continuous use, store at 2-8 °C 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.

免責事項

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.

安全性情報

• 保管分類コード: 10 - Combustible liquids
• 引火点(°F): Not applicable
• 引火点(℃): Not applicable