Anti-Dysferlin 抗体 (ab15108)
Key features and details
- Rabbit polyclonal to Dysferlin
- Suitable for: WB, IHC-P
- Reacts with: Human
- Isotype: IgG
リコンビナント抗体で、ロット間での高い再現性を実現
- 異なるロット間での安定した再現性
- 容易なスケールアップ
- 評価試験による特異性の確認済み
- 倫理基準に準拠 - アニマル・フリーの生産
製品の概要
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製品名
Anti-Dysferlin antibody
Dysferlin 一次抗体 製品一覧 -
製品の詳細
Rabbit polyclonal to Dysferlin -
由来種
Rabbit -
アプリケーション
適用あり: WB, IHC-Pmore details -
種交差性
交差種: Human
交差が予測される動物種: Mouse, Dog
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免疫原
Synthetic peptide within Human Dysferlin aa 1950-2050 (C terminal). The exact sequence is proprietary.
Database link: O75923 -
特記事項
This product is FOR RESEARCH USE ONLY. For commercial use, please contact partnerships@abcam.com.
The Life Science industry has been in the grips of a reproducibility crisis for a number of years. Abcam is leading the way in addressing this with our range of recombinant monoclonal antibodies and knockout edited cell lines for gold-standard validation. Please check that this product meets your needs before purchasing.
If you have any questions, special requirements or concerns, please send us an inquiry and/or contact our Support team ahead of purchase. Recommended alternatives for this product can be found below, along with publications, customer reviews and Q&As
製品の特性
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製品の状態
Liquid -
保存方法
Shipped at 4°C. Upon delivery aliquot and store at -20°C. Avoid freeze / thaw cycles. -
バッファー
pH: 7.60
Preservative: 0.1% Sodium azide
Constituents: PBS, 1% BSA -
Concentration information loading... -
精製度
Immunogen affinity purified -
ポリ/モノ
ポリクローナル -
アイソタイプ
IgG -
研究分野
関連製品
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Compatible Secondaries
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Isotype control
アプリケーション
The Abpromise guarantee
Abpromise保証は、 次のテスト済みアプリケーションにおけるab15108の使用に適用されます
アプリケーションノートには、推奨の開始希釈率がありますが、適切な希釈率につきましてはご検討ください。
| アプリケーション | Abreviews | 特記事項 |
|---|---|---|
| WB | (2) |
Use a concentration of 1 µg/ml. Predicted molecular weight: 231 kDa.
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| IHC-P |
1/50.
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| 特記事項 |
|---|
|
WB
Use a concentration of 1 µg/ml. Predicted molecular weight: 231 kDa. |
|
IHC-P
1/50. |
ターゲット情報
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機能
Key calcium ion sensor involved in the Ca(2+)-triggered synaptic vesicle-plasma membrane fusion. Plays a role in the sarcolemma repair mechanism of both skeletal muscle and cardiomyocytes that permits rapid resealing of membranes disrupted by mechanical stress. -
組織特異性
Expressed in skeletal muscle, myoblast, myotube and in the syncytiotrophoblast (STB) of the placenta (at protein level). Highly expressed in skeletal muscle. Also found in heart, brain, spleen, intestine, placenta and at lower levels in liver, lung, kidney and pancreas. -
関連疾患
Defects in DYSF are the cause of limb-girdle muscular dystrophy type 2B (LGMD2B) [MIM:253601]. LGMD2B is an autosomal recessive degenerative myopathy characterized by weakness and atrophy starting in the proximal pelvifemoral muscles, with onset in the late teens or later, massive elevation of serum creatine kinase levels and slow progression. Scapular muscle involvement is minor and not present at onset. Upper limb girdle involvement follows some years after the onset in lower limbs.
Defects in DYSF are the cause of Miyoshi muscular dystrophy type (MMD1) [MIM:254130]. MMD1 is a late-onset muscular dystrophy involving the distal lower limb musculature. It is characterized by weakness that initially affects the gastrocnemius muscle during early adulthood. Otherwise the phenotype overlaps with LGMD2B, especially in age at onset and creatine kinase elevation.
Defects in DYSF are the cause of distal myopathy with anterior tibial onset (DMAT) [MIM:606768]. Onset of the disorder is between 14 and 28 years of age and the anterior tibial muscles are the first muscle group to be involved. Inheritance is autosomal recessive. -
配列類似性
Belongs to the ferlin family.
Contains 5 C2 domains. -
発生段階
Expression in limb tissue from 5-6 weeks embryos; persists throughout development. -
ドメイン
The C2 domain 1 associates with lipid membranes in a calcium-dependent manner. -
細胞内局在
Cell membrane > sarcolemma. Cytoplasmic vesicle membrane. Colocalizes, during muscle differentiation, with BIN1 in the T-tubule system of myotubules and at the site of contact between two myotubes or a myoblast and a myotube. Wounding of myotubes led to its focal enrichment to the site of injury and to its relocalization in a Ca(2+)-dependent manner toward the plasma membrane. Colocalizes with AHNAK, AHNAK2 and PARVB at the sarcolemma of skeletal muscle. Detected on the apical plasma membrane of the syncytiotrophoblast. Reaches the plasmma membrane through a caveolin-independent mechanism. Retained by caveolin at the plasmma membrane (By similarity). Colocalizes, during muscle differentiation, with CACNA1S in the T-tubule system of myotubules (By similarity). Accumulates and colocalizes with fusion vesicles at the sarcolemma disruption sites. - Information by UniProt
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参照データベース
- Entrez Gene: 483121 Dog
- Entrez Gene: 8291 Human
- Entrez Gene: 26903 Mouse
- Omim: 603009 Human
- SwissProt: O75923 Human
- SwissProt: Q9ESD7 Mouse
- Unigene: 252180 Human
- Unigene: 220982 Mouse
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別名
- DMAT antibody
- DYSF antibody
- DYSF_HUMAN antibody
see all
画像
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Immunohistochemistry (Formalin/PFA-fixed paraffin-embedded sections) - Anti-Dysferlin antibody (ab15108)
ab15108 staining Dysferlin in human skeletal muscle by Immunohistochemistry (FFPE-sections).
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Western blot - Anti-Dysferlin antibody (ab15108)Anti-Dysferlin antibody (ab15108) at 1 µg/ml + Human skeletal muscle tissue lysate - total protein (ab29330) at 10 µg
Secondary
Goat polyclonal to Rabbit IgG - H&L - Pre-Adsorbed (HRP) at 1/3000 dilution
Predicted band size: 231 kDa
Observed band size: 231 kDa
Additional bands at: 240 kDa (possible post-translational modification)
プロトコール
データシートおよび資料
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SDS download
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Datasheet download
参考文献 (4)
ab15108 は 4 報の論文で使用されています。
- Whish S et al. The inner CSF-brain barrier: developmentally controlled access to the brain via intercellular junctions. Front Neurosci 9:16 (2015). IHC . PubMed: 25729345
- Guo LT et al. Evaluation of commercial dysferlin antibodies on canine, mouse and human skeletal muscle. Neuromuscul Disord : (2010). WB ; Human, Mouse, Dog . PubMed: 20817457
- Peter AK et al. Myogenic Akt signaling upregulates the utrophin-glycoprotein complex and promotes sarcolemma stability in muscular dystrophy. Hum Mol Genet 18:318-27 (2009). PubMed: 18986978
- Klinge L et al. From T-tubule to sarcolemma: damage-induced dysferlin translocation in early myogenesis. FASEB J 21:1768-76 (2007). PubMed: 17363620