Anti-Dysferlin 抗体 (ab85802)
Key features and details
- Rabbit polyclonal to Dysferlin
- Suitable for: IHC-P, WB
- 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: IHC-P, WBmore details -
種交差性
交差種: Human
交差が予測される動物種: Orangutan -
免疫原
Synthetic peptide corresponding to Human Dysferlin aa 2050 to the C-terminus (C terminal) conjugated to keyhole limpet haemocyanin.
(Peptide available asab97417) -
ポジティブ・コントロール
- This antibody gave a positive signal in Human skeletal muscle tissue lysate.
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特記事項
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. Store at +4°C short term (1-2 weeks). Upon delivery aliquot. Store at -20°C or -80°C. Avoid freeze / thaw cycle. -
バッファー
pH: 7.40
Preservative: 0.02% Sodium azide
Constituent: PBS
Batches of this product that have a concentration < 1mg/ml may have BSA added as a stabilising agent. If you would like information about the formulation of a specific lot, please contact our scientific support team who will be happy to help. -
Concentration information loading...
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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ab85802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 |
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IHC-P |
Use a concentration of 5 µg/ml. Perform heat mediated antigen retrieval before commencing with IHC staining protocol.
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WB |
Use a concentration of 1 µg/ml. Detects a band of approximately 248 kDa (predicted molecular weight: 237 kDa).
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特記事項 |
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IHC-P
Use a concentration of 5 µg/ml. Perform heat mediated antigen retrieval before commencing with IHC staining protocol. |
WB
Use a concentration of 1 µg/ml. Detects a band of approximately 248 kDa (predicted molecular weight: 237 kDa). |
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: 8291 Human
- Entrez Gene: 100174519 Orangutan
- Omim: 603009 Human
- SwissProt: O75923 Human
- Unigene: 252180 Human
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別名
- DMAT antibody
- DYSF antibody
- DYSF_HUMAN antibody
see all
画像
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Anti-Dysferlin antibody (ab85802) 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
Developed using the ECL technique.
Performed under reducing conditions.
Predicted band size: 237 kDa
Observed band size: 248 kDa why is the actual band size different from the predicted?
Additional bands at: 31 kDa, 44 kDa, 61 kDa. We are unsure as to the identity of these extra bands.
Exposure time: 5 minutes -
IHC image of Dysferlin staining in human skeletal muscle formalin fixed paraffin embedded tissue section, performed on a Leica BondTM system using the standard protocol F. The section was pre-treated using heat mediated antigen retrieval with sodium citrate buffer (pH6, epitope retrieval solution 1) for 20 mins. The section was then incubated with ab85802, 5µg/ml, for 15 mins at room temperature and detected using an HRP conjugated compact polymer system. DAB was used as the chromogen. The section was then counterstained with haematoxylin and mounted with DPX.
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 (0)
ab85802 は論文での使用が確認できていません。