Anti-Dysferlin 抗体 [JAI-1-49-3] (ab124684)
Key features and details
- Produced recombinantly (animal-free) for high batch-to-batch consistency and long term security of supply
- Rabbit monoclonal [JAI-1-49-3] to Dysferlin
- Suitable for: ICC/IF, IHC-Fr, WB, IHC-P
- Reacts with: Mouse, Human
Related conjugates and formulations
製品の概要
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製品名
Anti-Dysferlin antibody [JAI-1-49-3]
Dysferlin 一次抗体 製品一覧 -
製品の詳細
Rabbit monoclonal [JAI-1-49-3] to Dysferlin -
由来種
Rabbit -
アプリケーション
適用あり: ICC/IF, IHC-Fr, WB, IHC-Pmore details
適用なし: Flow Cyt -
種交差性
交差種: Mouse, Human -
免疫原
Synthetic peptide within Human Dysferlin aa 100-200. The exact sequence is proprietary.
Database link: O75923 -
ポジティブ・コントロール
- WB: Human and mouse skeletal muscle tissue lysates. IHC-P: Human skeletal muscle tissue. IHC-Fr: Human and mouse skeletal muscle tissues. ICC/IF: A673 cells.
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特記事項
This antibody was made in collaboration with the Jain Foundation whose goal is to hasten EVERY avenue that may lead to the cure for LGMD2B/Miyoshi.
This product is a recombinant monoclonal antibody, which offers several advantages including:
- - High batch-to-batch consistency and reproducibility
- - Improved sensitivity and specificity
- - Long-term security of supply
- - Animal-free production
Our RabMAb® technology is a patented hybridoma-based technology for making rabbit monoclonal antibodies. For details on our patents, please refer to RabMAb® patents.
Rat: We have preliminary internal testing data to indicate this antibody may not react with this species. Please contact us for more information.
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. Avoid freeze / thaw cycle. -
解離定数(KD 値)
KD = 7.20 x 10 -11 M Learn more about KD -
バッファー
pH: 7.20
Preservative: 0.01% Sodium azide
Constituents: 40% Glycerol (glycerin, glycerine), 0.05% BSA, 59% PBS -
Concentration information loading...
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精製度
Protein A purified -
ポリ/モノ
モノクローナル -
クローン名
JAI-1-49-3 -
アイソタイプ
IgG -
研究分野
関連製品
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Alternative Versions
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Compatible Secondaries
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Isotype control
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Positive Controls
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ab124684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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ICC/IF |
1/200 - 1/400.
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IHC-Fr | (1) |
1/200.
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WB | (3) |
1/1000. Detects a band of approximately 280 kDa (predicted molecular weight: 237 kDa).
For unpurified use at 1/1000 - 1/10000. |
IHC-P |
1/500. Perform heat mediated antigen retrieval before commencing with IHC staining protocol.
See IHC antigen retrieval protocols. For unpurified use at 1/50 - 1/100. |
特記事項 |
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ICC/IF
1/200 - 1/400. |
IHC-Fr
1/200. |
WB
1/1000. Detects a band of approximately 280 kDa (predicted molecular weight: 237 kDa). For unpurified use at 1/1000 - 1/10000. |
IHC-P
1/500. Perform heat mediated antigen retrieval before commencing with IHC staining protocol. See IHC antigen retrieval protocols. For unpurified use at 1/50 - 1/100. |
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: 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) analysis of human skeletal muscle tissue labelling Dysferlin with purified ab124684 at 1/500. Heat mediated antigen retrieval was performed using Tris/EDTA buffer pH 9. ab97051, a HRP-conjugated goat anti-rabbit IgG (H+L) was used as the secondary antibody (1/500). Negative control using PBS instead of primary antibody. Counterstained with hematoxylin.
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Immunohistochemistry (Formalin/PFA-fixed paraffin-embedded sections) analysis of human skeletal muscle tissue labelling Dysferlin with unpurified ab124684 at a dilution of 1/50.
Perform heat mediated antigen retrieval before commencing with IHC staining protocol.
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Immunohistochemistry (Frozen sections) analysis of unfixed frozen human skeletal muscle tissue (control) labelling Dysferlin with unpurified ab124684 at a dilution of 1/200.
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Immunocytochemistry/Immunofluorescence analysis of A673 cells labelling Dysferlin with purified ab124684 at 1/300. Cells were fixed with 4% paraformaldehyde and permeabilized with 0.1% Triton X-100. ab150077, an Alexa Fluor® 488-conjugated goat anti-rabbit IgG (1/500) was used as the secondary antibody. DAPI (blue) was used as the nuclear counterstain. ab7291, a mouse anti-tubulin (1/1000) and ab150120, an Alexa Fluor® 594-conjugated goat anti-mouse IgG (1/500) were also used.
Control 1: primary antibody (1/300) and secondary antibody, ab150120, an Alexa Fluor® 594-conjugated goat anti-mouse IgG (1/500).
Control 2: ab7291 (1/1000) and secondary antibody, ab150077, an Alexa Fluor® 488-conjugated goat anti-rabbit IgG (1/500).
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Anti-Dysferlin antibody [JAI-1-49-3] (ab124684) at 1/1000 dilution (purified) + Mouse muscle tissue lysate at 10 µg
Secondary
Peroxidase-conjugated goat anti-rabbit IgG, (H+L) at 1/1000 dilution
Predicted band size: 237 kDa
Observed band size: 280 kDa why is the actual band size different from the predicted?Blocking and dilution buffer: 5% NFDM/TBST.
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Anti-Dysferlin antibody [JAI-1-49-3] (ab124684) at 1/1000 dilution (purified) + Human skeletal muscle tissue lysate at 20 µg
Secondary
Peroxidase-conjugated goat anti-rabbit IgG, (H+L) at 1/1000 dilution
Predicted band size: 237 kDa
Observed band size: 280 kDa why is the actual band size different from the predicted?Blocking and dilution buffer: 5% NFDM/TBST.
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All lanes : Anti-Dysferlin antibody [JAI-1-49-3] (ab124684) at 1/1000 dilution (unpurified)
Lane 1 : Human skeletal muscle tissue lysate (control)
Lane 2 : Human skeletal muscle tissue lysate (LGMD2B)
Lane 3 : Mouse skeletal muscle tissue lysate (wild-type mice)
Lane 4 : Mouse skeletal muscle tissue lysate (Dysf-/- transgenic mouse)
Lysates/proteins at 10 µg per lane.
Secondary
All lanes : HRP-conjugated goat anti-rabbit IgG at 1/2000 dilution
Predicted band size: 237 kDa
Observed band size: 280 kDa why is the actual band size different from the predicted? -
Immunohistochemistry (Frozen sections) analysis of unfixed frozen mouse skeletal muscle tissue (wild type) labelling Dysferlin with unpurified ab124684 at a dilution of 1/200.
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 (34)
ab124684 は 34 報の論文で使用されています。
- Tominaga K et al. 4-Phenylbutyrate restores localization and membrane repair to human dysferlin mutations. iScience 25:103667 (2022). PubMed: 35028538
- Sarikaya E et al. Natural history of a mouse model of X-linked myotubular myopathy. Dis Model Mech 15:N/A (2022). PubMed: 35694952
- Azar C et al. RNA-Seq identifies genes whose proteins are upregulated during syncytia development in murine C2C12 myoblasts and human BeWo trophoblasts. Physiol Rep 9:e14671 (2021). PubMed: 33403800
- Qian FY et al. A novel recessive mutation affecting DNAJB6a causes myofibrillar myopathy. Acta Neuropathol Commun 9:23 (2021). PubMed: 33557929
- Fukuoka M et al. MiR-199-3p enhances muscle regeneration and ameliorates aged muscle and muscular dystrophy. Commun Biol 4:427 (2021). PubMed: 33782502