Anti-FKRP 抗体 (ab65243)
Key features and details
- Rabbit polyclonal to FKRP
- Suitable for: WB
- Reacts with: Human
- Isotype: IgG
製品の概要
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製品名
Anti-FKRP antibody
FKRP 一次抗体 製品一覧 -
製品の詳細
Rabbit polyclonal to FKRP -
由来種
Rabbit -
アプリケーション
適用あり: WBmore details -
種交差性
交差種: Human
交差が予測される動物種: Mouse, Rat -
免疫原
Synthetic peptide derived from N terminal of human FKRP.
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ポジティブ・コントロール
- Jurkat and COLO205 cell extracts.
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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.
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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 -20°C. Stable for 12 months at -20°C. -
バッファー
pH: 7.40
Preservative: 0.02% Sodium azide
Constituents: PBS, 50% Glycerol (glycerin, glycerine), 0.87% Sodium chloride
Without Mg2+ and Ca2+ -
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
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Recombinant Protein
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ab65243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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WB |
1/500 - 1/1000. Detects a band of approximately 55 kDa (predicted molecular weight: 55 kDa).
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特記事項 |
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WB
1/500 - 1/1000. Detects a band of approximately 55 kDa (predicted molecular weight: 55 kDa). |
ターゲット情報
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機能
Could be a transferase involved in the modification of glycan moieties of alpha-dystroglycan (DAG1). -
組織特異性
Expressed predominantly in skeletal muscle, placenta, and heart and relatively weakly in brain, lung, liver kidney and pancreas. -
関連疾患
Defects in FKRP are the cause of muscular dystrophy-dystroglycanopathy congenital with brain and eye anomalies type A5 (MDDGA5) [MIM:613153]. MDDGA5 is an autosomal recessive disorder characterized by congenital muscular dystrophy associated with cobblestone lissencephaly and other brain anomalies, eye malformations, profound mental retardation, and death usually in the first years of life. Included diseases are the more severe Walker-Warburg syndrome and the slightly less severe muscle-eye-brain disease.
Defects in FKRP are the cause of muscular dystrophy-dystroglycanopathy congenital with or without mental retardation type B5 (MDDGB5) [MIM:606612]. MDDGB5 is a congenital muscular dystrophy characterized by a severe phenotype with inability to walk, muscle hypertrophy, marked elevation of serum creatine kinase, a secondary deficiency of laminin alpha2, and a marked reduction in alpha-dystroglycan expression. Only a subset of MDDGB5 patients have brain involvements.
Defects in FKRP are the cause of muscular dystrophy-dystroglycanopathy limb-girdle type C5 (MDDGC5) [MIM:607155]; also known as limb-girdle muscular dystrophy type 2I. MDDGC5 is an autosomal recessive disorder with age of onset ranging from childhood to adult life, and variable severity. Clinical features include proximal muscle weakness, waddling gait, calf hypertrophy, cardiomyopathy and respiratory insufficiency. A reduction of alpha-dystroglycan and laminin alpha-2 expression can be observed on skeletal muscle biopsy from MDDGC5 patients. -
配列類似性
Belongs to the licD transferase family. -
翻訳後修飾
N-glycosylated. -
細胞内局在
Golgi apparatus. Secreted. Cell membrane > sarcolemma. Rough endoplasmic reticulum. According to some studies the N-terminal hydrophobic domain is cleaved after translocation to the Golgi apparatus and the protein is secreted. According to others the N-terminal hydrophobic domain is a transmembrane domain and the protein is a type II transmembrane type targeted to the Golgi apparatus by a non-cleavable signal anchor sequence. Localization at the cell membrane may require the presence of dystroglycan. At the Golgi apparatus localizes most likely at the cis-compartment. Detected in rough endoplasmic reticulum in myocytes. In general, mutants associated with severe clinical phenotypes are retained within the endoplasmic reticulum. - Information by UniProt
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参照データベース
- Entrez Gene: 79147 Human
- Entrez Gene: 243853 Mouse
- Entrez Gene: 308390 Rat
- Omim: 606596 Human
- SwissProt: Q9H9S5 Human
- SwissProt: Q8CG64 Mouse
- Unigene: 515493 Human
- Unigene: 39703 Mouse
see all -
別名
- Fkrp antibody
- FKRP_HUMAN antibody
- FLJ12576 antibody
see all
画像
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All lanes : Anti-FKRP antibody (ab65243) at 1/500 dilution
Lane 1 : Jurkat cell extract
Lane 2 : COLO205 cell extract
Lane 3 : COLO205 cell extract with immunising peptide
Predicted band size: 55 kDa
Observed band size: 55 kDa
Additional bands at: 22 kDa, 24 kDa. We are unsure as to the identity of these extra bands.
The amount of positive control loading for the WB is 5-30 ug of total protein. The amount of the peptide for the WB is 5-10 ug.
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)
ab65243 は論文での使用が確認できていません。