Anti-HLCS 抗体 (ab100925)
Key features and details
- Rabbit polyclonal to HLCS
- Suitable for: WB, IHC-P
- Reacts with: Human
- Isotype: IgG
製品の概要
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製品名
Anti-HLCS antibody -
製品の詳細
Rabbit polyclonal to HLCS -
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, Rat -
免疫原
Recombinant fragment, corresponding to amino acids 544-725 of Human HLCS (BC060787).
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ポジティブ・コントロール
- Human Fetal Lung, Human Fetal Kidney, Human Fetal Skeletal Muscle
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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
Lyophilized:Reconstitute with 200ul distilled sterile water. Please note that if you receive this product in liquid form it has already been reconstituted as described and no further reconstitution is necessary. -
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
Constituent: PBS -
Concentration information loading...
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精製度
Protein A 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ab100925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. Predicted molecular weight: 81 kDa.
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IHC-P |
1/100 - 1/500.
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特記事項 |
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WB
1/500 - 1/1000. Predicted molecular weight: 81 kDa. |
IHC-P
1/100 - 1/500. |
ターゲット情報
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機能
Post-translational modification of specific protein by attachment of biotin. Acts on various carboxylases such as acetyl-CoA-carboxylase, pyruvate carboxylase, propionyl CoA carboxylase, and 3-methylcrotonyl CoA carboxylase. -
組織特異性
Mostly expressed in muscle, placenta, in lesser extent in the brain, kidney, pancreas, liver and lung. -
関連疾患
Defects in HLCS are the cause of holocarboxylase synthetase deficiency (HLCS deficiency) [MIM:253270]; also known as biotin-responsive multiple carboxylase deficiency. HLCS deficiency is a neonatal form of multiple carboxylase deficiency, an autosomal recessive disorder characterized by metabolic ketoacidosis, hyperammonemia, excretion of abnormal organic acid metabolites and dermatitis. Clinical and biochemical symptoms improve dramatically with administration of biotin. -
配列類似性
Belongs to the biotin--protein ligase family. -
細胞内局在
Cytoplasm. Mitochondrion. - Information by UniProt
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参照データベース
- Entrez Gene: 3141 Human
- Entrez Gene: 110948 Mouse
- Entrez Gene: 288240 Rat
- Omim: 609018 Human
- SwissProt: P50747 Human
- SwissProt: Q920N2 Mouse
- Unigene: 371350 Human
- Unigene: 30921 Mouse
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別名
- Biotin [acetyl CoA carboxylase] ligase antibody
- Biotin [methylcrotonoyl CoA carboxylase] ligase antibody
- Biotin [methylmalonyl CoA carboxytransferase] ligase antibody
see all
画像
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All lanes : Anti-HLCS antibody (ab100925) at 1/500 dilution
Lane 1 : Human fetal lung lysate
Lane 2 : Human fetal kidney lysate
Predicted band size: 81 kDa -
Cytoplasmic staining of HLCS in a Formalin/PFA-fixed paraffin-embedded section of Human Fetal Skeletal Muscle using ab100925 at a dilution of 1/100.
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)
ab100925 は論文での使用が確認できていません。