Anti-HLCS抗体(ab100925)
Key features and details
- Rabbit polyclonal to HLCS
- Suitable for: WB, IHC-P
- Reacts with: Human
- Isotype: IgG
概述
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产品名称
Anti-HLCS抗体 -
描述
兔多克隆抗体to HLCS -
宿主
Rabbit -
经测试应用
适用于: WB, IHC-Pmore details -
种属反应性
与反应: Human
预测可用于: Mouse, Rat -
免疫原
Recombinant fragment, corresponding to amino acids 544-725 of Human HLCS (BC060787).
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阳性对照
- Human Fetal Lung, Human Fetal Kidney, Human Fetal Skeletal Muscle
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常规说明
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
性能
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形式
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. -
存放说明
Shipped at 4°C. Upon delivery aliquot and store at -20°C. Avoid freeze / thaw cycles. -
存储溶液
Constituent: PBS -
Concentration information loading...
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纯度
Protein A purified -
克隆
多克隆 -
同种型
IgG -
研究领域
相关产品
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Compatible Secondaries
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Isotype control
应用
The Abpromise guarantee
Abpromise™承诺保证使用ab100925于以下的经测试应用
“应用说明”部分 下显示的仅为推荐的起始稀释度;实际最佳的稀释度/浓度应由使用者检定。
应用 | Ab评论 | 说明 |
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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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说明 |
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WB
1/500 - 1/1000. Predicted molecular weight: 81 kDa. |
IHC-P
1/100 - 1/500. |
靶标
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功能
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. -
组织特异性
Mostly expressed in muscle, placenta, in lesser extent in the brain, kidney, pancreas, liver and lung. -
疾病相关
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. -
序列相似性
Belongs to the biotin--protein ligase family. -
细胞定位
Cytoplasm. Mitochondrion. - Information by UniProt
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数据库链接
- 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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别名
- Biotin [acetyl CoA carboxylase] ligase antibody
- Biotin [methylcrotonoyl CoA carboxylase] ligase antibody
- Biotin [methylmalonyl CoA carboxytransferase] ligase antibody
see all
图片
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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.
数据表及文件
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SDS download
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Datasheet download
文献 (0)
ab100925 尚未被引用在任何文献中。