重组Anti-ALAS2/ASB抗体[EPR15112(B)] - C-terminal (ab184964)
Key features and details
- Produced recombinantly (animal-free) for high batch-to-batch consistency and long term security of supply
- Rabbit monoclonal [EPR15112(B)] to ALAS2/ASB - C-terminal
- Suitable for: WB, IP
- Reacts with: Mouse, Rat, Human
Related conjugates and formulations
概述
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产品名称
Anti-ALAS2/ASB抗体[EPR15112(B)] - C-terminal
参阅全部 ALAS2/ASB 一抗 -
描述
兔单克隆抗体[EPR15112(B)] to ALAS2/ASB - C-terminal -
宿主
Rabbit -
经测试应用
适用于: WB, IPmore details -
种属反应性
与反应: Mouse, Rat, Human -
免疫原
Synthetic peptide. This information is proprietary to Abcam and/or its suppliers.
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阳性对照
- WB: NIH/3T3, PC-12, Rat brain, Jurkat, 293 and Human fetal liver. IP: K-562 cells.
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常规说明
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.
性能
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形式
Liquid -
存放说明
Shipped at 4°C. Store at +4°C short term (1-2 weeks). Upon delivery aliquot. Store at -20°C long term. Avoid freeze / thaw cycle. -
存储溶液
pH: 7.2
Preservative: 0.01% Sodium azide
Constituents: 59% PBS, 40% Glycerol (glycerin, glycerine), 0.05% BSA -
Concentration information loading...
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纯度
Protein A purified -
克隆
单克隆 -
克隆编号
EPR15112(B) -
同种型
IgG -
研究领域
相关产品
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Alternative Versions
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Conjugation kits
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Isotype control
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Positive Controls
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Recombinant Protein
应用
The Abpromise guarantee
Abpromise™承诺保证使用ab184964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 |
1/1000 - 1/2000. Detects a band of approximately 65 kDa (predicted molecular weight: 65 kDa).
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IP |
1/30 - 1/50.
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说明 |
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WB
1/1000 - 1/2000. Detects a band of approximately 65 kDa (predicted molecular weight: 65 kDa). |
IP
1/30 - 1/50. |
靶标
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组织特异性
Erythroid specific. -
通路
Porphyrin metabolism; protoporphyrin-IX biosynthesis; 5-aminolevulinate from glycine: step 1/1. -
疾病相关
Defects in ALAS2 are a cause of anemia sideroblastic X-linked (XLSA) [MIM:300751]. Sideroblastic anemia is characterized by anemia of varying severity, hypochromic peripheral erythrocytes, systemic iron overload secondary to chronic ineffective erythropoiesis, and the presence of bone marrow ringed sideroblasts. Sideroblasts are characterized by iron-loaded mitochondria clustered around the nucleus. XLSA shows a variable hematologic response to pharmacologic doses of pyridoxine.
Defects in ALAS2 are the cause of erythropoietic protoporphyria X-linked dominant (XLDPT) [MIM:300752]. Porphyrias are inherited defects in the biosynthesis of heme, resulting in the accumulation and increased excretion of porphyrins or porphyrin precursors. They are classified as erythropoietic or hepatic, depending on whether the enzyme deficiency occurs in red blood cells or in the liver. XLDPT is a form of porphyria characterized biochemically by a high proportion of zinc-protoporphyrin in erythrocytes, in which a mismatch between protoporphyrin production and the heme requirement of differentiating erythroid cells leads to overproduction of protoporphyrin in amounts sufficient to cause photosensitivity and liver disease. Note=Gain of function mutations in ALS2 are responsible for XLDPT, but they can also be a possible aggravating factor in congenital erythropoietic porphyria and other erythropoietic disorders caused by mutations in other genes (PubMed:21309041). -
序列相似性
Belongs to the class-II pyridoxal-phosphate-dependent aminotransferase family. -
细胞定位
Mitochondrion matrix. - Information by UniProt
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数据库链接
- Entrez Gene: 212 Human
- Entrez Gene: 11656 Mouse
- Entrez Gene: 25748 Rat
- Omim: 301300 Human
- SwissProt: P22557 Human
- SwissProt: P08680 Mouse
- SwissProt: Q63147 Rat
- Unigene: 522666 Human
see all -
别名
- 5 @aminolevulinate synthase erythroid specific antibody
- 5 aminolevulinate synthase 2 antibody
- 5 aminolevulinate synthase 5 aminolevulinate synthase 2 antibody
see all
图片
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All lanes : Anti-ALAS2/ASB antibody [EPR15112(B)] - C-terminal (ab184964) at 1/1000 dilution (Purified)
Lane 1 : NIH/3T3 (Mouse embryonic fibroblast) whole cell lysates
Lane 2 : PC-12 (Rat adrenal gland pheochromocytoma) whole cell lysates
Lane 3 : Rat brain lysates
Lysates/proteins at 15 µg per lane.
Secondary
All lanes : Goat Anti-Rabbit IgG H&L (HRP) (ab97051) at 1/20000 dilution
Predicted band size: 65 kDa
Observed band size: 65 kDa -
ab184964 (Purified) at 1:30 dilution (2 µg) immunoprecipitating ALAS2/ASB in K-562 whole cell lysate.
Lane 1 (input): K-562 (Human chronic myelogenous leukemia lymphoblast) whole cell lysate 10 µg
Lane 2 (+): ab184964 & K-562 whole cell lysate
Lane 3 (-): Rabbit monoclonal IgG (ab172730) instead of ab184964 in K-562 whole cell lysate
For western blotting, VeriBlot for IP Detection Reagent (HRP) (ab131366) was used at 1:1000 dilution.
Blocking and diluting buffer: 5% NFDM/TBST. -
All lanes : Anti-ALAS2/ASB antibody [EPR15112(B)] - C-terminal (ab184964) at 1/1000 dilution
Lane 1 : K562 lysate
Lane 2 : Jurkat lysate
Lane 3 : 293 lysate
Lysates/proteins at 20 µg per lane.
Secondary
All lanes : Goat Anti-Rabbit IgG, (H+L), Peroxidase conjugated at 1/1000 dilution
Predicted band size: 65 kDa -
Anti-ALAS2/ASB antibody [EPR15112(B)] - C-terminal (ab184964) at 1/1000 dilution + Human fetal liver at 10 µg
Secondary
Anti-Rabbit IgG (HRP), specific to the non-reduced form of IgG at 1/1000 dilution
Predicted band size: 65 kDa -
Western blot analysis of immunoprecipitation pellet from 293 cell lysate immunoprecipitated using ab184964 at 1/50 dilution.
Goat Anti-Rabbit IgG, (H+L), Peroxidase conjugated secondary used at a 1/1000 dilution.
实验方案
数据表及文件
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SDS download
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Datasheet download
Certificate of Compliance
文献 (8)
ab184964 被引用在 8 文献中.
- Ochi T et al. Exploring the mechanistic link between SF3B1 mutation and ring sideroblast formation in myelodysplastic syndrome. Sci Rep 12:14562 (2022). PubMed: 36028755
- Jin T et al. ANTXR1 Regulates Erythroid Cell Proliferation and Differentiation through wnt/β-Catenin Signaling Pathway In Vitro and in Hematopoietic Stem Cell. Dis Markers 2022:1226697 (2022). PubMed: 36065334
- Barman-Aksözen J et al. Delta-aminolevulinic acid synthase 2 expression in combination with iron as modifiers of disease severity in erythropoietic protoporphyria. Mol Genet Metab N/A:N/A (2019). PubMed: 31076252
- Maio N et al. Dimeric ferrochelatase bridges ABCB7 and ABCB10 homodimers in an architecturally defined molecular complex required for heme biosynthesis. Haematologica N/A:N/A (2019). PubMed: 30765471
- Chen CP et al. Synergistic antitumor activity of artesunate and HDAC inhibitors through elevating heme synthesis via synergistic upregulation of ALAS1 expression. Acta Pharm Sin B 9:937-951 (2019). PubMed: 31649844
- Paul BT et al. Sideroflexin 4 affects Fe-S cluster biogenesis, iron metabolism, mitochondrial respiration and heme biosynthetic enzymes. Sci Rep 9:19634 (2019). PubMed: 31873120
- Liu J et al. Long non-coding RNA-dependent mechanism to regulate heme biosynthesis and erythrocyte development. Nat Commun 9:4386 (2018). PubMed: 30349036
- Yien YY et al. Mutation in human CLPX elevates levels of d-aminolevulinate synthase and protoporphyrin IX to promote erythropoietic protoporphyria. Proc Natl Acad Sci U S A 114:E8045-E8052 (2017). PubMed: 28874591