重组Anti-HMBS/PBGD抗体[EPR8105] (ab129092)
Key features and details
- Produced recombinantly (animal-free) for high batch-to-batch consistency and long term security of supply
- Rabbit monoclonal [EPR8105] to HMBS/PBGD
- Suitable for: WB
- Reacts with: Human
Related conjugates and formulations
概述
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产品名称
Anti-HMBS/PBGD抗体[EPR8105]
参阅全部 HMBS/PBGD 一抗 -
描述
兔单克隆抗体[EPR8105] to HMBS/PBGD -
宿主
Rabbit -
经测试应用
适用于: WBmore details
不适用于: Flow Cyt,ICC/IF,IHC-P or IP -
种属反应性
与反应: Human
预测可用于: Mouse, Rat -
免疫原
Synthetic peptide. This information is proprietary to Abcam and/or its suppliers.
(Peptide available asab123176) -
阳性对照
- HepG2, HeLa, U937, and K562 cell lysates
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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 -20°C. Stable for 12 months at -20°C. -
解离常数(KD)
KD = 2.94 x 10 -11 M Learn more about KD -
存储溶液
pH: 7.20
Preservative: 0.01% Sodium azide
Constituents: 9% PBS, 40% Glycerol (glycerin, glycerine), 0.05% BSA, 50% Tissue culture supernatant -
Concentration information loading...
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纯度
Protein A purified -
克隆
单克隆 -
克隆编号
EPR8105 -
同种型
IgG -
研究领域
相关产品
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Alternative Versions
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Immunizing Peptide (Blocking)
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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ab129092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 - 1/10000. Detects a band of approximately 42-44 kDa (predicted molecular weight: 39 kDa).Can be blocked with Recombinant Human HMBS/PBGD protein (ab123176).
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说明 |
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WB
1/1000 - 1/10000. Detects a band of approximately 42-44 kDa (predicted molecular weight: 39 kDa).Can be blocked with Recombinant Human HMBS/PBGD protein (ab123176). |
靶标
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功能
Tetrapolymerization of the monopyrrole PBG into the hydroxymethylbilane pre-uroporphyrinogen in several discrete steps. -
组织特异性
Isoform 1 is ubiquitously expressed. Isoform 2 is found only in erythroid cells. -
通路
Porphyrin metabolism; protoporphyrin-IX biosynthesis; coproporphyrinogen-III from 5-aminolevulinate: step 2/4. -
疾病相关
Defects in HMBS are the cause of acute intermittent porphyria (AIP) [MIM:176000]. AIP is a form of porphyria. 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. AIP is an autosomal dominant form of hepatic porphyria characterized by acute attacks of neurological dysfunctions with abdominal pain, hypertension, tachycardia, and peripheral neuropathy. Most attacks are precipitated by drugs, alcohol, caloric deprivation, infections, or endocrine factors. -
序列相似性
Belongs to the HMBS family. -
细胞定位
Cytoplasm. - Information by UniProt
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数据库链接
- Entrez Gene: 3145 Human
- Entrez Gene: 15288 Mouse
- Entrez Gene: 25709 Rat
- Omim: 609806 Human
- SwissProt: P08397 Human
- SwissProt: P22907 Mouse
- SwissProt: P19356 Rat
- Unigene: 82609 Human
see all -
别名
- HEM3_HUMAN antibody
- HMBS antibody
- Hydroxymethylbilane synthase antibody
see all
图片
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All lanes : Anti-HMBS/PBGD antibody [EPR8105] (ab129092) at 1/1000 dilution
Lane 1 : HepG2 cell lysates
Lane 2 : HeLa cell lysates
Lane 3 : U937 cell lysates
Lane 4 : K562 cell lysates
Lysates/proteins at 10 µg per lane.
Secondary
All lanes : HRP labelled goat anti-rabbit at 1/2000 dilution
Predicted band size: 39 kDa
Observed band size: 42-44 kDa why is the actual band size different from the predicted?
数据表及文件
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SDS download
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Datasheet download
文献 (6)
ab129092 被引用在 6 文献中.
- Ma L et al. A role of splenic heme biosynthesis pathway in the persistent prophylactic actions of arketamine in lipopolysaccharide-treated mice. Transl Psychiatry 13:269 (2023). PubMed: 37491335
- Xu J et al. ZO-2/Tjp2 suppresses Yap and Wwtr1/Taz-mediated hepatocyte to cholangiocyte transdifferentiation in the mouse liver. NPJ Regen Med 7:55 (2022). PubMed: 36151109
- Biancur DE et al. Functional Genomics Identifies Metabolic Vulnerabilities in Pancreatic Cancer. Cell Metab 33:199-210.e8 (2021). PubMed: 33152323
- Yang Y et al. A novel heterozygous mutation in the HMBS gene in a patient with acute intermittent porphyria and posterior reversible encephalopathy syndrome. Mol Med Rep 22:516-524 (2020). PubMed: 32377710
- Chen B et al. Identification and characterization of 40 novel hydroxymethylbilane synthase mutations that cause acute intermittent porphyria. J Inherit Metab Dis 42:186-194 (2019). PubMed: 30740734
- 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