Anti-PDHA1抗体[9H9AF5] (ab110330)
Key features and details
- Mouse monoclonal [9H9AF5] to PDHA1
- Suitable for: WB, ICC/IF, Flow Cyt
- Knockout validated
- Reacts with: Mouse, Rat, Cow, Human
- Isotype: IgG1
Related conjugates and formulations
概述
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产品名称
Anti-PDHA1抗体[9H9AF5]
参阅全部 PDHA1 一抗 -
描述
小鼠单克隆抗体[9H9AF5] to PDHA1 -
宿主
Mouse -
经测试应用
适用于: WB, ICC/IF, Flow Cytmore details -
种属反应性
与反应: Mouse, Rat, Cow, Human -
免疫原
Recombinant full length protein corresponding to Human PDHA1.
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阳性对照
- Isolated mitochondria from Human, Bovine, Rat and Mouse hearts, HepG2 lysate; cultured, normal Human embryonic lung fibroblasts (strain MRC5); HL60 cells.
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常规说明
This antibody clone is manufactured by Abcam. If you require a custom buffer formulation or conjugation for your experiments, please contact orders@abcam.com.
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
Product was previously marketed under the MitoSciences sub-brand.
性能
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形式
Liquid -
存放说明
Shipped at 4°C. Store at +4°C. Do Not Freeze. -
存储溶液
pH: 7.5
Preservative: 0.02% Sodium azide
Constituent: HEPES buffered saline -
Concentration information loading...
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纯度
IgG fraction -
纯化说明
ab110330 was produced in vitro using hybridomas grown in serum-free medium, and then purified by biochemical fractionation. -
克隆
单克隆 -
克隆编号
9H9AF5 -
同种型
IgG1 -
轻链类型
kappa -
研究领域
相关产品
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Alternative Versions
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Compatible Secondaries
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Conjugation kits
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Isotype control
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Recombinant Protein
应用
The Abpromise guarantee
Abpromise™承诺保证使用ab110330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 | (6) |
Use a concentration of 1 µg/ml. Predicted molecular weight: 43 kDa.
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ICC/IF | (1) |
Use a concentration of 5 µg/ml.
(heat-induced antigen-retrieval improves signal) |
Flow Cyt |
Use a concentration of 1 µg/ml.
ab170190 - Mouse monoclonal IgG1, is suitable for use as an isotype control with this antibody. |
说明 |
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WB
Use a concentration of 1 µg/ml. Predicted molecular weight: 43 kDa. |
ICC/IF
Use a concentration of 5 µg/ml. (heat-induced antigen-retrieval improves signal) |
Flow Cyt
Use a concentration of 1 µg/ml. ab170190 - Mouse monoclonal IgG1, is suitable for use as an isotype control with this antibody. |
靶标
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功能
The pyruvate dehydrogenase complex catalyzes the overall conversion of pyruvate to acetyl-CoA and CO(2). It contains multiple copies of three enzymatic components: pyruvate dehydrogenase (E1), dihydrolipoamide acetyltransferase (E2) and lipoamide dehydrogenase (E3). -
组织特异性
Ubiquitous. -
疾病相关
Defects in PDHA1 are a cause of pyruvate decarboxylase E1 component deficiency (PDHE1 deficiency) [MIM:312170]. PDHE1 deficiency is the most common enzyme defect in patients with primary lactic acidosis. It is associated with variable clinical phenotypes ranging from neonatal death to prolonged survival complicated by developmental delay, seizures, ataxia, apnea, and in some cases to an X-linked form of Leigh syndrome (X-LS).
Defects in PDHA1 are the cause of X-linked Leigh syndrome (X-LS) [MIM:308930]. X-LS is an early-onset progressive neurodegenerative disorder with a characteristic neuropathology consisting of focal, bilateral lesions in one or more areas of the central nervous system, including the brainstem, thalamus, basal ganglia, cerebellum, and spinal cord. The lesions are areas of demyelination, gliosis, necrosis, spongiosis, or capillary proliferation. Clinical symptoms depend on which areas of the central nervous system are involved. The most common underlying cause is a defect in oxidative phosphorylation. LS may be a feature of a deficiency of any of the mitochondrial respiratory chain complexes. -
细胞定位
Mitochondrion matrix. - Information by UniProt
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数据库链接
- Entrez Gene: 407109 Cow
- Entrez Gene: 5160 Human
- Entrez Gene: 18597 Mouse
- Entrez Gene: 29554 Rat
- Omim: 300502 Human
- SwissProt: A7MB35 Cow
- SwissProt: P08559 Human
- SwissProt: P35486 Mouse
see all -
别名
- ODPA_HUMAN antibody
- PDH antibody
- PDHA antibody
see all
图片
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All lanes : Anti-PDHA1 antibody [9H9AF5] (ab110330) at 1 µg/ml
Lane 1 : Wild-type HeLa whole cell lysate
Lane 2 : PDHA1 knockout HeLa whole cell lysate
Lane 3 : HEK-293 whole cell lysate
Lysates/proteins at 20 µg per lane.
Predicted band size: 43 kDa
Observed band size: 43 kDaLanes 1 - 3: Merged signal (red and green). Green - ab110330 observed at 43 kDa. Red - loading control, ab52866, observed at 50 kDa.
ab110330 was shown to specifically react with PDHA1 in wild-type HeLa cells as signal was lost in PDHA1 knockout cells. Wild-type and PDHA1 knockout samples were subjected to SDS-PAGE. Ab110330 and ab52866 (Rabbit anti alpha Tubulin loading control) were incubated overnight at 4°C at 1 ug/ml and 1/1000 dilution respectively. Blots were developed with Goat anti-Mouse IgG H&L (IRDye® 800CW) preabsorbed ab216772 and Goat anti-Rabbit IgG H&L (IRDye® 680RD) preabsorbed ab216777 secondary antibodies at 1/20000 dilution for 1 hour at room temperature before imaging.
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All lanes : Anti-PDHA1 antibody [9H9AF5] (ab110330) at 1 µg/ml
Lane 1 : Isolated mitochondria from Human heart at 10 µg
Lane 2 : Isolated mitochondria from Bovine heart at 4 µg
Lane 3 : Isolated mitochondria from Rat heart at 10 µg
Lane 4 : Isolated mitochondria from Mouse heart at 10 µg
Lane 5 : hepG2 cell lysate at 20 µg
Predicted band size: 43 kDa -
Immunocytochemistry analysis using ab110330 at 5µg/ml staining PDHA1 in cultured, normal Human embryonic lung fibroblasts (strain MRC5) followed by AlexaFluor® 488 goat anti-mouse IgG1 secondary antibody (2 µg/ml).
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Immunocytochemical analysis of SH-SY5Y human neuroblastoma labeling PDHA1 with ab110330 at 1/500 dilution.
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Flow cytometric analysis using ab110330 at 1µg/ml staining PDHA1 in HL60 cells (blue). Isotype control antibody (red).
实验方案
数据表及文件
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SDS download
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Datasheet download
文献 (78)
ab110330 被引用在 78 文献中.
- Sato T et al. Enhanced glucose metabolism through activation of HIF-1α covers the energy demand in a rat embryonic heart primordium after heartbeat initiation. Sci Rep 12:74 (2022). PubMed: 34996938
- Williams AS et al. Nicotinamide riboside supplementation confers marginal metabolic benefits in obese mice without remodeling the muscle acetyl-proteome. iScience 25:103635 (2022). PubMed: 35028529
- Han A et al. Pyruvate dehydrogenase inactivation causes glycolytic phenotype in BAP1 mutant uveal melanoma. Oncogene 41:1129-1139 (2022). PubMed: 35046531
- Erdem A et al. The Glycolytic Gatekeeper PDK1 defines different metabolic states between genetically distinct subtypes of human acute myeloid leukemia. Nat Commun 13:1105 (2022). PubMed: 35232995
- Uchenunu O et al. Mitochondrial complex IV defects induce metabolic and signaling perturbations that expose potential vulnerabilities in HCT116 cells. FEBS Open Bio 12:959-982 (2022). PubMed: 35302710