Anti-MTCO3抗体[DA5BC4] (ab110259)
![Western blot - Anti-MTCO3 antibody [DA5BC4] (ab110259)](https://www.abcam.cn/ps/products/110/ab110259/Images/ab110259-123444-anti-mtco3-antibody-da5bc4-western-blot.jpg "Western blot - Anti-MTCO3 antibody [DA5BC4] (ab110259)")
Key features and details
- Mouse monoclonal [DA5BC4] to MTCO3
- Suitable for: WB
- Reacts with: Mouse, Rat, Human, Saccharomyces cerevisiae
- Isotype: IgG2a
概述
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产品名称
Anti-MTCO3抗体[DA5BC4] -
描述
小鼠单克隆抗体[DA5BC4] to MTCO3 -
宿主
Mouse -
经测试应用
适用于: WBmore details -
种属反应性
与反应: Mouse, Rat, Human, Saccharomyces cerevisiae -
免疫原
Full length native protein (purified). This information is considered to be commercially sensitive.
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阳性对照
- Isolated mitochondria from Human , Rat and Mouse hearts.
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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... -
纯度
IgG fraction -
纯化说明
ab110259 was produced in vitro using hybridomas grown in serum-free medium, and then purified by biochemical fractionation. Near homogeneity ab110259 was judged as near homogeneity by SDS-PAGE. -
克隆
单克隆 -
克隆编号
DA5BC4 -
同种型
IgG2a -
轻链类型
kappa -
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ab110259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 |
|---|---|---|
| WB | (3) |
Use a concentration of 2 - 4 µg/ml. Predicted molecular weight: 30 kDa.
Due to the hydrophobic nature of this protein, it is recommended that the WB is performed on a PVDF membrane with a 90 minute CAPS-buffer transfer. |
| 说明 |
|---|
|
WB
Use a concentration of 2 - 4 µg/ml. Predicted molecular weight: 30 kDa. Due to the hydrophobic nature of this protein, it is recommended that the WB is performed on a PVDF membrane with a 90 minute CAPS-buffer transfer. |
靶标
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功能
Subunits I, II and III form the functional core of the enzyme complex. -
疾病相关
Defects in MT-CO3 are a cause of Leber hereditary optic neuropathy (LHON) [MIM:535000]. LHON is a maternally inherited disease resulting in acute or subacute loss of central vision, due to optic nerve dysfunction. Cardiac conduction defects and neurological defects have also been described in some patients. LHON results from primary mitochondrial DNA mutations affecting the respiratory chain complexes.
Defects in MT-CO3 are a cause of mitochondrial complex IV deficiency (MT-C4D) [MIM:220110]; also known as cytochrome c oxidase deficiency. A disorder of the mitochondrial respiratory chain with heterogeneous clinical manifestations, ranging from isolated myopathy to severe multisystem disease affecting several tissues and organs. Features include hypertrophic cardiomyopathy, hepatomegaly and liver dysfunction, hypotonia, muscle weakness, excercise intolerance, developmental delay, delayed motor development and mental retardation. A subset of patients manifest Leigh syndrome.
Defects in MT-CO3 are associated with recurrent myoglobinuria mitochondrial (RM-MT) [MIM:550500]. Recurrent myoglobinuria is characterized by recurrent attacks of rhabdomyolysis (necrosis or disintegration of skeletal muscle) associated with muscle pain and weakness, and followed by excretion of myoglobin in the urine. -
序列相似性
Belongs to the cytochrome c oxidase subunit 3 family. -
细胞定位
Mitochondrion inner membrane. - Information by UniProt
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数据库链接
- Entrez Gene: 4514 Human
- Entrez Gene: 17710 Mouse
- Entrez Gene: 26204 Rat
- Omim: 516050 Human
- SwissProt: P00414 Human
- SwissProt: P00416 Mouse
- SwissProt: P05505 Rat
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别名
- COIII antibody
- COX3 antibody
- COX3_HUMAN antibody
see all
图片
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Western blot - Anti-MTCO3 antibody [DA5BC4] (ab110259)All lanes : Anti-MTCO3 antibody [DA5BC4] (ab110259) at 4 µg/ml
Lane 1 : Isolated mitochondria from Human heart at 5 µg
Lane 2 : Isolated mitochondria Rat heart at 10 µg
Lane 3 : Isolated mitochondria from Mouse heart at 10 µg
Predicted band size: 30 kDa
数据表及文件
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SDS download
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Datasheet download
文献 (45)
ab110259 被引用在 45 文献中.
- Mori MP et al. Mitochondrial respiration reduces exposure of the nucleus to oxygen. J Biol Chem 299:103018 (2023). PubMed: 36796514
- Rimoldi M et al. Prominent muscle involvement in a familial form of mitochondrial disease due to a COA8 variant. Front Genet 14:1278572 (2023). PubMed: 38098475
- White AJ et al. Loss of Num1-mediated cortical dynein anchoring negatively impacts respiratory growth. J Cell Sci 135:N/A (2022). PubMed: 36185004
- Esparza-Moltó PB et al. Generation of mitochondrial reactive oxygen species is controlled by ATPase inhibitory factor 1 and regulates cognition. PLoS Biol 19:e3001252 (2021). PubMed: 33983919
- Xu M et al. Identification of a Novel Variant in MT-CO3 Causing MELAS. Front Genet 12:638749 (2021). PubMed: 34054915