Anti-HMGCL抗体[9F5AB9] (ab110295)
Key features and details
- Mouse monoclonal [9F5AB9] to HMGCL
- Suitable for: ICC/IF
- Reacts with: Human
- Isotype: IgG2b
概述
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产品名称
Anti-HMGCL抗体[9F5AB9]
参阅全部 HMGCL 一抗 -
描述
小鼠单克隆抗体[9F5AB9] to HMGCL -
宿主
Mouse -
经测试应用
适用于: ICC/IFmore details -
种属反应性
与反应: Human -
免疫原
Full length native protein (purified). This information is proprietary to Abcam and/or its suppliers.
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阳性对照
- HeLa cells and human liver mitochondria.
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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
Ammonium Sulphate Precipitation -
纯化说明
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
9F5AB9 -
同种型
IgG2b -
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
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Recombinant Protein
应用
The Abpromise guarantee
Abpromise™承诺保证使用ab110295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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ICC/IF |
Use a concentration of 4 µg/ml.
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说明 |
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ICC/IF
Use a concentration of 4 µg/ml. |
靶标
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功能
Involved in the catabolism of branched amino acids such as leucine. -
组织特异性
Fibroblasts, liver and lymphoblasts. -
通路
Metabolic intermediate metabolism; (S)-3-hydroxy-3-methylglutaryl-CoA degradation; acetoacetate from (S)-3-hydroxy-3-methylglutaryl-CoA: step 1/1. -
疾病相关
Defects in HMGCL are the cause of 3-hydroxy-3-methylglutaryl-CoA lyase deficiency (HMGCLD) [MIM:246450]; also known as hydroxymethylglutaricaciduria or HL deficiency. An autosomal recessive disease affecting ketogenesis and L-leucine catabolism. The disease usually appears in the first year of life after a fasting period and its clinical acute symptoms include vomiting, seizures, metabolic acidosis, hypoketotic hypoglycemia and lethargy. These symptoms sometimes progress to coma, with fatal outcome in some cases. -
序列相似性
Belongs to the HMG-CoA lyase family. -
细胞定位
Mitochondrion matrix. - Information by UniProt
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数据库链接
- Entrez Gene: 3155 Human
- Omim: 246450 Human
- Omim: 613898 Human
- SwissProt: P35914 Human
- Unigene: 533444 Human
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别名
- 3 hydroxy 3 methylglutaryl CoA lyase antibody
- 3 hydroxy 3 methylglutaryl Coenzyme A lyase antibody
- 3 hydroxymethyl 3 methylglutaryl Coenzyme A lyase (hydroxymethylglutaricaciduria) antibody
see all
图片
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Immunocytochemistry image of ab110295 stained Human HeLa cells. The cells were paraformaldehyde fixed (4%, 20 minutes) and Triton X-100 permeabilized (0.1%, 15 minutes). The cells were incubated with ab110295 at 5 µg/ml for 2 hours at room temperature or over night at 4°C. The secondary antibody was (green) Alexa Fluor® 488 goat anti-mouse IgG (H+L) used at a 1/1000 dilution for 1 hour. 10% Goat serum was used as the blocking agent for all blocking steps. Target protein locates mainly in mitochondria.
数据表及文件
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SDS download
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Datasheet download
文献 (0)
ab110295 尚未被引用在任何文献中。