验证数据展示
经过测试的应用
| Positive WB detected in | HEK-293 cells, HeLa cells, HepG2 cells |
| Positive IP detected in | HepG2 cells |
| Positive IHC detected in | human liver cancer tissue, human prostate cancer tissue Note: suggested antigen retrieval with TE buffer pH 9.0; (*) Alternatively, antigen retrieval may be performed with citrate buffer pH 6.0 |
| Positive IF-P detected in | mouse brain tissue |
推荐稀释比
| 应用 | 推荐稀释比 |
|---|---|
| Western Blot (WB) | WB : 1:2000-1:12000 |
| Immunoprecipitation (IP) | IP : 0.5-4.0 ug for 1.0-3.0 mg of total protein lysate |
| Immunohistochemistry (IHC) | IHC : 1:50-1:500 |
| Immunofluorescence (IF)-P | IF-P : 1:50-1:500 |
| It is recommended that this reagent should be titrated in each testing system to obtain optimal results. | |
| Sample-dependent, Check data in validation data gallery. | |
产品信息
17034-1-AP targets Methylmalonyl Coenzyme A mutase/MUT in WB, IHC, IF-P, IP, ELISA applications and shows reactivity with human, mouse, rat samples.
| 经测试应用 | WB, IHC, IF-P, IP, ELISA Application Description |
| 文献引用应用 | WB, IHC, ELISA |
| 经测试反应性 | human, mouse, rat |
| 文献引用反应性 | human, mouse |
| 免疫原 |
CatNo: Ag10523 Product name: Recombinant human MUT protein Source: e coli.-derived, PGEX-4T Tag: GST Domain: 401-750 aa of BC016282 Sequence: SARIARNTQIIIQEESGIPKVADPWGGSYMMECLTNDVYDAALKLINEIEEMGGMAKAVAEGIPKLRIEECAARRQARIDSGSEVIVGVNKYQLEKEDTVEVLAIDNTSVRNRQIEKLKKIKSSRDQALAERCLAALTECAASGDGNILALAVDASRARCTVGEITDALKKVFGEHKANDRMVSGAYRQEFGESKEITSAIKRVHKFMEREGRRPRLLVAKMGQDGHDRGAKVIATGFADLGFDVDIGPLFQTPREVAQQAVDADVHAVGVSTLAAGHKTLVPELIKELNSLGRPDILVMCGGVIPPQDYEFLFEVGVSNVFGPGTRIPKAAVQVLDDIEKCLEKKQQSV 种属同源性预测 |
| 宿主/亚型 | Rabbit / IgG |
| 抗体类别 | Polyclonal |
| 产品类型 | Antibody |
| 全称 | methylmalonyl Coenzyme A mutase |
| 别名 | MUT, EC:5.4.99.2, MCM, Methylmalonyl CoA isomerase, Methylmalonyl Coenzyme A mutase |
| 计算分子量 | 750 aa, 83 kDa |
| 观测分子量 | 78 kDa |
| GenBank蛋白编号 | BC016282 |
| 基因名称 | MUT |
| Gene ID (NCBI) | 4594 |
| RRID | AB_2147263 |
| 偶联类型 | Unconjugated |
| 形式 | Liquid |
| 纯化方式 | Antigen affinity purification |
| UNIPROT ID | P22033 |
| 储存缓冲液 | PBS with 0.02% sodium azide and 50% glycerol, pH 7.3. |
| 储存条件 | Store at -20°C. Stable for one year after shipment. Aliquoting is unnecessary for -20oC storage. |
背景介绍
Methylmalonyl Coenzyme A mutase (MUT) is an enzyme that plays a crucial role in the metabolism of certain amino acids and fatty acids (PMID: 15647267). Mutations in the MUT gene can lead to methylmalonic acidemia, a metabolic disorder characterized by the accumulation of toxic compounds such as methylmalonyl-CoA and propionyl-CoA (PMID: 30428564), resulting in life-threatening metabolic acidosis, respiratory distress, neurological impairment, hyperammonemia, and ketosis (PMID: 32679819). MUT is essential for maintaining normal metabolic processes and its dysfunction can have significant health implications, highlighting its importance in both basic metabolism and clinical medicine (PMID: 23041189).
实验方案
| Product Specific Protocols | |
|---|---|
| IF protocol for Methylmalonyl Coenzyme A mutase/MUT antibody 17034-1-AP | Download protocol |
| IHC protocol for Methylmalonyl Coenzyme A mutase/MUT antibody 17034-1-AP | Download protocol |
| IP protocol for Methylmalonyl Coenzyme A mutase/MUT antibody 17034-1-AP | Download protocol |
| WB protocol for Methylmalonyl Coenzyme A mutase/MUT antibody 17034-1-AP | Download protocol |
| Standard Protocols | |
|---|---|
| Click here to view our Standard Protocols |
发表文章
| Species | Application | Title |
|---|---|---|
Hepatology Promoterless, Nuclease-Free Genome Editing Confers a Growth Advantage for Corrected Hepatocytes in Mice With Methylmalonic Acidemia. | ||
Cells Inactivity of Peptidase ClpP Causes Primary Accumulation of Mitochondrial Disaggregase ClpX with Its Interacting Nucleoid Proteins, and of mtDNA. | ||
PLoS One Novel AAV-mediated genome editing therapy improves health and survival in a mouse model of methylmalonic acidemia | ||
EMBO Mol Med A coordinated multiorgan metabolic response contributes to human mitochondrial myopathy | ||
Biochim Biophys Acta Mol Basis Dis ACSS3 regulates the metabolic homeostasis of epithelial cells and alleviates pulmonary fibrosis | ||
Am J Pathol Aging-associated Metabolite Methylmalonic Acid Increases Susceptibility to Pulmonary Fibrosis
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