| 产品编号 | bs-18719R |
| 英文名称 | MCCC2 Rabbit pAb |
| 中文名称 | MCCC2蛋白抗体 |
| 别 名 | 3 methylcrotonyl CoA carboxylase 2; 3 methylcrotonyl CoA carboxylase non biotin containing subunit; 3 methylcrotonyl CoA:carbon dioxide ligase subunit beta; 3-methylcrotonyl-CoA carboxylase 2; 3-methylcrotonyl-CoA carboxylase non-biotin-containing subunit |
| 研究领域 | 细胞生物 信号转导 新陈代谢 |
| 抗体来源 | Rabbit |
| 克隆类型 | Polyclonal |
| 交叉反应 | Human, Mouse, (predicted: Rat, Dog, Pig, Cow, Horse, Rabbit, Xenopus tropicalis) |
| 产品应用 | WB=1:500-2000
not yet tested in other applications. optimal dilutions/concentrations should be determined by the end user. |
| 理论分子量 | 61 kDa |
| 检测分子量 | |
| 细胞定位 | 细胞浆 |
| 性 状 | Liquid |
| 浓 度 | 1mg/ml |
| 免 疫 原 | KLH conjugated synthetic peptide derived from human MCCC2: 351-450/563 |
| 亚 型 | IgG |
| 纯化方法 | affinity purified by Protein A |
| 缓 冲 液 | 0.01M TBS (pH7.4) with 1% BSA, 0.02% Proclin300 and 50% Glycerol. |
| 保存条件 | Shipped at 4℃. Store at -20℃ for one year. Avoid repeated freeze/thaw cycles. |
| 注意事项 | This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications. |
| PubMed | PubMed |
| 产品介绍 |
This gene encodes the small subunit of 3-methylcrotonyl-CoA carboxylase. This enzyme functions as a heterodimer and catalyzes the carboxylation of 3-methylcrotonyl-CoA to form 3-methylglutaconyl-CoA. Mutations in this gene are associated with 3-Methylcrotonylglycinuria, an autosomal recessive disorder of leucine catabolism. [provided by RefSeq, Jul 2008] Function: Carboxyltransferase subunit of the 3-methylcrotonyl-CoA carboxylase, an enzyme that catalyzes the conversion of 3-methylcrotonyl-CoA to 3-methylglutaconyl-CoA, a critical step for leucine and isovaleric acid catabolism. Subunit: Probably a dodecamer composed of six biotin-containing alpha subunits (MCCC1) and six beta (MCCC2) subunits. Subcellular Location: Mitochondrion matrix DISEASE: Defects in MCCC2 are the cause of methylcrotonoyl-CoA carboxylase deficiency type 2 (MCC2 deficiency) [MIM:210210]. MCC2 deficiency is an autosomal recessive disorder of leucine catabolism. The phenotype is variable, ranging from neonatal onset with severe neurological involvement to asymptomatic adults. There is a characteristic organic aciduria with massive excretion of 3-hydroxyisovaleric acid and 3-methylcrotonylglycine, usually in combination with a severe secondary carnitine deficiency. Similarity: Belongs to the AccD/PCCB family. Contains 1 carboxyltransferase domain. SWISS: Q9HCC0 Gene ID: 64087 Database links: Entrez Gene: 64087 Human Entrez Gene: 78038 Mouse Omim: 609014 Human SwissProt: Q9HCC0 Human SwissProt: Q3ULD5 Mouse Unigene: 604789 Human Unigene: 137327 Mouse Unigene: 33635 Rat |
| 产品图片 |
Sample:
Kidney (Mouse) Lysate at 40 ug
DU145(Human) Cell Lysate at 30 ug
Primary: Anti-MCCC2 (bs-18719R) at 1/1000 dilution
Secondary: IRDye800CW Goat Anti-Rabbit IgG at 1/20000 dilution
Predicted band size: 61 kD
Observed band size: 61 kD
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| 1、抗体溶解方法 | |
| 2、抗体修复方式 | |
| 3、常用试剂的配制 | |
| 4、免疫组化操作步骤 | |
| 5、免疫组化问题解答 | |
| 6、Western Blotting 操作步骤 | |
| 7、Western Blotting 问题解答 | |
| 8、关于肽链的设计 | |
| 9、多肽的溶解与保存 | |
| 10、酶标抗体效价测定程序 | |
