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货号： bs-18719R-AF555 基本售价： 2980.0 元 规格： 100ul
产品信息
- 产品编号
- bs-18719R-AF555
- 英文名称
- Anti-MCCC2/AF555
- 中文名称
- AF555标记的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; 3-methylcrotonyl-CoA:carbon dioxide ligase subunit beta; Biotin carboxylase; MCCase subunit beta; MCCB; MCCB_HUMAN; MCCC 2; Mccc2; Methylcrotonoyl CoA carboxylase 2 (beta); Methylcrotonoyl CoA carboxylase beta chain mitochondrial; Methylcrotonoyl Coenzyme A carboxylase 2 (beta); Methylcrotonoyl-CoA carboxylase beta chain; mitochondrial; Non biotin containing subunit of 3 methylcrotonyl CoA carboxylase.
- 规格价格
- 100ul/2980元购买 大包装/询价
- 说 明 书
- 100ul
- 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, Rat, Dog, Pig, Cow, Horse, Rabbit, Xenopus tropicalis
- 产品应用
- ICC=1:50-200 IF=1:50-200
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.
- 分 子 量
- 61kDa
- 性 状
- Lyophilized or Liquid
- 浓 度
- 1mg/ml
- 免 疫 原
- KLH conjugated synthetic peptide derived from human MCCC2
- 亚 型
- IgG
- 纯化方法
- affinity purified by Protein A
- 储 存 液
- 0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol
- 保存条件
- Store at -20 °C for one year. Avoid repeated freeze/thaw cycles. The lyophilized antibody is stable at room temperature for at least one month and for greater than a year when kept at -20°C. When reconstituted in sterile pH 7.4 0.01M PBS or diluent of antibody the antibody is stable for at least two weeks at 2-4 °C.
- 产品介绍
- background:
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.
Database links:Entrez Gene: 64087Human
Entrez Gene: 78038Mouse
Entrez Gene: 361884Rat
Omim: 609014Human
SwissProt: Q9HCC0Human
SwissProt: Q3ULD5Mouse
SwissProt: Q5XIT9Rat
Unigene: 604789Human
Unigene: 137327Mouse
Unigene: 33635Rat
Important Note:
This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.