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Anti-MCCC2抗体

产品价格: ¥1380.00

最小起订量:暂无 可售数量:暂无

发货时限:
暂无
所在地区:
全国
有效期至:
长期有效
最后更新:
2019-06-11 16:44:11
浏览次数:
36

产品详情

品牌名称:
Ybscience

 Anti-MCCC2抗体

产品编号 YB-18719R
英文名称 MCCC2
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; 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/1380元 购买    200ul/2200元 购买    大包装/询价
说 明 书 100ul  200ul
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 WB=1:500-2000   not yet tested in other applications. optimal dilutions/concentrations should be determined by the end user.
分 子 量 61kDa
细胞定位 细胞浆 
性    状 Lyophilized or 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
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.
PubMed PubMed
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.  SWISS: Q9HCC0 Gene ID: 64087 Database links:

Entrez Gene: 64087 Human

Entrez Gene: 78038 Mouse

Entrez Gene: 361884 Rat

Omim: 609014 Human

SwissProt: Q9HCC0 Human

SwissProt: Q3ULD5 Mouse

SwissProt: Q5XIT9 Rat

Unigene: 604789 Human

Unigene: 137327 Mouse

Unigene: 33635 Rat

Important Note: This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.   
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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