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Rabbit Anti-MCCC2/BF555 Conjugated antibody (bs-18719R-BF555)
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說 明 書: 100ul  
100ul/2980.00元
大包裝/詢價(jià)
產(chǎn)品編號(hào) bs-18719R-BF555
英文名稱 Rabbit Anti-MCCC2/BF555 Conjugated antibody
中文名稱 BF555標(biāo)記的MCCC2蛋白抗體
別    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.  
規(guī)格價(jià)格 100ul/2980元 購買        大包裝/詢價(jià)
說 明 書 100ul  
研究領(lǐng)域 細(xì)胞生物  信號(hào)轉(zhuǎn)導(dǎo)  新陳代謝  
抗體來源 Rabbit
克隆類型 Polyclonal
交叉反應(yīng) Human, Mouse,  (predicted: Rat, Dog, Pig, Cow, Horse, Rabbit, Xenopus tropicalis)
產(chǎn)品應(yīng)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    狀 Lyophilized or Liquid
濃    度 1mg/ml
免 疫 原 KLH conjugated synthetic peptide derived from human MCCC2
亞    型 IgG
純化方法 affinity purified by Protein A
儲(chǔ) 存 液 0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol
保存條件 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.
產(chǎn)品介紹 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: 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.
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