日日操日日操,亚洲日本午夜激情,人妻内射精品一区二区,国产一区北条麻妃

掃碼關(guān)注公眾號           掃碼咨詢技術(shù)支持           掃碼咨詢技術(shù)服務(wù)
  
客服熱線:400-901-9800  客服QQ:4009019800  技術(shù)答疑  技術(shù)支持  質(zhì)量反饋  關(guān)于我們  聯(lián)系我們
伊人久九大香蕉精品视频网,国产懂色av,亚洲 日韩 中文字幕 电影网
Rabbit Anti-MCCC2/BF594 Conjugated antibody (bs-18719R-BF594)
訂購熱線:400-901-9800
訂購郵箱:sales@bioss.com.cn
訂購QQ:  400-901-9800
技術(shù)支持:techsupport@bioss.com.cn
說 明 書: 100ul  
100ul/2980.00元
大包裝/詢價(jià)
產(chǎn)品編號 bs-18719R-BF594
英文名稱 Rabbit Anti-MCCC2/BF594 Conjugated antibody
中文名稱 BF594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 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.
版權(quán)所有 2004-2026 m.bklrv.cn 北京博奧森生物技術(shù)有限公司
通過國際質(zhì)量管理體系ISO 9001:2015 GB/T 19001-2016    證書編號: 00124Q34771R2M/1100
通過國際醫(yī)療器械-質(zhì)量管理體系ISO 13485:2016 GB/T 42061-2022    證書編號: CQC24QY10047R0M/1100
京ICP備05066980號-1         京公網(wǎng)安備110107000727號
久久久999免费精彩视频| 蜜臀av高清在线观看x| 久久久亚洲AⅤ无码精品色| 日韩美中文| 91国自产精品| ,精品久久人人爽天天玩人人妻| 国产高清人人肏| 人人操天天操人人操| 熟 少妇 久久| 嗯嗯啊啊啊国产一区二区三区| 亚洲精品在| 色。就。久| 久久久区一区二区二| 精品一网曝| 人人妻,一区二区三区| 日韩中文人妻少妇| 亚洲综合一区二区日韩欧美| 久久婷婷五月综合色丁香| 国产日产欧产美韩久久久久| 欧美一级 Ⅹ| 男人久久久精品乱码一区二区| 91精品一区二区丝袜美腿| 人体艺术国产精品一区| 亚洲三级AV伦理片网页| 中文字幕熟女人妻日韩AV在线| 色婷婷久久综合粉嫩影视| 操哭她了正在播放免费| 国产精品久久久白浆| 日韩 欧美 一区 中文字幕| 国产美女和男生一起桶桶的网站| 丝袜美五月| 精昆品三区| 曰b视频免费看| 操 插 在线视频| 一区二区成人激情视频| 狠狠躁夜夜躁人人爽h| 亚洲综合无码30p| 成人超碰成| 啊啊啊恩嗯嗯在线观看| 欧美亚洲校园另类小说| 日韩成人小视频在线观看不卡|