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MCCA rabbit pAb

商品貨號(hào)： PLA020632

適應(yīng) 性： 人,小鼠,大鼠

￥600元

規(guī)格：

40μL 100μL 200μL

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MSDS

說明書

商品描述

基因名稱： MCCC1 MCCA
蛋白名稱： MCCA
Human_gene_id： 56922
Human_gene_link： http://www.ncbi.nlm.nih.gov/sites/entrez?db=gene&term=56922
Human_swiss_prot_no： Q96RQ3
Human_swiss_link： https://www.uniprot.org/uniprotkb/Q96RQ3/entry
Mouse_gene_id： 72039
Mouse_gene_link： https://www.uniprot.org/uniprot/72039
Mouse_swiss_prot_no： Q99MR8
Mouse_swiss_link： https://www.uniprot.org/uniprotkb/Q99MR8
Rat_gene_id： 294972
Rat_gene_link： https://www.uniprot.org/uniprot/294972
Rat_swiss_prot_no： Q5I0C3
Rat_swiss_link： https://www.uniprot.org/uniprotkb/Q5I0C3
特異性： This antibody detects endogenous levels of MCCA at Human/Mouse/Rat
組成： Liquid in PBS containing 50% glycerol, 0.5% BSA and 0.02% sodium azide.
來源： Polyclonal, Rabbit,IgG
稀釋： WB 1:500-2000;IHC-p 1:50-300
純化工藝： The antibody was affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogen.
濃度： 1 mg/ml
儲(chǔ)存： -15°C to -25°C/1 year(Do not lower than -25°C)
說明書： 1
Msds： MSDS_Antibody.pdf
分子量： 80kD
功能： catalytic activity:ATP + 3-methylcrotonoyl-CoA + HCO(3)(-) = ADP + phosphate + 3-methylglutaconyl-CoA.,cofactor:Biotin.,disease:Defects in MCCC1 are the cause of methylcrotonoyl-CoA carboxylase deficiency type 1 (MCC1 deficiency) [MIM:210200]. MCC1 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.,pathway:Amino-acid degradation; L-leucine degradation; HMG-CoA from 3-isovaleryl-CoA: step 2/3.,similarity:Contains 1 ATP-grasp domain.,similarity:Contains 1 biotin carboxylation domain.,similarity:Contains 1 biotinyl-binding domain.,subunit:Probably a dodecamer composed of six biotin-containing alpha subunits and six beta subunits.,
相關(guān)產(chǎn)品： RS0001,RS0002,YM3028,YM3029
細(xì)胞定位： Mitochondrion matrix .
科研貨號(hào)： PLA020632

MCCA rabbit pAb

Catalog No PLA020632

Product information

基因名稱： MCCC1 MCCA
蛋白名稱： MCCA
Human_gene_id： 56922
Human_gene_link： http://www.ncbi.nlm.nih.gov/sites/entrez?db=gene&term=56922
Human_swiss_prot_no： Q96RQ3
Human_swiss_link： https://www.uniprot.org/uniprotkb/Q96RQ3/entry
Mouse_gene_id： 72039
Mouse_gene_link： https://www.uniprot.org/uniprot/72039
Mouse_swiss_prot_no： Q99MR8
Mouse_swiss_link： https://www.uniprot.org/uniprotkb/Q99MR8
Rat_gene_id： 294972
Rat_gene_link： https://www.uniprot.org/uniprot/294972
Rat_swiss_prot_no： Q5I0C3
Rat_swiss_link： https://www.uniprot.org/uniprotkb/Q5I0C3
特異性： This antibody detects endogenous levels of MCCA at Human/Mouse/Rat
組成： Liquid in PBS containing 50% glycerol, 0.5% BSA and 0.02% sodium azide.
來源： Polyclonal, Rabbit,IgG
稀釋： WB 1:500-2000;IHC-p 1:50-300
純化工藝： The antibody was affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogen.
濃度： 1 mg/ml
儲(chǔ)存： -15°C to -25°C/1 year(Do not lower than -25°C)
說明書： 1
Msds： MSDS_Antibody.pdf
分子量： 80kD
功能： catalytic activity:ATP + 3-methylcrotonoyl-CoA + HCO(3)(-) = ADP + phosphate + 3-methylglutaconyl-CoA.,cofactor:Biotin.,disease:Defects in MCCC1 are the cause of methylcrotonoyl-CoA carboxylase deficiency type 1 (MCC1 deficiency) [MIM:210200]. MCC1 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.,pathway:Amino-acid degradation; L-leucine degradation; HMG-CoA from 3-isovaleryl-CoA: step 2/3.,similarity:Contains 1 ATP-grasp domain.,similarity:Contains 1 biotin carboxylation domain.,similarity:Contains 1 biotinyl-binding domain.,subunit:Probably a dodecamer composed of six biotin-containing alpha subunits and six beta subunits.,
相關(guān)產(chǎn)品： RS0001,RS0002,YM3028,YM3029
細(xì)胞定位： Mitochondrion matrix .
科研貨號(hào)： PLA020632

Hunan UPT Biotechnology Co.,Ltd

Website：m.tjdqdt.com Servive hotline :4006916686

E-mail：service@uptbio.com

Address：

Room 402, Building 13, Xinggong International Industrial Park, 100 Guyuan Road, Yuelu District, Changsha City, Hunan Province, China.

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