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ACADM Recombinant Rabbit mAb (bsm-63329R)  
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25ul/800.00元
50ul/1400.00元
100ul/2500.00元
大包装/询价

产品编号 bsm-63329R
英文名称 ACADM Recombinant Rabbit mAb
中文名称 酰基辅酶A脱氢酶中链重组兔单抗
别    名 mitochondrial antibody; ACAD 1; ACAD1; Acyl coenzyme A dehydrogenase; Acyl coenzyme A dehydrogenase C 4 to C 12 straight chain; MCAD; MCADH; Medium chain acyl CoA dehydrogenase; Medium chain fatty acyl CoA dehydrogenase; Medium chain specific acyl CoA deh  
抗体来源 Rabbit
克隆类型 Recombinant
克 隆 号 5C9
交叉反应 Human
产品应用 IHC-P=1:50-200,IHC-F=1:50-200,IF=1:50-200,Flow-Cyt=1:50-100
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.
理论分子量 47 kDa
检测分子量
细胞定位 细胞膜 
性    状 Liquid
浓    度 1mg/ml
免 疫 原 KLH conjugated synthetic peptide derived from human ACADM 
亚    型 IgG
纯化方法 affinity purified by Protein A
缓 冲 液 0.01M TBS (pH7.4) with 1% BSA, 0.02% Proclin300 and 50% Glycerol.
保存条件 Shipped at 4℃. Store at -20℃ for one year. Avoid repeated freeze/thaw cycles.
注意事项 This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.
PubMed PubMed
产品介绍 This gene encodes the medium-chain specific (C4 to C12 straight chain) acyl-Coenzyme A dehydrogenase. The homotetramer enzyme catalyzes the initial step of the mitochondrial fatty acid beta-oxidation pathway. Defects in this gene cause medium-chain acyl-CoA dehydrogenase deficiency, a disease characterized by hepatic dysfunction, fasting hypoglycemia, and encephalopathy, which can result in infantile death. Alternatively spliced transcript variants encoding different isoforms have been found for this gene. [provided by RefSeq, Jul 2008]

Function:
This enzyme is specific for acyl chain lengths of 4 to 16.

Subunit:
Homotetramer. Interacts with the heterodimeric electron transfer flavoprotein ETF.

Subcellular Location:
Mitochondrion matrix.

DISEASE:
Acyl-CoA dehydrogenase medium-chain deficiency (ACADMD) [MIM:201450]: An inborn error of mitochondrial fatty acid beta-oxidation which causes fasting hypoglycemia, hepatic dysfunction and encephalopathy, often resulting in death in infancy. Note=The disease is caused by mutations affecting the gene represented in this entry.

Similarity:
Belongs to the acyl-CoA dehydrogenase family.

SWISS:
P11310

Gene ID:
34

Database links:

Entrez Gene : 34 Human

SwissProt : P11310 Human



酰基辅酶,是一种特殊的酰基链长度为4-16的脱氢酶,它催化脂肪酸β氧化的起始步骤。利用电子转移黄蛋白(ETF)作为电子受体,通过ETF-泛醌氧化还原酶(ETF脱氢酶)将电子转移到线粒体呼吸链。
产品图片
Paraformaldehyde-fixed, paraffin embedded Human Heart; Antigen retrieval by boiling in sodium citrate buffer (pH6.0) for 15 min; The section was incubated with ACADM Monoclonal Antibody, Unconjugated (bsm-63329R ) at 1:200 overnight at 4°C, followed by conjugation to the bs-0295G-HRP and DAB (C-0010) staining.
Paraformaldehyde-fixed, paraffin embedded Human Stomach; Antigen retrieval by boiling in sodium citrate buffer (pH6.0) for 15 min; The section was incubated with ACADM Monoclonal Antibody, Unconjugated (bsm-63329R ) at 1:200 overnight at 4°C, followed by conjugation to the bs-0295G-HRP and DAB (C-0010) staining.
Paraformaldehyde-fixed, paraffin embedded Human Liver; Antigen retrieval by boiling in sodium citrate buffer (pH6.0) for 15 min; The section was incubated with ACADM Monoclonal Antibody, Unconjugated (bsm-63329R ) at 1:200 overnight at 4°C, followed by conjugation to the bs-0295G-HRP and DAB (C-0010) staining.
Paraformaldehyde-fixed, paraffin embedded Human Kidney; Antigen retrieval by boiling in sodium citrate buffer (pH6.0) for 15 min; The section was incubated with ACADM Monoclonal Antibody, Unconjugated (bsm-63329R ) at 1:200 overnight at 4°C, followed by conjugation to the bs-0295G-HRP and DAB (C-0010) staining.
The Hela (H) cells were fixed with 4% PFA (10 min at r.t.) and then permeabilized with 90% ice-cold methanol for 20 min at -20℃,the cells then were incubated in 5%BSA to block non-specific protein-protein interactions (30 min at r.t.).Primary Antibody (green):Rabbit Anti-ACADM antibody (bsm-63329R): 1 μg/10^6 cells; Secondary Antibody (white blue): Goat anti-Rabbit IgG-BF488 (bs-60295G-BF488): 1 μg/test. Isotype Control (orange): Rabbit IgG (bs-0295P). Blank control (black): PBS. Acquisition of 20,000 events was performed.
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