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ACADM Recombinant Rabbit mAb (bsm-63329R)  
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產品編號 bsm-63329R
英文名稱 ACADM Recombinant Rabbit mAb
中文名稱 酰基輔酶A脫氫酶中鏈重組兔單抗
別    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 dehydrogenase; Medium chain specific acyl CoA dehydrogenase mitochondrial; FLJ18227; FLJ93013; FLJ99884; ACADM_HUMAN.  
抗體來源 Rabbit
克隆類型 Recombinant
克 隆 號 5C9
交叉反應 Human
產品應用 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.
理論分子量 47 kDa
檢測分子量
細胞定位 細胞膜 
性    狀 Liquid
濃    度 1mg/ml
免 疫 原 A synthesized peptide derived from human ACADM: 200-265/421 
亞    型 IgG
純化方法 affinity purified by Protein A
緩 沖 液 0.01M TBS (pH7.4) with 1% BSA, 0.02% Proclin300 and 50% Glycerol.
保存條件 Shipped at 4℃. Store at -20℃ for one year. Avoid repeated freeze/thaw cycles.
注意事項 This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.
PubMed PubMed
產品介紹 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



酰基輔酶,是一種特殊的酰基鏈長度為4-16的脫氫酶,它催化脂肪酸β氧化的起始步驟。利用電子轉移黃蛋白(ETF)作為電子受體,通過ETF-泛醌氧化還原酶(ETF脫氫酶)將電子轉移到線粒體呼吸鏈。
產品圖片
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): 1:50-100/10^6 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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