| 產(chǎn)品編號(hào) | bs-10020R |
| 英文名稱 | HADHSC Rabbit pAb |
| 中文名稱 | 短鏈L-3羥烷基輔酶A脫氫酶抗體 |
| 別 名 | HAD; HADH; HADH1; HADHSC; HCDH; HCDH_HUMAN; HHF4; Hydroxyacyl CoA dehydrogenase; Hydroxyacyl-coenzyme A dehydrogenase; hydroxyacyl-coenzyme A dehydrogenase, mitochondrial; L 3 hydroxyacyl Coenzyme A dehydrogenase short chain; M SCHAD; Medium and short cha |
| 研究領(lǐng)域 | 腫瘤 細(xì)胞生物 信號(hào)轉(zhuǎn)導(dǎo) 激酶和磷酸酶 糖尿病 新陳代謝 線粒體 |
| 抗體來源 | Rabbit |
| 克隆類型 | Polyclonal |
| 交叉反應(yīng) | Mouse,Rat (predicted: Human,Pig,Cow) |
| 產(chǎn)品應(yīng)用 | WB=1:500-2000,IHC-P=1:100-500,IHC-F=1:100-500,IF=1:100-500
not yet tested in other applications. optimal dilutions/concentrations should be determined by the end user. |
| 理論分子量 | 33 kDa |
| 檢測分子量 | |
| 細(xì)胞定位 | 細(xì)胞漿 線粒體 |
| 性 狀 | Liquid |
| 濃 度 | 1mg/ml |
| 免 疫 原 | KLH conjugated synthetic peptide derived from human HADHSC: 241-314/314 |
| 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(xiàng) | This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications. |
| PubMed | PubMed |
| 產(chǎn)品介紹 |
This gene is a member of the 3-hydroxyacyl-CoA dehydrogenase gene family. The encoded protein functions in the mitochondrial matrix to catalyze the oxidation of straight-chain3-hydroxyacyl-CoAs as part of the beta-oxidation pathway. Its enzymatic activity is highest with medium-chain-length fatty acids. Mutations in this gene cause one form of familial hyperinsulinemic hypoglycemia. The human genome contains a related pseudogene of this gene on chromosome 15. [provided by RefSeq.] Function: Plays an essential role in the mitochondrial beta-oxidation of short chain fatty acids. Exerts it highest activity toward 3-hydroxybutyryl-CoA. Subunit: Homodimer. Subcellular Location: Mitochondrion matrix. Tissue Specificity: Expressed in liver, kidney, pancreas, heart and skeletal muscle. DISEASE: Defects in HADH are the cause of 3-alpha-hydroxyacyl-CoA dehydrogenase deficiency (HADH deficiency) [MIM:231530]. HADH deficiency is a metabolic disorder with various clinical presentations including hypoglycemia, hepatoencephalopathy, myopathy or cardiomyopathy, and in some cases sudden death. Defects in HADH are the cause of familial hyperinsulinemic hypoglycemia type 4 (HHF4) [MIM:609975]; also known as persistent hyperinsulinemic hypoglycemia of infancy (PHHI) or congenital hyperinsulinism. HHF is the most common cause of persistent hypoglycemia in infancy and is due to defective negative feedback regulation of insulin secretion by low glucose levels. It causes nesidioblastosis, a diffuse abnormality of the pancreas in which there is extensive, often disorganized formation of new islets. Unless early and aggressive intervention is undertaken, brain damage from recurrent episodes of hypoglycemia may occur. HHF4 should be easily recognizable by analysis of acylcarnitine species and that this disorder responds well to treatment with diazoxide. It provides the first 'experiment of nature' that links impaired fatty acid oxidation to hyperinsulinism and that provides support for the concept that a lipid signaling pathway is implicated in the control of insulin secretion. Similarity: Belongs to the 3-hydroxyacyl-CoA dehydrogenase family. SWISS: Q16836 Gene ID: 3033 Database links: Entrez Gene: 3033 Human Entrez Gene: 15107 Mouse Omim: 601609 Human SwissProt: Q16836 Human SwissProt: Q61425 Mouse Unigene: 438289 Human Unigene: 260164 Mouse
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| 產(chǎn)品圖片 |
Sample:
Lane 1: Mouse Liver tissue lysates
Lane 2: Mouse Kidney tissue lysates
Lane 3: Mouse Heart tissue lysates
Lane 4: Mouse Pancreas tissue lysates
Primary: Anti-HADHSC (bs-10020R) at 1/1000 dilution
Secondary: IRDye800CW Goat Anti-Rabbit IgG at 1/20000 dilution
Predicted band size: 33 kDa
Observed band size: 34 kDa
Tissue/cell: mouse fetal liver; 4% Paraformaldehyde-fixed and paraffin-embedded;
Antigen retrieval: citrate buffer ( 0.01M, pH 6.0 ), Boiling bathing for 15min; Block endogenous peroxidase by 3% Hydrogen peroxide for 30min; Blocking buffer (normal goat serum,C-0005) at 37℃ for 20 min;
Incubation: Anti-HADHSC Polyclonal Antibody, Unconjugated(bs-10020R) 1:200, overnight at 4°C, followed by conjugation to the secondary antibody(SP-0023) and DAB(C-0010) staining
Paraformaldehyde-fixed, paraffin embedded (Rat pancreas); Antigen retrieval by boiling in sodium citrate buffer (pH6.0) for 15min; Block endogenous peroxidase by 3% hydrogen peroxide for 20 minutes; Blocking buffer (normal goat serum) at 37°C for 30min; Antibody incubation with (HADHSC) Polyclonal Antibody, Unconjugated (bs-10020R) at 1:400 overnight at 4°C, followed by operating according to SP Kit(Rabbit) (sp-0023) instructionsand DAB staining.
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