OTC
OTC,全稱為鳥氨酸氨甲酰轉(zhuǎn)移酶,常用別名鳥氨酸轉(zhuǎn)氨甲酰酶。它主要在肝臟中大量表達(dá),是尿素循環(huán)里的關(guān)鍵代謝物質(zhì),能夠參與體內(nèi)多余氨的代謝清除,把有毒的氨轉(zhuǎn)化為尿素排出體外,對(duì)維持體內(nèi)氮代謝平衡、避免氨中毒起到不可替代的作用。
OTC功能缺陷會(huì)引發(fā)先天性尿素循環(huán)障礙,也就是鳥氨酸氨甲酰轉(zhuǎn)移酶缺乏癥,患者體內(nèi)氨大量堆積,易出現(xiàn)神經(jīng)系統(tǒng)損傷、發(fā)育遲緩甚至昏迷,是常見的遺傳性代謝病。目前臨床以低蛋白飲食、排氨藥物對(duì)癥控制為主,酶替代療法與肝臟基因治療處于臨床研究階段,可有效改善體內(nèi)氨代謝異常,為這類罕見遺傳病帶來長期治療新方向。
熱銷產(chǎn)品
Recombinant Human Ornithine transcarbamylase, mitochondrial (OTC) (CSB-EP017270HUc7)
驗(yàn)證數(shù)據(jù)
(Tris-Glycine gel) Discontinuous SDS-PAGE (reduced) with 5% enrichment gel and 15% separation gel.
OTC Antibody (CSB-PA017270LA01HU)
驗(yàn)證數(shù)據(jù)
IHC image of CSB-PA017270LA01HU diluted at 1:400 and staining in paraffin-embedded human liver tissue performed on a Leica BondTM system. After dewaxing and hydration, antigen retrieval was mediated by high pressure in a citrate buffer (pH 6.0). Section was blocked with 10% normal goat serum 30min at RT. Then primary antibody (1% BSA) was incubated at 4°C overnight. The primary is detected by a biotinylated secondary antibody and visualized using an HRP conjugated SP system.
IHC image of CSB-PA017270LA01HU diluted at 1:400 and staining in paraffin-embedded human liver cancer performed on a Leica BondTM system. After dewaxing and hydration, antigen retrieval was mediated by high pressure in a citrate buffer (pH 6.0). Section was blocked with 10% normal goat serum 30min at RT. Then primary antibody (1% BSA) was incubated at 4°C overnight. The primary is detected by a biotinylated secondary antibody and visualized using an HRP conjugated SP system.
Human Ornithine carbamoyltransferase, mitochondrial(OTC) ELISA kit (CSB-EL017270HU)
驗(yàn)證數(shù)據(jù)
貨號(hào):CSB-EL017270HU
規(guī)格:96T/48T
靈敏度:0.312 mU/mL
檢測范圍:1.25 mU/mL-80 mU/mL
These standard curves are provided for demonstration only. A standard curve should be generated for each set of samples assayed.
OTC Antibodies
OTC for Homo sapiens (Human)
| 產(chǎn)品貨號(hào) | 產(chǎn)品名稱 | 種屬反應(yīng)性 | 應(yīng)用類型 |
|---|---|---|---|
| CSB-PA017270LA01HU | OTC Antibody | Human | ELISA, IHC |
| CSB-PA017270LB01HU | OTC Antibody, HRP conjugated | Human | ELISA |
| CSB-PA017270LC01HU | OTC Antibody, FITC conjugated | Human | |
| CSB-PA189809 | OTC Antibody | Human,Mouse,Rat | ELISA,WB,IHC |
| CSB-PA033931 | OTC Antibody | Human,Mouse,Rat | ELISA,WB,IHC |
OTC Proteins
OTC Proteins for Homo sapiens (Human)
| 產(chǎn)品貨號(hào) | 產(chǎn)品名稱 | 來源 |
|---|---|---|
| CSB-YP017270HU CSB-BP017270HU CSB-MP017270HU CSB-EP017270HU-B |
Recombinant Human Ornithine carbamoyltransferase, mitochondrial (OTC) | Yeast Baculovirus Mammalian cell In Vivo Biotinylation in E.coli |
| CSB-EP017270HU | Recombinant Human Ornithine transcarbamylase, mitochondrial (OTC) | E.coli |
| CSB-EP017270HUc7 | Recombinant Human Ornithine transcarbamylase, mitochondrial (OTC) | E.coli |
OTC ELISA Kit
OTC ELISA Kit for Homo sapiens (Human)
| 產(chǎn)品貨號(hào) | 產(chǎn)品名稱 | 樣本類型 | 靈敏度 |
|---|---|---|---|
| CSB-EL017270HU | Human Ornithine carbamoyltransferase, mitochondrial(OTC) ELISA kit | serum, plasma, tissue homogenates, cell lysates | 0.312 mU/mL |




