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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)

CSB-EP017270HUc7

(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)

CSB-PA017270LA01HU

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.

CSB-PA017270LA01HU

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)

CSB-EL017270HU 標(biāo)準(zhǔn)曲線

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)

OTC Proteins

OTC Proteins for Homo sapiens (Human)

OTC ELISA Kit

OTC ELISA Kit for Homo sapiens (Human)



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