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OTC鸟氨酸氨基甲酰转移酶抗体

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  • ¥780 - 2200
  • LMAI Bio
  • LM-6724R
  • 进口/国产
  • 2026年01月06日
  • WB=1:500-2000 ELISA=1:500-1000 IHC-P=1:400-800 IHC-F=1:400-800 IF=1:100-500 (石蜡切片需做抗原修复)
  • Rabbit
  • Human, Mouse, Rat, Dog, Pig, Cow, Horse,
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    • 详细信息
    • 文献和实验
    • 技术资料
    • 供应商

      上海联迈生物工程有限公司

    • 库存

      大量

    • 目录编号

      LM-6724R

    • 克隆性

      多克隆

    • 抗原来源

      Rabbit

    • 保质期

      1年

    • 抗体英文名

      OTC

    • 抗体名

      鸟氨酸氨基甲酰转移酶抗体

    • 宿主

      Rabbit

    • 适应物种

      Human, Mouse, Rat, Dog, Pig, Cow, Horse,

    • 免疫原

      KLH conjugated synthetic peptide derived from human OTC:51-150/354

    • 亚型

      IgG

    • 形态

      Lyophilized or Liquid

    • 应用范围

      WB=1:500-2000 ELISA=1:500-1000 IHC-P=1:400-800 IHC-F=1:400-800 IF=1:100-500 (石蜡切片需做抗原修复)

    • 浓度

      1mg/ml

    • 保存条件

      Store at -20 °C

    • 规格

      50ul  100ul  200ul

    OTC鸟氨酸氨基甲酰转移酶抗体
    英文名称 OTC
    中文名称 鸟氨酸氨基甲酰转移酶抗体
    别    名 OCTD; Ornithine carbamoyltransferase mitochondrial; Ornithine Carbamoyltransferase; Ornithine carbamoyltransferase, mitochondrial; Ornithine transcarbamylase; Otc; OTC_HUMAN; OTCase.  
    规格价格 50ul/780元 购买    100ul/1380元 购买    200ul/2200元 购买    大包装/询价
    说 明 书 50ul  100ul  200ul
    研究领域 细胞生物  信号转导  转录调节因子  线粒体  
    抗体来源 Rabbit
    克隆类型 Polyclonal
    交叉反应 Human, Mouse, Rat, Dog, Pig, Cow, Horse, 
    产品应用 WB=1:500-2000 ELISA=1:500-1000 IHC-P=1:400-800 IHC-F=1:400-800 IF=1:100-500 (石蜡切片需做抗原修复) 
    not yet tested in other applications.
    optimal dilutions/concentrations should be determined by the end user.
    分 子 量 40kDa
    细胞定位 细胞浆 线粒体
    性    状 Lyophilized or Liquid
    浓    度 1mg/ml
    免 疫 原 KLH conjugated synthetic peptide derived from human OTC:51-150/354 
    亚    型 IgG
    纯化方法 affinity purified by Protein A
    储 存 液 0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol.
    保存条件 Store at -20 °C for one year. Avoid repeated freeze/thaw cycles. The lyophilized antibody is stable at room temperature for at least one month and for greater than a year when kept at -20°C. When reconstituted in sterile pH 7.4 0.01M PBS or diluent of antibody the antibody is stable for at least two weeks at 2-4 °C.
    PubMed PubMed
    产品介绍 background:
    Defects in OTC are the cause of ornithine carbamoyltransferase deficiency (OTCD) [MIM:311250]. OTCD is an X-linked disorder of the urea cycle which causes a form of hyperammonemia. Mutations with no residual enzyme activity are always expressed in hemizygote males by a very severe neonatal hyperammonemic coma that generally proves to be fatal. Heterozygous females are either asymptomatic or express orotic aciduria spontaneously or after protein intake. The disorder is treatable with supplemental dietary arginine and low protein diet. The arbitrary classification of patients into the 'neonatal' group (clinical hyperammonemia in the first few days of life) and 'late' onset (clinical presentation after the neonatal period) has been used to differentiate severe from mild forms.

    Subunit:
    Homotrimer.

    Subcellular Location:
    Mitochondrion matrix.

    Tissue Specificity:
    Mainly expressed in liver and intestinal mucosa.

    Post-translational modifications:
    Acetylation at Lys-88 negatively regulates ornithine carbamoyltransferase activity in response to nutrient signals.

    DISEASE:
    Defects in OTC are the cause of ornithine carbamoyltransferase deficiency (OTCD) [MIM:311250]. OTCD is an X-linked disorder of the urea cycle which causes a form of hyperammonemia. Mutations with no residual enzyme activity are always expressed in hemizygote males by a very severe neonatal hyperammonemic coma that generally proves to be fatal. Heterozygous females are either asymptomatic or express orotic aciduria spontaneously or after protein intake. The disorder is treatable with supplemental dietary arginine and low protein diet. The arbitrary classification of patients into the 'neonatal' group (clinical hyperammonemia in the first few days of life) and 'late' onset (clinical presentation after the neonatal period) has been used to differentiate severe from mild forms.

    Similarity:
    Belongs to the ATCase/OTCase family.

    SWISS:
    P00480

    Gene ID:
    5009

    Database links:

    Entrez Gene: 5009 Human

    Entrez Gene: 18416 Mouse

    Entrez Gene: 25611 Rat

    Omim: 300461 Human

    SwissProt: P00480 Human

    SwissProt: P11725 Mouse

    SwissProt: P00481 Rat

    Unigene: 117050 Human

    Unigene: 2611 Mouse

    Unigene: 2391 Rat



    Important Note:
    This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications. 
     
    产品图片 产品细节图片1
    Sample: HepG2 Cell (Human) Lysate at 40 ug
    Primary: Anti-OTC (bs-6724R) at 1/300 dilution
    Secondary: IRDye800CW Goat Anti-Rabbit IgG at 1/20000 dilution
    Predicted band size: 40 kD
    Observed band size: 45 kD

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    图标文献和实验
    相关实验
    • Nat Immunol:华中科技大学黄波团队揭示记忆性 T 细胞通过氨解毒促进记忆形成的机制

      步是转氨基作用介导的谷氨酸或谷氨酰胺的生成,第二步则是谷氨酸或谷氨酰胺的脱氨基作用生成。一般认为,氨在肝脏中通过尿素循环进行处理,肝细胞从外周循环中吸收氨,并使用氨基甲酰磷酸合成酶-1(CPS1)催化氨和碳酸氢盐(HCO3-),在线粒体中形成氨基甲酰磷酸(CP)。然后, CP 与鸟氨酸反应,通过鸟氨酸氨基甲酰磷酸转移酶(OTC)生成瓜氨酸。瓜氨酸之后进入胞浆,通过精氨酸琥珀酸合成酶 1(ASS1)与天冬氨酸结合生成精氨酸,然后精氨酸裂解酶(ASL)将其代谢为精氨酸和延胡索酸最终精氨酸酶 1(ARG

    • 尿素循环(urea cycle)

         亦称鸟氨酸循环,是排尿素动物在肝脏中合成尿素的一个循环机制。肝细胞胞浆中的氨基酸经转氨作用与α -酮戊二酸形成的谷氨酸,透过线粒体膜进入线粒体基质,在谷氨酸脱氢酶作用下脱氨形成游离氨。形成的氨( NH 4 )与三羧酸循环产生的二氧化碳、 2分子 ATP,在氨基甲酰合成酶 I的催化下生成氨基甲酰磷酸。氨基甲酰磷酸在线粒体的鸟氨酸氨基甲酰酶的催化下,将氨基甲酰转移鸟氨酸生成瓜氨酸。瓜氨酸形成后即离开线粒体进入胞浆,在 ATP的存在下,由精氨酸代琥珀酸合成酶的催化

    • 氨的代谢

      3,同时也提供了该反应所需要的能量ATP。氨基甲酰磷酸合成酶I将有毒的氨转变成氨基甲酰磷酸,反应中生成的ADP又是谷氨酸脱氢酶的变构激活剂,促进谷氨酸进一步氧化脱氨。这种紧密偶联有利于迅速将氨固定在肝细胞线粒体内,防止氨逸出线粒体进入细胞浆,进而透过细胞膜进入血液,引起血氨升高。 2.瓜氨酸(citrulline)的生成: 乌氨酸氨基甲酰转移酶(ornithine transcarbamoylase)存在于线粒体中,通常与CPS-I形成酶的复合物催化氨基甲酰磷酸转甲酰基给鸟氨酸生成瓜氨酸

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