概述

  • 产品名称Anti-CPS1抗体
    参阅全部 CPS1 一抗
  • 描述
    兔多克隆抗体to CPS1
  • 经测试应用适用于: WBmore details
  • 种属反应性
    与反应: Human
  • 免疫原

    Synthetic peptide conjugated to KLH, corresponding to a region within N-terminal amino acids 261-291 of Human CPS1 (NP_001116105.1, NP_001866.2, NP_001116106.1).

  • 阳性对照
    • Hela cell line lysate

性能

应用

Our Abpromise guarantee covers the use of ab123859 in the following tested applications.

The application notes include recommended starting dilutions; optimal dilutions/concentrations should be determined by the end user.

应用 Ab评论 说明
WB 1/100 - 1/500. Predicted molecular weight: 165 kDa.

靶标

  • 功能Involved in the urea cycle of ureotelic animals where the enzyme plays an important role in removing excess ammonia from the cell.
  • 组织特异性Primarily in the liver and small intestine.
  • 疾病相关Defects in CPS1 are the cause of carbamoyl phosphate synthetase 1 deficiency (CPS1D) [MIM:237300]. CPS1D is an autosomal recessive disorder of the urea cycle causing hyperammonemia. Clinical features include protein intolerance, intermittent ataxia, seizures, lethargy, developmental delay and mental retardation.
    Note=Genetic variations in CPS1 influence the availability of precursors for nitric oxide (NO) synthesis and play a role in clinical situations where endogenous NO production is critically important, such as neonatal pulmonary hypertension, increased pulmonary artery pressure following surgical repair of congenital heart defects or hepatovenocclusive disease following bone marrow transplantation. Infants with neonatal pulmonary hypertension homozygous for Thr-1406 have lower L-arginine concentrations than neonates homozygous for Asn-1406.
  • 序列相似性Contains 2 ATP-grasp domains.
    Contains 1 glutamine amidotransferase type-1 domain.
  • 结构域The type-1 glutamine amidotransferase domain is defective.
  • 细胞定位Mitochondrion.
  • Information by UniProt
  • 数据库链接
  • 别名
    • Carbamoyl phosphate synthase [ammonia] antibody
    • Carbamoyl phosphate synthase [ammonia] mitochondrial antibody
    • Carbamoyl phosphate synthase antibody
    • Carbamoyl phosphate synthetase 1 antibody
    • Carbamoyl phosphate synthetase 1 mitochondrial antibody
    • Carbamoyl phosphate synthetase I antibody
    • Carbamoyl-phosphate synthase [ammonia] antibody
    • Carbamoyl-phosphate synthetase I antibody
    • Carbamoylphosphate synthase antibody
    • Carbamoylphosphate synthetase 1 antibody
    • Carbamoylphosphate synthetase I antibody
    • CPS 1 antibody
    • Cps1 antibody
    • CPSase 1 antibody
    • CPSase I antibody
    • CPSASE1 antibody
    • CPSM_HUMAN antibody
    • mitochondrial antibody
    • MS738 antibody
    see all

Anti-CPS1 antibody 图像

  • Anti-CPS1 antibody (ab123859) at 1/100 dilution + Hela cell line lysate at 35 µg

    Predicted band size : 165 kDa

Anti-CPS1 antibody (ab123859)参考文献

ab123859 has not yet been referenced specifically in any publications.

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