概述

  • 产品名称
    重组人CPS1蛋白
  • 蛋白长度
    Protein fragment

描述

  • 性质
    Recombinant
  • 来源
    Wheat germ
  • 氨基酸序列
    • Accession
    • 种属
      Human
    • 序列
      ANNVPATPVAWPSQEGQNPSLSSIRKLIRDGSIDLVINLPNNNTKFVHDN YVIRRTAVDSGIPLLTNFQVTKLFAEAVQKSRKVDSKSLFHYRQYSAGKA A
    • 分子量
      37 kDa including tags
    • 氨基酸
      1400 to 1500

相关产品

技术指标

Our Abpromise guarantee covers the use of ab152296 in the following tested applications.

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

  • 应用

    Western blot

    SDS-PAGE

    ELISA

  • 形式
    Liquid
  • 补充说明
    Protein concentration is above or equal to 0.05 µg/µl.
  • Concentration information loading...

制备和贮存

  • 稳定性和存储

    Shipped on dry ice. Upon delivery aliquot and store at -80ºC. Avoid freeze / thaw cycles.

    pH: 8.00
    Constituents: 0.31% Glutathione, 0.79% Tris HCl

常规信息

  • 别名
    • Carbamoyl phosphate synthase
    • Carbamoyl phosphate synthase [ammonia]
    • Carbamoyl phosphate synthase [ammonia] mitochondrial
    • Carbamoyl phosphate synthetase 1
    • Carbamoyl phosphate synthetase 1 mitochondrial
    • Carbamoyl phosphate synthetase I
    • Carbamoyl-phosphate synthase [ammonia]
    • Carbamoyl-phosphate synthetase I
    • Carbamoylphosphate synthase
    • Carbamoylphosphate synthetase 1
    • Carbamoylphosphate synthetase I
    • CPS 1
    • Cps1
    • CPSase 1
    • CPSase I
    • CPSASE1
    • CPSM_HUMAN
    • mitochondrial
    • MS738
    see all
  • 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

图片

  • 12.5% SDS-PAGE showing ab152296 stained with Coomassie Blue.

文献

ab152296 has not yet been referenced specifically in any publications.

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