Recombinant人PCK1 protein (ab114362)

概述

描述

  • 性质Recombinant
  • 来源Wheat germ
  • 氨基酸序列
    • AccessionP35558
    • 种属Human
    • 序列MPPQLQNGLNLSAKVVQGSLDSLPQAVREFLENNAELCQPDHIHICDGSE EENGRLLGQMEEEGILRRLKKYDNCWLALTDPRDVARIESKTVIVTQEQR DTVPIPKTGLSQLGRWMSEEDFEKAFNARFPGCMKGRTMYVIPFSMGPLG SPLSKIGIELTDSPYVVASMRIMTRMGTPVLEALGDGEFVKCLHSVGCPL PLQKPLVNNWPCNPELTLIAHLPDRREIISFGSGYGGNSLLGKKCFALRM ASRLAKEEGWLAEHMLVLGITNPEGEKKYLAAAFPSACGKTNLAMMNPSL PGWKVECVGDDIAWMKFDAQGHLRAINPENGFFGVAPGTSVKTNPNAIKT IQKNTIFTNVAETSDGGVYWEGIDEPLASGVTITSWKNKEWSSEDGEPCA HPNSRFCTPASQCPIIDAAWESPEGVPIEGIIFGGRRPAGVPLVYEALSW QHGVFVGAAMRSEATAAAEHKGKIIMHDPFAMRPFFGYNFGKYLAHWLSM AQHPAAKLPKIFHVNWFRKDKEGKFLWPGFGENSRVLEWMFNRIDGKAST KLTPIGYIPKEDALNLKGLGHINMMELFSISKEFWEKEVEDIEKYLEDQV NADLPCEIEREILALKQRISQM
    • 分子量95 kDa including tags
    • 氨基酸1 to 623

技术指标

Our Abpromise guarantee covers the use of ab114362 in the following tested applications.

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

  • 应用

    ELISA

    SDS-PAGE

    Western blot

  • 形式Liquid
  • 补充说明Protein concentration is above or equal to 0.05 mg/ml. Best use within three months from the date of receipt of this protein.
  • 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: 0.3% Glutathione, 0.79% Tris HCl

常规信息

  • 别名
    • cytosolic [GTP]
    • GTP
    • PCK1
    • PCKGC_HUMAN
    • PEP carboxykinase
    • PEPCK-C
    • PEPCK1
    • PEPCKC
    • Phosphoenolpyruvate carboxykinase
    • Phosphoenolpyruvate carboxykinase 1
    • Phosphoenolpyruvate carboxykinase 1 (soluble)
    • Phosphoenolpyruvate carboxykinase, cytosolic
    • Phosphoenolpyruvate carboxykinase, cytosolic [GTP]
    • Phosphoenolpyruvate carboxylase
    • Phosphopyruvate carboxylase
    see all
  • 功能Catalyzes the conversion of oxaloacetate (OAA) to phosphoenolpyruvate (PEP), the rate-limiting step in the metabolic pathway that produces glucose from lactate and other precursors derived from the citric acid cycle.
  • 组织特异性Major sites of expression are liver, kidney and adipocytes.
  • 通路Carbohydrate biosynthesis; gluconeogenesis.
  • 疾病相关Defects in PCK1 are the cause of cytosolic phosphoenolpyruvate carboxykinase deficiency (cytosolic PEPCK deficiency) [MIM:261680]. PEPCK deficiency is a metabolic disorder resulting from impaired gluconeogenesis. It is a rare disease with less than 10 cases reported in the literature. Clinical characteristics include hypotonia, hepatomegaly, failure to thrive, lactic acidosis and hypoglycemia. Autoposy reveals fatty infiltration of both the liver and kidneys. The disorder is transmitted as an autosomal recessive trait.
  • 序列相似性Belongs to the phosphoenolpyruvate carboxykinase [GTP] family.
  • 翻译后修饰Acetylation is increased on addition of glucose and appears to regulate the protein stability.
  • 细胞定位Cytoplasm.
  • Information by UniProt

Recombinant Human PCK1 protein 图像

  • 12.5% SDS-PAGE showing ab114362 at approximately 94.53kDa stained with Coomassie Blue.

Recombinant Human PCK1 protein (ab114362)参考文献

ab114362 has not yet been referenced specifically in any publications.

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