Recombinant Human PCK1/PEPC Protein (Active)

Recombinant Human PCK1/PEPC Protein (Active)

Cat. No.: DPP-001130

Size: 50 µg Size: 100 µg Size: Costomer Size
Product Overview
Species Human
Expression System Escherichia coli
Format Lyophilized
Purity ≥ 70% by HPLC
Nature Recombinant
Target Information
Gene Name PCK1
UniProt No. P35558
Gene ID 5105
Molecular Weight 71 kDa including tags
Alternative Names 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
Function 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.
Involvement In Disease Defects in PCK1 are the cause of cytosolic phosphoenolpyruvate carboxykinase deficiency (cytosolic PEPCK deficiency). 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.
Cellular Localization Cytoplasm.
Protein Length Full length protein
Sequence PPQLQNGLNLSAKVVQGSLDSLPQAVREFLENNAELCQPDHIHICDGSEE ENGRLLGQMEEEGILRRLKKYDNCWLALTDPRDVARIESKTVIVTQEQRD TVPIPKTGLSQLGRWMSEEDFEKAFNARFPGCMKGRTMYVIPFSMGPLGS PLSKIGIELTDSPYVVASMRIMTRMGTPVLEAVGDGEFVKCLHSVGCPLP LQKPLVNNWPCNPELTLIAHLPDRREIISFGSGYGGNSLLGKKCFALRMA SRLAKEEGWLAEHMLILGITNPEGEKKYLAAAFPSACGKTNLAMMNPSLP GWKVECVGDDIAWMKFDAQGHLRAINPENGFFGVAPGTSVKTNPNAIKTI QKNTIFTNVAETSDGGVYWEGIDEPLASGVTITSWKNKEWSSEDGEPCAH PNSRFCTPASQCPIIDAAWESPEGVPIEGIIFGGRRPAGVPLVYEALSWQ HGVFVGAAMRSEATAAAEHKGKIIMHDPFAMRPFFGYNFGKYLAHWLSMA QHPAAKLPKIFHVNWFRKDKEGKFLWPGFGENSRVLEWMFNRIDGKASTK LTPIGYIPKEDALNLKGLGHINMMELFSISKEFWEKEVEDIEKYLEDQVN ADLPCEIEREILALKQRISQM,Belongs to the phosphoenolpyruvate carboxykinase [GTP] family.
Shipping & Storage
Shipping Shipped on dry ice.
Storage Store at +4°C short term (1-2 weeks). Upon delivery aliquot. Store at -20°C or -80°C. Avoid freeze / thaw cycle.
All of our services and products are intended for preclinical research use only and cannot be used to diagnose, treat or manage patients.
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