8j7o | pdb_00008j7o

Human pyruvate carboxylase in BCCP-CTS state without BC

spinqualitylabelsall models 8j7o, resolution 3.83Å Show:Asymmetric Unit Biological Assembly Drag the structure with the mouse to rotate   Export Animated Image   Print3D Structural highlights 8j7o is a 4 chain structure with sequence from Homo sapiens. Full crystallographic information is available from OCA. For a guided tour on the structure components use FirstGlance. Method: Electron Microscopy, Resolution 3.83Å Ligands: BTI Resources: FirstGlance, OCA, PDBe, RCSB, PDBsum, ProSAT Disease PYC_HUMAN Defects in PC are the cause of pyruvate carboxylase deficiency (PC deficiency) [MIM:266150. PC deficiency leads to lactic acidosis, mental retardation and death. It occurs in three forms: mild or type A, severe neonatal or type B, and a very mild lacticacidemia. Function PYC_HUMAN Pyruvate carboxylase catalyzes a 2-step reaction, involving the ATP-dependent carboxylation of the covalently attached biotin in the first step and the transfer of the carboxyl group to pyruvate in the second. Catalyzes in a tissue specific manner, the initial reactions of glucose (liver, kidney) and lipid (adipose tissue, liver, brain) synthesis from pyruvate.

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