Glucose-6-phosphatase catalytic subunit 1

Definition:

Hydrolyzes glucose-6-phosphate to glucose in the endoplasmic reticulum. Forms with the glucose-6-phosphate transporter (SLC37A4/G6PT) the complex responsible for glucose production in the terminal step of glycogenolysis and gluconeogenesis. Hence, it is the key enzyme in homeostatic regulation of blood glucose levels.

References:

[1]. C J Pan, et al. Transmembrane topology of glucose-6-phosphatase. J Biol Chem. 1998 Mar 13;273(11):6144-8. [Content Brief]

[2]. R Parvari, et al. Glycogen storage disease type 1a in Israel: biochemical, clinical, and mutational studies. Am J Med Genet. 1997 Oct 31;72(3):286-90. [Content Brief]

[3]. B W Weston, et al. Glucose-6-phosphatase mutation G188R confers an atypical glycogen storage disease type 1b phenotype. Pediatr Res. 2000 Sep;48(3):329-34. [Content Brief]

[4]. Celia J Angaroni, et al. Glycogen storage disease type Ia in Argentina: two novel glucose-6-phosphatase mutations affecting protein stability. Mol Genet Metab. 2004 Nov;83(3):276-9. [Content Brief]

[5]. Abhijit Ghosh, et al. The catalytic center of glucose-6-phosphatase. HIS176 is the nucleophile forming the phosphohistidine-enzyme intermediate during catalysis. J Biol Chem. 2002 Sep 6;277(36):32837-42. [Content Brief]

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