Glucose-6-phosphatase Catalytic Subunit Gene Family

Abstract

Glucose-6-phosphatase catalyzes the hydrolysis of glucose 6-phosphate (G6P) to glucose and inorganic phosphate. It is a multicomponent system located in the endoplasmic reticulum that comprises several integral membrane proteins, namely a catalytic subunit (G6PC) and transporters for G6P, inorganic phosphate, and glucose. The G6PC gene family contains three members, designated G6PC, G6PC2, and G6PC3. The tissuespecific expression patterns of these genes differ, and mutations in all three genes have been linked to distinct diseases in humans. This minireview discusses the disease association and transcriptional regulation of the G6PC genes as well as the biological functions of the encoded proteins.

Mechanisms of transcriptional control of glucose metabolism in hepatocytes

“…Как и в случае с ФЕП-карбоксикиназой, регуляция активности глюкозо-6-фосфатазы осущест-вляется не посттрансляционно, а посредством измене-ния уровня ее экспрессии. Промотор гена этого фермента несет многочисленные участки связывания с гормончув-ствительными транскрипционными факторами и коакти-ваторами [12] (рис. 1) Регуляция глюконеогенеза может осуществляться двумя путями: посттрансляционно и претрансляционно.…”

<b>Importance of Hepatocyte Nuclear Factor 4α in Glycerol-induced Glucose-6-phosphatase Expression in </b><b>Liver </b>

“…In accord with this, it has been reported that HNF4α plays a critical role in fatty acid-induced DISCUSSION Gluconeogenesis is determined primarily by G6Pase and PEPCK, two major enzymes involved in the rate limiting steps of this pathway. As G6Pase, as well as PEPCK, regulates gluconeogenesis at the levels of transcription, the molecular mechanism of G6pc transcription has been studied extensively (9,10,19,24). G6pc transcription is known to be modulated by nutrients, metabolites, and hormones (8,11,13,18).…”

Pathogenic mechanisms and clinical implications of congenital neutropenia syndromes

“…However, the current mutational spectrum in a limited number of patients has not yet allowed a clear genotypephenotype correlation [160 && ]. G6PC3 is a ubiquitously expressed metabolic enzyme of the glucose 6 phosphatase (G6P) family, that is G6PC1-3, which localizes to the endoplasmic reticulum membrane where it is supposed to form a functional complex with glucose-6-phosphate transporter (G6PT), also known as solute carrier family 37 member 4 (SLC37A4) [161,162]. Neutrophil granulocyte G6PC3 deficiency deranges glucose recycling between the endoplasmic reticulum and the cytoplasm and leads to reduced levels of glucose uptake and its energy-carrying catabolites obtained by glycolysis [163].…”