glucose-6-phosphatase 3 (G6Pase 3, G-6-Pase 3, ubiquitous glucose-6-phosphatase catalytic subunit-related protein, glucose-6-phosphatase beta, G6Pase-beta, G6PC3, UGRP)
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Function
- hydrolyzes glucose-6-phosphate to glucose in the endoplasmic reticulum
- may form with the glucose-6-phosphate transporter (SLC37A4/G6PT) a ubiquitously expressed complex responsible for glucose production through glycogenolysis & gluconeogenesis
- probably required for normal neutrophil function
- inhibited by vanadate
- carbohydrate biosynthesis; gluconeogenesis
- may not have hydrolytic activity[2]
D-glucose 6-phosphate + H2O <--> D-glucose + phosphate
Kinetic parameters:
- KM=1.0 mM for glucose-6-phosphate (at pH 5.5.)
- KM=2.0 mM for glucose-6-phosphate (at pH 6.5.)
Structure
belongs to the glucose-6-phosphatase family
Compartment
- endoplasmic reticulum membrane
Expression
- ubiquitously expressed
- highly expressed in skeletal muscle, at intermediate levels in heart, brain, placenta, kidney, colon, thymus, spleen & pancreas
- also detected in testis, prostate, ovary, liver, lung, small intestine & peripheral blood lymphocytes
Pathology
- defects associated with congenital neutropenia G6PC3-type
More general terms
References
- ↑ UniProt http://www.uniprot.org/uniprot/Q9BUM1.html
- ↑ 2.0 2.1 Martin CC Identification and characterization of a human cDNA and gene encoding a ubiquitously expressed glucose-6-phosphatase catalytic subunit-related protein. J Mol Endocrinol 2002, 29:205 PMID: https://www.ncbi.nlm.nih.gov/pubmed/12370122
- ↑ Boztug K et al. A syndrome with congenital neutropenia and mutations in G6PC3. N Engl J Med 2009 Jan 1; 360:32. PMID: https://www.ncbi.nlm.nih.gov/pubmed/19118303
Dale DC and Link DC. The many causes of severe congenital neutropenia. N Engl J Med 2009 Jan 1; 360:3. PMID: https://www.ncbi.nlm.nih.gov/pubmed/19118300