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The SLC37 family of phosphate-linked sugar phosphate antiporters
- Source :
- Molecular Aspects of Medicine. 34:601-611
- Publication Year :
- 2013
- Publisher :
- Elsevier BV, 2013.
-
Abstract
- The SLC37 family consists of four sugar-phosphate exchangers, A1, A2, A3, and A4, which are anchored in the endoplasmic reticulum (ER) membrane. The best characterized family member is SLC37A4, better known as the glucose-6-phosphate (G6P) transporter (G6PT). SLC37A1, SLC37A2, and G6PT function as phosphate (Pi)-linked G6P antiporters catalyzing G6P:Pi and Pi:Pi exchanges. The activity of SLC37A3 is unknown. G6PT translocates G6P from the cytoplasm into the lumen of the ER where it couples with either glucose-6-phosphatase-α (G6Pase-α) or G6Pase-β to hydrolyze intraluminal G6P to glucose and Pi. The functional coupling of G6PT with G6Pase-α maintains interprandial glucose homeostasis and the functional coupling of G6PT with G6Pase-β maintains neutrophil energy homeostasis and functionality. A deficiency in G6PT causes glycogen storage disease type Ib, an autosomal recessive disorder characterized by impaired glucose homeostasis, neutropenia, and neutrophil dysfunction. Neither SLC37A1 nor SLC37A2 can functionally couple with G6Pase-α or G6Pase-β, and there are no known disease associations for them or SLC37A3. Since only G6PT matches the characteristics of the physiological ER G6P transporter involved in blood glucose homeostasis and neutrophil energy metabolism, the biological roles for the other SLC37 proteins remain to be determined.
- Subjects :
- Models, Molecular
Monosaccharide Transport Proteins
Protein Conformation
Clinical Biochemistry
Phosphatase
Glycogen Storage Disease Type I
Biology
Endoplasmic Reticulum
Models, Biological
Biochemistry
Antiporters
Article
Energy homeostasis
Glycogen Storage Disease Type Ib
medicine
Homeostasis
Humans
Glucose homeostasis
Molecular Biology
Glycogen storage disease type I
Endoplasmic reticulum
General Medicine
medicine.disease
Glucose
Multigene Family
biology.protein
Molecular Medicine
Sugar Phosphates
Glucose 6-phosphatase
Subjects
Details
- ISSN :
- 00982997
- Volume :
- 34
- Database :
- OpenAIRE
- Journal :
- Molecular Aspects of Medicine
- Accession number :
- edsair.doi.dedup.....4703970af922cfc23415a3b3bb4df7b7