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SLC37A4 Transports glucose-6-phosphate from the cytoplasm to the lumen of the endoplasmic reticulum. Forms with glucose-6- phosphatase the complex responsible for glucose production through glycogenolysis and gluconeogenesis. Hence, it plays a central role in homeostatic regulation of blood glucose levels. Defects in SLC37A4 are the cause of glycogen storage disease type 1B (GSD1B). GSD1B is a metabolic disorder characterized by impairment of terminal steps of glycogenolysis and gluconeogenesis. GSD1 patients manifest a wide range of clinical symptoms and biochemical abnormalities, including hypoglycemia, severe hepatomegaly due to excessive accumulation of glycogen, kidney enlargement, growth retardation, lactic acidemia, hyperlipidemia, and hyperuricemia. GSD1B patients also present a tendency towards infections associated with neutropenia, relapsing aphthous gingivostomatitis, and inflammatory bowel disease. Defects in SLC37A4 are the cause of glycogen storage disease type 1C (GSD1C). Defects in SLC37A4 are the cause of glycogen storage disease type 1D (GSD1D). Belongs to the major facilitator superfamily. Organophosphate:Pi antiporter (OPA) (TC 2.A.1.4) family. 2 isoforms of the human protein are produced by alternative splicing. Note: This description may include information from UniProtKB.
Protein type: Membrane protein, integral; Membrane protein, multi-pass; Transporter; Transporter, SLC family
Chromosomal Location of Human Ortholog: 11q23.3
Cellular Component: endoplasmic reticulum; endoplasmic reticulum membrane; integral component of endoplasmic reticulum membrane; integral component of membrane; membrane
Molecular Function: glucose-6-phosphate transmembrane transporter activity; transporter activity
Biological Process: carbohydrate transport; gluconeogenesis; glucose metabolic process; glucose-6-phosphate transport; transport
Disease: Glycogen Storage Disease Ib; Glycogen Storage Disease Ic
Reference #:  O43826 (UniProtKB)
Alt. Names/Synonyms: G6PT; G6PT1; G6PT2; G6PT3; Glucose-5-phosphate transporter; glucose-6-phosphatase, transport (glucose) protein 3; glucose-6-phosphatase, transport (glucose-6-phosphate) protein 1; glucose-6-phosphatase, transport (phosphate/pyrophosphate) protein 2; Glucose-6-phosphate translocase; GSD1b; GSD1c; GSD1d; MGC15729; microsomal glucose-6-phosphate transporter; PRO0685; SLC37A4; solute carrier family 37 (glucose-6-phosphate transporter), member 4; solute carrier family 37 (glycerol-6-phosphate transporter), member 4; Solute carrier family 37 member 4; Transformation-related gene 19 protein; TRG-19; TRG19
Gene Symbols: SLC37A4
Molecular weight: 46,360 Da
Basal Isoelectric point: 8.85  Predict pI for various phosphorylation states
Select Structure to View Below


Protein Structure Not Found.

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