Reaction: Defective SLC37A4 does not exchange G6P and Pi across the ER membrane
- in pathway: Glycogen storage disease type Ib (SLC37A4)
The SLC37A4 transport protein in the endoplasmic reticulum membrane normally mediates the exchange of cytosolic glucose-6-phosphate and orthophosphate from the endoplasmic reticulum lumen. Defects in this transporter are associated with glycogen storage disease type Ib. The two missense mutant forms of SLC37A4 annotated here are examples of the many that have been described (Gerin et al. 1997; Chen et al. 2008; Veiga-da-Cunha et al. 1998).
Reaction - small molecule participants:
Pi [endoplasmic reticulum lumen]
G6P [cytosol]
Reactome.org reaction link: R-HSA-3229118
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Reaction input - small molecules:
hydrogenphosphate
alpha-D-glucose 6-phosphate(2-)
Reaction output - small molecules:
Reactome.org link: R-HSA-3229118