[HTML][HTML] Bone marrow-derived cells require a functional glucose 6-phosphate transporter for normal myeloid functions
Glycogen storage disease type Ib (GSD-Ib) is caused by a deficiency in the ubiquitously
expressed glucose 6-phosphate transporter (Glc-6-PT). Glc-6-PT activity has been shown to
be critical in the liver and kidney where a deficiency disrupts glucose homeostasis. GSD-Ib
patients also have defects in the neutrophil respiratory burst, chemotaxis, and calcium flux.
They also manifest neutropenia, but whether Glc-6-PT deficiency in the bone marrow
underlies myeloid dysfunctions in GSD-Ib remains controversial. To address this, we …
expressed glucose 6-phosphate transporter (Glc-6-PT). Glc-6-PT activity has been shown to
be critical in the liver and kidney where a deficiency disrupts glucose homeostasis. GSD-Ib
patients also have defects in the neutrophil respiratory burst, chemotaxis, and calcium flux.
They also manifest neutropenia, but whether Glc-6-PT deficiency in the bone marrow
underlies myeloid dysfunctions in GSD-Ib remains controversial. To address this, we …
