Article
Glucose-6-phosphatase dependent substrate transport in the glycogen storage disease type-1a mouse.
Nature genetics - 1 Jun 1996
Lei K J, Chen H, Pan C J, Ward J M, Mosinger B, Lee E J, Westphal H, Mansfield B C, Chou J Y
Abstract excerpt
Glycogen storage disease type 1a (GSD-1a) is caused by a deficiency in microsomal glucose-6-phosphatase (G6Pase), the key enzyme in glucose homeostasis. A G6Pase knockout mouse which mimics the pathophysiology of human GSD-1a patients was created to understand the pathogenesis of this disorder, t...
Topics
- Animals
- Animals, Newborn
- Base Sequence
- Biological Transport
- Blood Glucose
- Glucose-6-Phosphatase
- Glucose-6-Phosphate
- Glucosephosphates
- Glycogen Storage Disease Type I
- Kidney
- Liver
- Mice
- Mice, Knockout
- Models, Biological
- Molecular Sequence Data
- Phenotype