| First Author | Peng WT | Year | 2009 |
| Journal | Genesis | Volume | 47 |
| Issue | 9 | Pages | 590-4 |
| PubMed ID | 19548314 | Mgi Jnum | J:155990 |
| Mgi Id | MGI:4418429 | Doi | 10.1002/dvg.20538 |
| Citation | Peng WT, et al. (2009) Generation of mice with a conditional allele for G6pc. Genesis 47(9):590-4 |
| abstractText | Glucose-6-phosphatase-alpha (G6Pase-alpha or G6PC) catalyzes the hydrolysis of glucose-6-phosphate to glucose and is a key enzyme in interprandial glucose homeostasis. Mutations in the human G6PC gene, expressed primarily in the liver, kidney, and intestine, cause glycogen storage disease Type Ia (GSD-Ia), an autosomal recessive disorder characterized by a disturbed glucose homeostasis. For better understanding of the roles of G6Pase-alpha in different tissues and in pathological conditions, we have generated mice harboring a conditional null allele for G6pc by flanking Exon 3 of the G6pc gene with loxP sites. We confirmed the null phenotype by using the EIIa-Cre transgenic approach to generate mice lacking Exon 3 of the G6pc gene. The resulting homozygous Cre-recombined null mice manifest a phenotype mimicking G6Pase-alpha-deficient mice and human GSD-Ia patients. This G6pc conditional null allele will be valuable to examine the consequence of tissue-specific G6Pase-alpha deficiency and the mechanisms of long-term complications in GSD-Ia. |
