Phenotypes for G6pc1 Tg(Kap-icre)29066/2Sig MGI:5823404 MGI Mouse

renal/urinary system

• hypercitraturia in tamoxifen treated mice

• however, microlithiasis is not seen and no changes in urine excretion, urea level, ketone bodies level, or creatinuria are seen

increased urine magnesium level ( J:238264 )

• in tamoxifen treated mice

decreased urine pH ( J:238264 )

• urinary pH is more acidic in tamoxifen treated mice than in controls

increased urine phosphate level ( J:238264 )

• in tamoxifen treated mice

increased urine microalbumin level ( J:238264 )

• microalbuminuria in tamoxifen treated mice

increased urine uric acid level ( J:238264 )

• uric acid levels are increased 3-fold in the urine of tamoxifen treated mice

abnormal kidney morphology ( J:238264 )

• marker analysis indicates that kidneys from tamoxifen treated mice show epithelial-mesenchymal transition-like changes, with loss of epithelial markers and overexpression of fibronectin

• lipid deposits in the internal part of the renal cortex of tamoxifen treated mice

• however, glomerulosclerosis is not seen

enlarged kidney ( J:238264 )

• enlarged kidneys in tamoxifen treated mice

increased kidney weight ( J:238264 )

increased kidney glycogen level ( J:238264 )

• glycogen accumulation in the tubules in the external part of the kidney cortex of tamoxifen treated mice

abnormal kidney cortex morphology ( J:238264 )

• alterations of the kidney cortex with a clarification and enlargement of tubular epithelial cells in tamoxifen treated mice

• lipid deposits in the internal part of the renal cortex of tamoxifen treated mice

abnormal podocyte morphology ( J:238264 )

• marker analysis indicates podocyte damage in tamoxifen treated mice

abnormal renal tubule morphology ( J:238264 )

• tubule dilation due to massive accumulation of glycogen

homeostasis/metabolism

increased blood uric acid level ( J:238264 )

• plasma uric acid concentrations are slightly higher in mice treated with tamoxifen at 6-8 weeks of age compared with controls

• however, plasma cholesterol, triglycerides, lactate, and blood urea nitrogen levels are normal in tamoxifen treated mice

increased kidney glycogen level ( J:238264 )

• glycogen accumulation in the tubules in the external part of the kidney cortex of tamoxifen treated mice

abnormal urine homeostasis ( J:238264 )

• hypercitraturia in tamoxifen treated mice

• however, microlithiasis is not seen and no changes in urine excretion, urea level, ketone bodies level, or creatinuria are seen

increased urine magnesium level ( J:238264 )

• in tamoxifen treated mice

decreased urine pH ( J:238264 )

• urinary pH is more acidic in tamoxifen treated mice than in controls

increased urine phosphate level ( J:238264 )

• in tamoxifen treated mice

increased urine microalbumin level ( J:238264 )

• microalbuminuria in tamoxifen treated mice

increased urine uric acid level ( J:238264 )

• uric acid levels are increased 3-fold in the urine of tamoxifen treated mice

growth/size/body

enlarged kidney ( J:238264 )

• enlarged kidneys in tamoxifen treated mice

increased kidney weight ( J:238264 )

Mouse Models of Human Disease		DO ID	OMIM ID(s)	Ref(s)
glycogen storage disease Ia		DOID:2749	OMIM:232200	J:238264

♀	phenotype observed in females
♂	phenotype observed in males
N	normal phenotype

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