Phenotypes Associated with This Genotype

Genotype
MGI:5441067

• Allelic Composition: Shh^tm1Amc/Shh^tm2Amc; Tg(Hoxb7-cre)13Amc/0
• Genetic Background: involves: 129S1/Sv * 129X1/SvJ * C57BL/6

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

renal/urinary system

abnormal renal glomerulus morphology ( J:79481 )

• at the newborn stage, cortical glomerular density is increased by 24% while glomerular density of the whole kidney is increased by 26% relative to that in wild-type controls

• however, no gross differences in glomerular size are observed

decreased renal glomerulus number ( J:79481 )

• newborn mice exhibit a 40% reduction in glomerular number

kidney cortex hypoplasia ( J:79481 )

• at the newborn stage, cortical volume is reduced by 51%

abnormal kidney inner medulla morphology ( J:79481 )

• at 4 months of age, most of the inner medulla is lost in hydronephric kidneys

abnormal kidney outer medulla inner stripe morphology ( J:79481 )

• at 4 months of age, most of the inner stripe of the outer medulla is lost in hydronephric kidneys

kidney medulla hypoplasia ( J:79481 )

• at the newborn stage, medullary volume is reduced by 46%

hydronephrosis ( J:79481 )

• at 4 months of age, 50% of mice exhibit hydronephrosis

• however, no hydronephrosis is detected in newborn pups

small kidney ( J:79481 )

• neonatal kidneys are 52% smaller than wild-type kidneys

renal hypoplasia ( J:79481 )

abnormal ureter development ( J:79481 )

• at E14.5, fewer mesenchymal cells line the ureteral epithelium relative to wild-type controls

• at E14.5, the mitotic index of the proximal and distal ureter mesenchyme is ~50% of that in wild-type controls, indicating reduced cell proliferation

• however, no differences in ureteral mesenchyme apoptosis are observed by TUNEL analysis

abnormal ureter smooth muscle morphology ( J:79481 )

• a delay in smooth muscle differentiation is observed along the proximodistal axis of the ureter

• at E15.0, no smooth muscle alpha-actin protein (SMA), an early marker of smooth muscle differentiation, is detected at any axial level of the ureter, unlike in wild-type embryos where SMA is detected in the proximal ureter

• at the newborn stage, SMA is detected in the proximal ureter but, in contrast to wild-type controls, almost no SMA is detected in the distal-most part of the ureter, closest to the bladder

• in addition, mesenchymal cells in the distal ureter are not as condensed as those in wild-type controls

hydroureter ( J:79481 )

• newborn mice exhibit prominent hydroureter, usually more severe in the proximal region

short ureter ( J:79481 )

• at E14.5, ureter length is ~21% shorter than in wild-type controls