Phenotypes for Col4a4 MGI:6501951 MGI Mouse

premature death ( J:285510 )

• mice have a mean survival time of 70 days, ranging from 37-103 days

• Background Sensitivity: G3 mice outcrossed one generation on C3H/HeH and then intercrossed show reduced survival time to a mean of 47 days while mice outcrossed one generation on C57BL/6J and the intercrossed show an increased mean survival time to 97 days

decreased body weight ( J:285510 )

• mice are lighter by 5 weeks of age

decreased urine potassium level ( J:285510 )

• Background Sensitivity: mice outcrossed one generation to C3H/HeH show a greater decrease in urine potassium levels at 4 weeks of age than mice outcrossed one generation to C57BL/6J

decreased urine sodium level ( J:285510 )

• mice outcrossed one generation to C3H/HeH show a greater decrease in urine sodium levels at 4 weeks of age than mice outcrossed one generation to C57BL/6J

increased urine protein level ( J:285510 )

• protein to creatinine ratio is elevated from 4 weeks of age but not significantly

• mice outcrossed one generation to C3H/HeH show a similar increase in proteinuria at 7-weeks of age as mice outcrossed one generation to C57BL/6J

• Background Sensitivity: proteinuria level is higher in 4-week old mice outcrossed one generation to C3H/HeH than in mice outcrossed one generation to C57BL/6J

hematuria ( J:285510 )

• Background Sensitivity: hematuria level is higher in 4-week old mice outcrossed one generation to C3H/HeH than in mice outcrossed one generation to C57BL/6J

abnormal kidney morphology ( J:285510 )

• Background Sensitivity: mice outcrossed one generation to C3H/HeH show increased levels of markers of kidney injury compared to mice outcrossed one generation to C57BL/6J, indicating slower progression of disease on the C57BL/6J background than on a C3H/HeH background

abnormal podocyte foot process morphology ( J:285510 )

• Background Sensitivity: foot processes are thickened, flattened, and irregular in 4-week old mice outcrossed one generation to C3H/HeH but not in mice outcrossed one generation to C57BL/6J

podocyte foot process effacement ( J:285510 )

• Background Sensitivity: podocytes often show complete effacement in 7-week old mice outcrossed one generation to C3H/HeH which is not seen in mice outcrossed one generation to C57BL/6J

podocyte hypertrophy ( J:285510 )

• Background Sensitivity: podocytes appear larger and swollen in 4-week old mice outcrossed one generation to C3H/HeH but not in mice outcrossed one generation to C57BL/6J

abnormal renal glomerulus morphology ( J:285510 )

• some 4-week old mice show moderate glomerulonephropathy in the kidneys and all mice show marked diffuse glomerulonephropathy affecting the whole cortex from week 7

• glomerulonephropathy is characterized by diffuse involvement of glomeruli and tubules with enlargement, increased cellularity and sclerosis of glomeruli and degenerative and regenerative changes in tubules and hyaline casts

• Background Sensitivity: mice outcrossed one generation to C3H/HeH show severe diffuse glomerulopathy at 7 weeks of age compared to minimal to moderate tubular changes but no significant glomerular changes in mice outcrossed one generation to C57BL/6J, indicating that C57BL/6J is a protective background for disease development

glomerulosclerosis ( J:285510 )

dilated renal tubule ( J:285510 )

• mice at 53 days of age exhibit degenerative and regenerative changes in tubules resulting in tubular dilatation

renal cast ( J:285510 )

• mice at 53 days of age exhibit hyaline casts

decreased creatinine clearance ( J:285510 )

• glomerular creatinine clearance decreases over time and is significantly lower by 7 weeks of age

• Background Sensitivity: mice backcrossed one generation to C3H/HeH show reduced glomerular creatinine clearance compared to mice backcrossed one generation to C57BL/6J

kidney failure ( J:285510 )

• Background Sensitivity: G3 mice outcrossed one generation to C3H/HeH show aggressive and early onset of renal failure while mice outcrossed one generation to C57BL/6J show slower progression of disease

polyuria ( J:285510 )

• mice produce more urine from 4 weeks of age but urine levels decrease with time

• mice outcrossed one generation to C3H/HeH show a similar increase in urine output as mice outcrossed one generation to C57BL/6J

increased fluid intake ( J:285510 )

• mice consume more water from 4 weeks of age

• mice outcrossed one generation to C3H/HeH show a similar increase in water intake as mice outcrossed one generation to C57BL/6J

increased leukocyte cell number ( J:285510 )

• Background Sensitivity: leukocyte levels are higher in 4-week old mice outcrossed one generation to C3H/HeH than in mice outcrossed one generation to C57BL/6J

increased circulating creatinine level ( J:285510 )

• plasma creatinine levels are increased starting at 5 weeks of age

• Background Sensitivity: mice outcrossed one generation to C3H/HeH show higher serum creatinine levels than mice outcrossed one generation to C57BL/6J which show similar levels seen in wild-type mice

increased blood urea nitrogen level ( J:285510 )

• mice show a progressive increase in plasma urea levels from 4 to 7 weeks of age

• Background Sensitivity: mice outcrossed one generation to C3H/HeH show higher serum urea levels than mice outcrossed one generation to C57BL/6J which show similar levels seen in wild-type mice

decreased circulating serum albumin level ( J:285510 )

• plasma albumin levels are decreased staring at 4 weeks of age

• mice outcrossed one generation to C3H/HeH show a similar level of hypoalbuminuria at 7 weeks of age as mice outcrossed one generation to C57BL/6J

• Background Sensitivity: however, serum albumin levels are reduced in 4-week old mice that are outcrossed one generation to C3H/HeH but not in mice that are outcrossed one generation to C57BL/6J which show similar level to wild-type mice indicating an earlier reduction in albumin levels in mice outcrossed to C3H/HeH than in C57BL/6J outcrossed mice