Phenotypes for Clu MGI:5432925 MGI Mouse

renal/urinary system phenotype ( J:74983 )

N	• mice exhibit no evidence of tubulointerstitial disease, vascular lesions, inflammatory infiltrates, necrosis, or amyloid deposits, regardless of the extent of glomerulopathy • glomerular basement membranes are normal and lack discernible deposits

abnormal glomerular capillary morphology ( J:74983 )

• collapse of capillary lumens at 21 months of age

hematuria ( J:74983 )

• an occasional mutant develops hematuria

abnormal glomerular mesangium morphology ( J:74983 )

• up to 75% of glomeruli exhibit moderate to severe mesangial lesions at 21 months of age

increased mesangial cell number ( J:74983 )

• mesangial hypercellularity noted in 6-mo-old mice that have undergone unilateral nephrectomy at 3 months and are then fed a high-protein diet

abnormal mesangial matrix morphology ( J:74983 )

• electron-dense tubulo-fibrillar structures arranged in parallel arrays are detected in the mesangial matrix at 21 months, consistent with immune complex deposits

• more electron-dense deposits found in 6-mo-old mice that have undergone unilateral nephrectomy at 3 months and are then fed a high-protein diet

expanded mesangial matrix ( J:74983 )

• variable mesangial expansion observed at 21 months, but not at 6 months, of age

• more severe mesangial expansion noted in 6-mo-old mice that have undergone unilateral nephrectomy at 3 months and are then fed a high-protein diet

cortical renal glomerulopathies ( J:74983 )

• at 21 months of age, mice display progressive mesangial expansion, collapse of capillary lumens, and deposition of collagen and immune complexes, unlike wild-type and heterozygous controls

renal glomerular protein deposits ( J:74983 )

• immune complexes of IgG, IgM, IgA, and in some cases C1q, C3, and C9 are detected in the mesangium at 21 months of age

• increased deposition of C1q and C3 in 6-mo-old mice that have undergone unilateral nephrectomy at 3 months and are then fed a high-protein diet

renal glomerular immunoglobulin deposits ( J:74983 )

• immune complexes of IgG, IgM and IgA are noted as early as 4 weeks of age

renal glomerulus fibrosis ( J:74983 )

• accumulation of collagen deposits in the glomeruli

homeostasis/metabolism phenotype ( J:74983 )

N	• mice display no signs of proteinuria or any alterations in serum and urine proteins relative to controls