All Phenotypes Wt1<tm2Hst> MGI Mouse

reproductive system

azoospermia ( J:103489 )

• the XXY heterozygous male had aspermic epididymides

small testis ( J:53585 , J:103489 )

(J:53585)

(J:103489)

abnormal reproductive system physiology ( J:103489 )

• male sex reversal does not occur, however, only 2 of 23 XX to XY chimeras developed as phenotypic males, a significantly lower proportion than the 40% found in wild-type XX to XY chimeras, indicating that the sex ratio is distorted

male infertility ( J:53585 )

• a single heterozygous male, that is 41, XXY, was obtained from a female chimera and is sterile

renal/urinary system

abnormal kidney vasculature morphology ( J:53585 )

• kidneys of the heterozygous male exhibit a secondary hypertensive nephropathy, indicating kidney disease

abnormal kidney interlobular artery morphology ( J:53585 )

• exhibit interlobular arteries with severe hypertensive damage, including fibrinoid necrosis, medial hypertrophy and hyperplasia, and loss of the arterial lumen

abnormal glomerular capillary morphology ( J:53585 )

• obliteration of the glomerular capillary bed

abnormal mesangial cell morphology ( J:53585 )

• mesangial cells show an increase in nuclear size, a prominent nucleolus, and hypercellularity

increased mesangial cell number ( J:53585 )

• mesangial hypercellularity

kidney cyst ( J:53585 )

• kidneys of the heterozygous male exhibit tubular epithelial microcyst formation

decreased glomerular capsule space ( J:53585 )

• obliteration of the urinary filtration space

absent podocyte foot process ( J:53585 )

• podocytes show loss of foot processes

podocyte hypertrophy ( J:53585 )

• podocyte hypertrophy and hyperplasia

podocyte microvillus transformation ( J:53585 )

• podocytes show microvillus transformation of the apical surface

expanded mesangial matrix ( J:53585 )

• kidneys of the heterozygous male and of chimeras, although to a lesser extent than the heterozygote, exhibit a diffuse and global mesangial sclerosis, indicating kidney disease

glomerulosclerosis ( J:53585 )

• global sclerosis of the glomerular tuft and obliteration of the glomerular capillary bed and urinary filtration space by an increase in extracellular mesangial matrix

glomerular crescent ( J:53585 )

• glomerulus shows focal crescent formation

renal interstitial fibrosis ( J:53585 )

dilated renal tubule ( J:53585 )

kidney failure ( J:53585 )

• the male heterozygote exhibits end-stage renal failure at 8 months of age

endocrine/exocrine glands