MGI:1915841Mice homozygous for a null allele exhibit premature death, renal failure due to obstructive nephropathy with xanthinuria, xanthine deposits, cystic tubular dilation, Tamm-Horsfall protein deposits, tubular cell necrosis with neutrophils, occasional hydronephrosis with urolithiasis, and altered metabolism of purines, amino acids, and phospholipids.