Phenotypes Associated with This Genotype

Genotype
MGI:3615499

• Allelic Composition: Lhx1^tm1Tmj/Lhx1^tm2.1Bhr; Tg(Pax2-cre)10Shwl/0
• Genetic Background: involves: 129S1/Sv * 129S7/SvEvBrd * C57BL/6 * CD-1 * SJL

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

mortality/aging

neonatal lethality, complete penetrance ( J:104938 )

renal/urinary system

abnormal kidney morphology ( J:104938 )

• kidneys are rudimentary in dead neonates

hydronephrosis ( J:104938 )

• some dead neonates have hydronephrotic kidneys

small kidney ( J:104938 )

• at E14.5, kidneys are approximately half the size of wild-type kidneys

renal hypoplasia ( J:104938 )

• at 3 weeks, mice displayed renal hypoplasia or renal hypoplasia with unilateral hydronephrosis and megaureter

duplex kidney ( J:104938 )

• 2 of 7 neonates necropsied on P1 had duplex kidneys

single kidney ( J:104938 )

• at E14.5, 20% of mutants have unilateral renal agenesis

abnormal ureter morphology ( J:104938 )

• the two ureters from the duplex kidneys join before entering bladder

• in some instances, the ureter and bladder do not separate or attach to the bladder

• the ureter is very tortuous

impaired branching involved in ureteric bud morphogenesis ( J:104938 )

• at E11.5, ureteric bud is Y-shaped not T-shaped suggesting branching is impaired

• at E12.4, reduced branching is observed in the ureteric bud

abnormal ureteric bud elongation ( J:104938 )

• at E11.5, ureteric bud outgrowth is delayed

embryo

rudimentary Mullerian ducts ( J:104938 )

• only rostral-most part of duct forms in mutants

• female sex ducts are rudimentary

abnormal Wolffian duct morphology ( J:104938 )

• at E10.75, extension of the nephric duct to urogenital sinus is impaired

Wolffian duct degeneration ( J:104938 )

• at E14.5, nephric duct is not maintained

rudimentary Wolffian ducts ( J:104938 )

• male sex ducts are rudimentary

• at E10.75, extension of the nephric duct to urogenital sinus is impaired