All Phenotypes Clmp<tm1Fgr> MGI Mouse

• survival rate is decreased after birth

postnatal lethality, incomplete penetrance ( J:258315 )

• less than 40% survival rate by P20

• body weight of surviving mice increases more slowly from P1 onwards with mice showing a severe delay to thrive

abnormal intestinal peristalsis ( J:258315 )

• intestines exhibit reduced amplitudes of pendular movements, with dueodenum having a 6.5-fold decrease and jejunum having a 17-fold decrease in the amplitude of movement

• intestinal tissue does not contract as narrow as control intestines

• mice show lack of coordinated contraction of the intestine and decreased motility of intestine

• however, frequency of contraction is normal

hydronephrosis ( J:258315 )

• bilateral hydronephrosis develops perinatally and aggravates rapidly by dilation of the renal pelvis and loss of renal parenchyma

• however, concentrations of ions, urea, creatinine, or total protein in the urine are normal

abnormal ureter smooth muscle morphology ( J:258315 )

• impaired and uncoordinated motility and cell-cell communication in ureteral smooth muscle cells

abnormal ureter physiology ( J:258315 )

• calcium transients in smooth muscle layers of the ureter are uncorrelated

impaired ureteric peristalsis ( J:258315 )

• cultured embryonic ureters are unable to form proximal-to-distal directed peristaltic waves and in extended culture, predominantly uncoordinated, fibrillation-like movements of the ureter wall without full contractions are seen, indicating impaired ureteral motility

• impaired and uncoordinated motility and cell-cell communication in ureteral and intestinal smooth muscle cells

abnormal reproductive system morphology ( J:258315 )

• hypomorphic reproductive tracts are seen in P40 mice of both genders