Phenotypes Associated with This Genotype

Genotype
MGI:3814590

• Allelic Composition: Stk11^tm1.1Rdp/Stk11^tm1.1Rdp; Tg(Pdx1-cre)89.1Dam/0
• Genetic Background: involves: 129S6/SvEvTac * C57BL/6 * CBA * FVB/N * ICR

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

digestive/alimentary system

abnormal exocrine pancreas morphology ( J:134078 )

• metaplastic lesions are observed with contributions from acinar and ductal cells consistent with active acinar-ductal transdifferentiation

abnormal pancreatic acinus morphology ( J:134078 )

• at P1 acinar units are less compact with distended lumens; in some regions, acinar units are fragmented and intermixed with endocrine cells

abnormal pancreatic acinar cell morphology ( J:134078 )

• from P2 to P8, a rapid loss of acinar cells is observed, associated with appearance of abnormal ductal structures

endocrine/exocrine glands

increased pancreas apoptosis ( J:134078 )

• at P3, TUNEL assays reveal an 18-fold increase in acinar cell death in the pancreas relative to controls

abnormal exocrine pancreas morphology ( J:134078 )

• metaplastic lesions are observed with contributions from acinar and ductal cells consistent with active acinar-ductal transdifferentiation

abnormal pancreatic acinus morphology ( J:134078 )

• at P1 acinar units are less compact with distended lumens; in some regions, acinar units are fragmented and intermixed with endocrine cells

abnormal pancreatic acinar cell morphology ( J:134078 )

• from P2 to P8, a rapid loss of acinar cells is observed, associated with appearance of abnormal ductal structures

abnormal pancreatic islet morphology ( J:134078 )

• islets are smaller and less well aggregated at P1 with a more diffuse distribution relative to wild-type; aggregation of islets is delayed with accumulation of smaller islets by P16

• at P1, there are decreased numbers of alpha, beta, and delta cells in the pancreas

decreased pancreatic alpha cell number ( J:134078 )

• at P1

decreased pancreatic beta cell number ( J:134078 )

• at P1

decreased pancreatic delta cell number ( J:134078 )

• at P1

small pancreatic islets ( J:134078 )

• islets are smaller and less well aggregated at P1 with a more diffuse distribution relative to wild-type; aggregation of islets is delayed with accumulation of smaller islets by P16

small pancreas ( J:134078 )

• at P1, pancreas is smaller than in littermate controls

pancreas fibrosis ( J:134078 )

• from P2 to P8, reactive fibrosis is observed in conjunction with acinar cell loss and abnormal ductal structures

pancreas inflammation ( J:134078 )

homeostasis/metabolism

increased circulating glucose level ( J:134078 )

• at P1, mice have elevated blood glucose levels but these return to normal by P8

improved glucose tolerance ( J:134078 )

• adult mutants show improved beta cell function relative to wild-type with faster clearance of blood glucose

cellular

increased pancreas apoptosis ( J:134078 )

• at P3, TUNEL assays reveal an 18-fold increase in acinar cell death in the pancreas relative to controls

immune system

pancreas inflammation ( J:134078 )

Mouse Models of Human Disease		DO ID	OMIM ID(s)	Ref(s)
Peutz-Jeghers syndrome		DOID:3852	OMIM:175200	J:134078