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Phenotypes associated with this allele
Allele Symbol
Allele Name
Allele ID
Ccpg1tm1a(EUCOMM)Hmgu
targeted mutation 1a, Helmholtz Zentrum Muenchen GmbH
MGI:5000356
Summary 1 genotype
Jump to Allelic Composition Genetic Background Genotype ID
hm1
Ccpg1tm1a(EUCOMM)Hmgu/Ccpg1tm1a(EUCOMM)Hmgu C57BL/6N-Ccpg1tm1a(EUCOMM)Hmgu MGI:6277073


Genotype
MGI:6277073
hm1
Allelic
Composition
Ccpg1tm1a(EUCOMM)Hmgu/Ccpg1tm1a(EUCOMM)Hmgu
Genetic
Background
C57BL/6N-Ccpg1tm1a(EUCOMM)Hmgu
Cell Lines HEPD0725_5_F09
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Ccpg1tm1a(EUCOMM)Hmgu mutation (1 available); any Ccpg1 mutation (93 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
endocrine/exocrine glands
• chief cells of the adult gastric epithelium exhibit loss of polarity, similar to that observed in pancreatic acinar cells
• at 6 weeks of age, exocrine pancreata exhibit a proteostasis defect in acinar cells
• however, no significant reductions in plasma amylase levels or mRNA levels of key differentiation markers of the pancreatic exocrine lineage are observed
• a significant fraction of Ki67+ cells are detected in the normally quiescent acinar cell population at 20 weeks of age, indicating compensatory proliferation of acinar cells in response to injury
• at 6 weeks of age, pancreatic tissues appear whitened and opaque, unlike in wild-type controls
• young adult pancreatic acinar cells accumulate endoplasmic reticulum (ER) that is laden with insoluble zymogen protein
• acinar ER-synthesized enzymes and ER luminal chaperones accumulate in insoluble aggregates
• condensed or heterogeneously sized inclusions are trapped within the lumen; many supernumerary inclusions are in fact intracisternal granule-like structures
• at 40 weeks of age, dead pancreatic acinar cells are often observed within the center of inflammatory infiltrates
• at 40 weeks of age, pancreata show numerous sporadic inflammatory infiltrates, esp. around necrotic cells and in the vicinity of ducts and blood vessels

digestive/alimentary system
• young adult pancreatic acinar cells accumulate endoplasmic reticulum (ER) that is laden with insoluble zymogen protein
• acinar ER-synthesized enzymes and ER luminal chaperones accumulate in insoluble aggregates
• condensed or heterogeneously sized inclusions are trapped within the lumen; many supernumerary inclusions are in fact intracisternal granule-like structures
• chief cells of the adult gastric epithelium exhibit loss of polarity, similar to that observed in pancreatic acinar cells
• at 6 weeks of age, exocrine pancreata exhibit a proteostasis defect in acinar cells
• however, no significant reductions in plasma amylase levels or mRNA levels of key differentiation markers of the pancreatic exocrine lineage are observed
• a significant fraction of Ki67+ cells are detected in the normally quiescent acinar cell population at 20 weeks of age, indicating compensatory proliferation of acinar cells in response to injury

cellular
• distension and disrupted cellular distribution of the rough ER within the pancreatic acinar cells
• loss of ER-phagy capacity, the selective sequestration of ER material into autophagosomes, in pancreatic acinar cells
• loss of ER proteostasis results in accumulated and distended ER within pancreatic acinar cells and hyperactivation of the unfolded protein response (UPR) by 6 weeks of age
• upregulation of molecular markers of ER stress, consistent with a loss of ER-phagic regulation of ER homeostasis and tissue injury of the exocrine pancreas

homeostasis/metabolism
• loss of ER-phagy capacity, the selective sequestration of ER material into autophagosomes, in pancreatic acinar cells

immune system
• at 40 weeks of age, pancreata show numerous sporadic inflammatory infiltrates, esp. around necrotic cells and in the vicinity of ducts and blood vessels





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last database update
11/19/2024
MGI 6.24
The Jackson Laboratory