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Phenotypes Associated with This Genotype
Genotype
MGI:4821386
Allelic
Composition		 Prnptm1Cwe/Prnptm1Cwe
Tg(Prnp)a20Cwe/0
Genetic
Background		 involves: 129S7/SvEvBrd * C57BL/6
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Prnptm1Cwe mutation (37 available); any Prnp mutation (142 available)
Tg(Prnp)a20Cwe mutation (2 available)
♀ phenotype observed in females
♂ phenotype observed in males
N normal phenotype
immune system
decreased thymocyte number ( J:129512 )
• mice exhibit a reduction in thymocyte numbers compared with wild-type mice
• however, supplementation with copper partially reverses lowered thymocyte numbers
abnormal T cell differentiation ( J:129512 )
• gammadelta T cell differentiation is promoted while alphabeta T cell differentiation is impaired compared to in wild-type mice
• T cell differentiation is partially arrested at DN3 unlike in wild-type mice
• however, supplementation with copper partially reverses defects in T cell differentiation
increased double-negative T cell number ( J:129512 )
• partially reversed by copper supplementation
decreased double-positive T cell number ( J:129512 )
• partially reversed by copper supplementation
increased gamma-delta T cell number ( J:129512 )
• 9-fold, partially reversed by copper supplementation
abnormal B cell morphology ( J:129512 )
• the proportion of CD19+ and B220+ B cells is increased compared to in wild-type mice
• however, the total number of B cells is normal

homeostasis/metabolism
abnormal copper level ( J:129512 )
• copper levels in the brain and thymus are increased compared to in wild-type mice
increased brain copper level ( J:126728 )
• after 12 months, mice exhibit increased brain copper levels compared with wild-type mice
• copper levels in the synaptosomes are increased compared to in wild-type mice
abnormal magnesium ion homeostasis ( J:126728 )
• mice exhibit an increase in brain magnesium level compared with wild-type mice

hematopoietic system
decreased thymocyte number ( J:129512 )
• mice exhibit a reduction in thymocyte numbers compared with wild-type mice
• however, supplementation with copper partially reverses lowered thymocyte numbers
abnormal T cell differentiation ( J:129512 )
• gammadelta T cell differentiation is promoted while alphabeta T cell differentiation is impaired compared to in wild-type mice
• T cell differentiation is partially arrested at DN3 unlike in wild-type mice
• however, supplementation with copper partially reverses defects in T cell differentiation
increased double-negative T cell number ( J:129512 )
• partially reversed by copper supplementation
decreased double-positive T cell number ( J:129512 )
• partially reversed by copper supplementation
increased gamma-delta T cell number ( J:129512 )
• 9-fold, partially reversed by copper supplementation
abnormal B cell morphology ( J:129512 )
• the proportion of CD19+ and B220+ B cells is increased compared to in wild-type mice
• however, the total number of B cells is normal

nervous system
increased brain copper level ( J:126728 )
• after 12 months, mice exhibit increased brain copper levels compared with wild-type mice
• copper levels in the synaptosomes are increased compared to in wild-type mice

endocrine/exocrine glands
decreased thymocyte number ( J:129512 )
• mice exhibit a reduction in thymocyte numbers compared with wild-type mice
• however, supplementation with copper partially reverses lowered thymocyte numbers

Contributing Projects:
Mouse Genome Database (MGD), Gene Expression Database (GXD), Mouse Models of Human Cancer database (MMHCdb) (formerly Mouse Tumor Biology (MTB)), Gene Ontology (GO)
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Send questions and comments to User Support. 		last database update
03/18/2025
MGI 6.24 		The Jackson Laboratory