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Phenotypes Associated with This Genotype
Genotype
MGI:5532579
Allelic
Composition
ClppGt(IST11134F10)Tigm/ClppGt(IST11134F10)Tigm
Genetic
Background
involves: C57BL/6N
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
ClppGt(IST11134F10)Tigm mutation (1 available); any Clpp mutation (11 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
mortality/aging
• fewer than expected mice are produced from breeding heterozygotes

reproductive system
• absence of spermatids and mature spermatozoa at 6 months
• in follicular granulosa cell layers
• reduced numbers due to apoptosis
• at 3 months
• spongiform changes with decreased number of dividing cells at 6 months
• at 3 months
• at an early stages before spermatid generation

behavior/neurological
• at 12 to 18 months
• in an open field
• in an open field, mice exhibit decreased horizontal activity, movement number, total distance traveled, movement time, stereotype count, margin distance and center distance compared with wild-type mice

growth/size/body
• throughout adult life

cellular
• absence of spermatids and mature spermatozoa at 6 months
• increased DNA in the testis, ovary, heart and brain
• in the subsarcolemmal space
• in the subsarcolemmal space with irregular cristae membrane organization
• in follicular granulosa cell layers
• in mouse embryonic fibroblasts
• in mouse embryonic fibroblasts
• slightly decreased in muscle and heart cells without compensatory mitochondrial proliferation
• however, respiration in brain cells is normal

immune system
• increased activation in the spleen
• less prominent ulcerative dermatitis compared with wild-type mice

integument
• less prominent ulcerative dermatitis compared with wild-type mice

endocrine/exocrine glands
• reduced numbers due to apoptosis
• at 3 months
• spongiform changes with decreased number of dividing cells at 6 months
• at 3 months

hematopoietic system
• increased activation in the spleen

Mouse Models of Human Disease
DO ID OMIM ID(s) Ref(s)
Perrault syndrome DOID:0050857 OMIM:233400
OMIM:614129
OMIM:614926
OMIM:615300
J:203140


Contributing Projects:
Mouse Genome Database (MGD), Gene Expression Database (GXD), Mouse Tumor Biology (MTB), Gene Ontology (GO), MouseCyc
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last database update
03/13/2019
MGI 6.13
The Jackson Laboratory