Phenotypes associated with this allele

• Allele Symbol: Agtpbp1^atms
• Allele Name: ataxia and male sterility
• Allele ID: MGI:3612160
• Summary: 4 genotypes

Jump to	Allelic Composition	Genetic Background	Genotype ID
hm1	Agtpbp1^atms/Agtpbp1^atms	MRL/Mp-Agtpbp1^atms	MGI:3613081
hm2	Agtpbp1^atms/Agtpbp1^atms	MRL/Mp-Agtpbp1^atms Fas^lpr	MGI:3613080
cx3	Agtpbp1^atms/Agtpbp1^atms Fas^lpr/Fas^lpr	MRL/Mp-Agtpbp1^atms Fas^lpr	MGI:3613073
cx4	Agtpbp1^atms/Agtpbp1^atms Fas^lpr/?	MRL/Mp-Agtpbp1^atms Fas^lpr	MGI:3613075

Genotype
MGI:3613081

hm1

• Allelic Composition: Agtpbp1^atms/Agtpbp1^atms
• Genetic Background: MRL/Mp-Agtpbp1^atms

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

immune system

abnormal T cell differentiation ( J:103944 )

abnormal lymphocyte cell number ( J:103944 )

decreased B cell number ( J:103944 )

abnormal T cell subpopulation ratio ( J:103944 )

• significant reduction in the CD4/CD8 ratio

increased CD4-positive, alpha-beta T cell number ( J:103944 )

• increase in CD4 T cells

increased CD8-positive, alpha-beta T cell number ( J:103944 )

• increase in CD8 T cells

abnormal spleen morphology ( J:103944 )

decreased spleen weight ( J:103944 )

• 50% reduction of spleen weight

spleen hypoplasia ( J:103944 )

• significant decrease in the total number of nucleated cells in the spleen, however the number of cells per weight of the spleen is larger

abnormal T cell physiology ( J:103944 )

• T cell response to ConA is not strictly controlled as splenocytes show a vague dose and time dependency and respond earlier in broader doses, however mice do not develop immune deficiency

behavior/neurological

ataxia ( J:103944 )

hematopoietic system

abnormal T cell differentiation ( J:103944 )

abnormal lymphocyte cell number ( J:103944 )

decreased B cell number ( J:103944 )

abnormal T cell subpopulation ratio ( J:103944 )

• significant reduction in the CD4/CD8 ratio

increased CD4-positive, alpha-beta T cell number ( J:103944 )

• increase in CD4 T cells

increased CD8-positive, alpha-beta T cell number ( J:103944 )

• increase in CD8 T cells

abnormal spleen morphology ( J:103944 )

decreased spleen weight ( J:103944 )

• 50% reduction of spleen weight

spleen hypoplasia ( J:103944 )

• significant decrease in the total number of nucleated cells in the spleen, however the number of cells per weight of the spleen is larger

abnormal T cell physiology ( J:103944 )

• T cell response to ConA is not strictly controlled as splenocytes show a vague dose and time dependency and respond earlier in broader doses, however mice do not develop immune deficiency

Genotype
MGI:3613080

hm2

• Allelic Composition: Agtpbp1^atms/Agtpbp1^atms
• Genetic Background: MRL/Mp-Agtpbp1^atms Fas^lpr

behavior/neurological

ataxia ( J:103944 )

• ataxia is seen in homozygous mice that may be heterozygous or wild-type for Fas^lpr, however they do not exhibit any of the phenotypes seen in Fas^lpr mice

immune system

decreased spleen weight ( J:103944 )

• 45% reduction in spleen weight

hematopoietic system

decreased spleen weight ( J:103944 )

• 45% reduction in spleen weight

Genotype
MGI:3613073

cx3

• Allelic Composition: Agtpbp1^atms/Agtpbp1^atms; Fas^lpr/Fas^lpr
• Genetic Background: MRL/Mp-Agtpbp1^atms Fas^lpr

behavior/neurological

ataxia ( J:103944 )

immune system

enlarged spleen ( J:103944 )

• spleen is enlarged but to a smaller extent than in single homozygous Fas^lpr mice

enlarged lymph nodes ( J:103944 )

• mild lymphadenopathy that occurs late and slowly compared to homozygous Fas^lpr mice

hematopoietic system

enlarged spleen ( J:103944 )

• spleen is enlarged but to a smaller extent than in single homozygous Fas^lpr mice

integument

integument phenotype ( J:103944 )

N • have a normal smooth fur appearance unlike single homozygous Fas^lpr mice

growth/size/body

enlarged spleen ( J:103944 )

• spleen is enlarged but to a smaller extent than in single homozygous Fas^lpr mice

Genotype
MGI:3613075

cx4

• Allelic Composition: Agtpbp1^atms/Agtpbp1^atms; Fas^lpr/?
• Genetic Background: MRL/Mp-Agtpbp1^atms Fas^lpr

behavior/neurological

ataxia ( J:103945 )

• subtle but abnormal motion of the body swaying laterally is seen at about 21 days of age

• persistent mild to moderate ataxia is noticeable throughout life, however it does not worsen with age

• width of steps is narrower, but do not see widening of the distance between both feet

impaired coordination ( J:103945 )

• cannot stay on the rotarod at all and fall down within the first 2-3 seconds

nervous system

abnormal stellate ganglion morphology ( J:103945 )

• greater density of stellate neurons along with reactive glia

decreased brain weight ( J:103945 )

• low in 4.5 month old mice (389 mg vs. 503 mg in controls)

abnormal cerebellum morphology ( J:103945 )

abnormal cerebellar foliation ( J:103945 )

• incomplete foliation, particularly in the central lobules

decreased Purkinje cell number ( J:103945 )

• 50% loss of Purkinje cells at 3 weeks of age, with cell loss rapidly accelerating so that by 6 weeks of age, almost all are lost, however do not see any severe loss of neural cells in the cerebellum and brainstem

thin cerebellar granule layer ( J:103945 )

• thinning of the granular layer seems to be due to a decrease in the number of granule cells per unit area

thin cerebellar molecular layer ( J:103945 )

• thinner at 4.5 months of age but not at 3 weeks of age, due to loss of Purkinje dendritic trees

small cerebellum ( J:103945 )

• small in 4.5 month old mice

reproductive system

oligozoospermia ( J:103945 )

♂ • mature sperm are seldom seen in the testis, however primary spermatocytes and spermatogonia are observed in the seminiferous tubules

arrest of spermatogenesis ( J:103945 )

♂ • cessation of differentiation mainly at the spermatid stage and degeneration of cells

female infertility ( J:103945 )

♀ • although females are infertile, no pathological change in the ovaries is observed and ova can be fertilized in vitro

male infertility ( J:103945 )

♂

cellular

oligozoospermia ( J:103945 )

♂ • mature sperm are seldom seen in the testis, however primary spermatocytes and spermatogonia are observed in the seminiferous tubules