Phenotypes associated with this allele

• Allele Symbol: Herc1^tbl
• Allele Name: tambaleante
• Allele ID: MGI:3613033
• Summary: 1 genotype

Jump to	Allelic Composition	Genetic Background	Genotype ID
hm1	Herc1^tbl/Herc1^tbl	involves: DW/J	MGI:3619135

Genotype
MGI:3619135

hm1

• Allelic Composition: Herc1^tbl/Herc1^tbl
• Genetic Background: involves: DW/J

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

Hind limbs clasping, impaired rotarod performance, and Purkinje cell degeneration in Herc1^tbl/Herc1^tbl mice

mortality/aging

decreased survivor rate ( J:161738 )

• less than 40% of the mutant mice survived longer than 40 weeks compared to control

nervous system

abnormal cerebellar Purkinje cell layer ( J:23733 )

• some climbing fibers with relatively normal contact with proximal dendrites of Purkinje cells

• most climbing fibers develop extensive plexi over Purkinje soma and make little contact with more distant dendrites

abnormal Purkinje cell morphology ( J:106414 )

• normal until around 2 months

Purkinje cell degeneration ( J:23733 )

• initially thickening of neurite and appearance of well developed terminal arbors

• axons and collaterals thicken and "torpedoes" appear

• atrophy of terminal plexi and axons become reduced

• reduction of dendrites

decreased Purkinje cell number ( J:106414 )

• very reduced in numbers by 3.5 months initial cell losses fairly uniform but symmetrical, poorly defined, regions resistant to degeneration apparent at 2.5 months very reduced in numbers by 3.5 months

• initial cell losses fairly uniform but symmetrical, poorly defined, regions resistant to degeneration apparent at 2.5 months

behavior/neurological

limb grasping ( J:161738 )

• abnormal hid limb clasping reflex which becomes obvious from two month of age and worsen with time

impaired coordination ( J:161738 )

• mutant mice stayed less time on the rotarod without falling

abnormal gait ( J:161738 )

• unstable gait which becomes obvious from two month of age and worsen with time

growth/size/body

decreased body height ( J:161738 )

• the weight of the mutant animals was 15 to 30% lower than control