Phenotypes associated with this allele

• Allele Symbol: Ltn1^lister
• Allele Name: lister
• Allele ID: MGI:3838105
• Summary: 2 genotypes

Jump to	Allelic Composition	Genetic Background	Genotype ID
hm1	Ltn1^lister/Ltn1^lister	C57BL/6-Ltn1^lister	MGI:3838107
ht2	Ltn1^Gt(RRR322)Byg/Ltn1^lister	involves: 129P2/OlaHsd * C57BL/6	MGI:3838109

Genotype
MGI:3838107

hm1

• Allelic Composition: Ltn1^lister/Ltn1^lister
• Genetic Background: C57BL/6-Ltn1^lister

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

Progressive impairment of neuronal and motor functions associated with weight loss and reduced life span in Ltn1^lister/Ltn1^lister mice

behavior/neurological

limb grasping ( J:146493 )

• one of the earliest signs of disease is loss of the hind limb extension reflex

abnormal motor coordination/balance ( J:146493 )

impaired righting response ( J:146493 )

• progressive age-dependent impairment

impaired balance ( J:146493 )

• significantly poorer performance on an accelerating rotarod compared to controls by 3 weeks of age

abnormal physical strength ( J:146493 )

decreased grip strength ( J:146493 )

• progressive age-dependent impairment

weaving ( J:146493 )

• listing

bradykinesia ( J:146493 )

• age-dependent progressive bradykinesia

paralysis ( J:146493 )

• progressive loss of locomotor ability

paraparesis ( J:146493 )

• age-dependent progressive and often asymmetric hind limb weakness

growth/size/body

decreased body size ( J:146493 )

• decrease in size compared to controls starting at about 3 weeks of age

decreased body weight ( J:146493 )

• starting at about 3 weeks of age

slow postnatal weight gain ( J:146493 )

nervous system

astrocytosis ( J:146493 )

• astrogliosis in the midbrain, brainstem and spinal cord starting at about 6 weeks of age

• mild astrogliosis is also seen in the thalamus and hypothalamus

abnormal neuron morphology ( J:146493 )

• dying neurons are present in the anterior horn of the lumbar region of the spinal cord

• post synaptic profiles often appear swollen with vacuolated mitochondria

abnormal neurite morphology ( J:146493 )

• dystrophic neurites are found in the brainstem and spinal cord

neuron degeneration ( J:146493 )

• scattered degenerating neurons are found in the white and gray matter

• axonal degeneration of sensory roots during disease progression

motor neuron degeneration ( J:146493 )

• in the lumbar region of the spinal cord

• axonal degeneration of motor roots during disease progression

• larger caliber axons are preferentially lost

• some distal ends of the femoral nerve are severely degenerated

abnormal neuron physiology ( J:146493 )

• hyperphosphorylation of MAPT proteins

muscle

decreased skeletal muscle fiber size ( J:146493 )

• reduced muscle fiber size

muscular atrophy ( J:146493 )

abnormal muscle regeneration ( J:146493 )

• isolated regeneration of muscle fibers is seen

Genotype
MGI:3838109

ht2

• Allelic Composition: Ltn1^{Gt(RRR322)Byg}/Ltn1^lister
• Genetic Background: involves: 129P2/OlaHsd * C57BL/6

behavior/neurological

abnormal motor capabilities/coordination/movement ( J:146493 )

• progressive motor deficits similar to those in mice homozygous for Rnf160^lister

limb grasping ( J:146493 )

• similar to mice homozygous for Rnf160^lister