Phenotypes Associated with This Genotype

Genotype
MGI:3713123

• Allelic Composition: Atg5^tm1Myok/Atg5^tm1Myok; Tg(Nes-cre)1Kln/?
• Genetic Background: involves: 129S/SvEv * C57BL/6 * SJL

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

mortality/aging

premature death ( J:110050 )

• some mice die after 3 weeks

nervous system

abnormal brain morphology ( J:110050 )

• ubiquitin-positive inclusions are found in the thalamus pon, medulla, dorsal root ganglion, midbrain, cerebral cortex, hippocampus (especially in CA3 and CA4), striatum and olfactory bulb autophagosomes formation in the brain is impaired

abnormal inferior colliculus morphology ( J:110050 )

• axonal swelling

abnormal thalamus morphology ( J:110050 )

• axonal swelling in the posterior thalamic nucleus

abnormal hippocampus morphology ( J:110050 )

• axonal swelling

abnormal cerebral cortex morphology ( J:110050 )

• axonal swelling

decreased cerebral cortex pyramidal cell number ( J:110050 )

• loss of pyramidal cells in the cerebral cortex

abnormal cerebellar granule cell morphology ( J:110050 )

• apoptosis is detected in granular cells

abnormal axon morphology ( J:110050 )

• axonal swelling in the cerebral cortex, nucleus gracilis, posterior thalamic nucleus, hippocampus, inferior colliculus, tricaudal pons and reticular nucleus

neuron degeneration ( J:110050 )

Purkinje cell degeneration ( J:110050 )

• however, few degenerating Purkinje cells exhibit inclusions

neuronal intranuclear inclusions ( J:110050 )

• ubiquitin-positive inclusions are found in the thalamus pon, medulla, dorsal root ganglion, midbrain, cerebral cortex, hippocampus (especially in CA3 and CA4), striatum and olfactory bulb

• inclusions are time-dependent and are more limited in newborns than in adults

behavior/neurological

abnormal behavior ( J:110050 )

• progressive motor and behavioral deficits after three weeks of age

abnormal suckling behavior ( J:110050 )

abnormal motor capabilities/coordination/movement ( J:110050 )

• progressive motor deficits after three weeks of age including ataxia and a decrease in mean stride length

limb grasping ( J:110050 )

• when suspended by their tails

tremors ( J:110050 )

• at 12 weeks

ataxia ( J:110050 )

impaired coordination ( J:110050 )

• coordination, balance and grip strength are impaired in a rotarod and wire hang task

decreased grip strength ( J:110050 )

• grip strength is impaired in a rotarod and wire hang task

short stride length ( J:110050 )

cellular

abnormal cell physiology ( J:110050 )

• autophagosomes formation in the brain is impaired

growth/size/body

decreased body weight ( J:110050 )

• body weight is about 1.5-times lower than that of wild-type

postnatal growth retardation ( J:110050 )