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Phenotypes Associated with This Genotype
Genotype
MGI:3723505
Allelic
Composition
Tg(HD)63Aron/?
Genetic
Background
involves: C57BL/6 * SJL
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Tg(HD)63Aron mutation (1 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
nervous system
• htt staining is substantially increased in the cytoplasm of striatal neurons as compared to control (J:72772)
• htt staining is substantially increased in the cytoplasm of striatal neurons as compared to control (J:72772)
• cytoplasmic huntingtin accumulation is prevalent in neurons of the frontal and cingulated cortices and occasionally present in the piriform and hippocampal cortices (J:72772)
• cortical pyramidal neurons exhibit retraction and disorientation of the apical dendrite, as well as dendritic abnormalities such as beading, small sharp bends, misalignment and bifurcation (J:72772)
• cytoplasmic huntingtin accumulation is prevalent in neurons of the frontal and cingulated cortices and occasionally present in the piriform and hippocampal cortices (J:72772)
• cortical pyramidal neurons exhibit retraction and disorientation of the apical dendrite, as well as dendritic abnormalities such as beading, small sharp bends, misalignment and bifurcation (J:72772)
• one severely affected mouse exhibited shrunken neurons in the hippocampus and cerebellum (J:72772)
• intracytoplasmic vacuoles and plasma membrane blebs appear in cell bodies and dendrites of neurons with an accumulation of huntingtin (J:72772)
• one severely affected mouse exhibited shrunken neurons in the hippocampus and cerebellum (J:72772)
• intracytoplasmic vacuoles and plasma membrane blebs appear in cell bodies and dendrites of neurons with an accumulation of huntingtin (J:72772)
• dendrites in spiny neurons have significantly more curved endings (J-dendrites) and sharp bends (wavy dendrites) than control (J:72772)
• dendrites in spiny neurons have significantly more curved endings (J-dendrites) and sharp bends (wavy dendrites) than control (J:72772)
• cortical pyramidal neurons exhibit retraction and disorientation of the apical dendrite, as well as dendritic abnormalities such as beading, small sharp bends, misalignment and bifurcation (J:72772)
• some, but not all, mice exhibit dysmorphic dendrites in hippocampal pyramidal neurons (J:72772)
• one severely affected mouse had dysmorphic dendrites in cerebellar Purkinje cells (J:72772)
• dendrites in spiny neurons have significantly more curved endings (J-dendrites) and sharp bends (wavy dendrites) than control (J:72772)
• cortical pyramidal neurons exhibit retraction and disorientation of the apical dendrite, as well as dendritic abnormalities such as beading, small sharp bends, misalignment and bifurcation (J:72772)
• some, but not all, mice exhibit dysmorphic dendrites in hippocampal pyramidal neurons (J:72772)
• one severely affected mouse had dysmorphic dendrites in cerebellar Purkinje cells (J:72772)
• dendrites in spiny neurons have significantly more curved endings (J-dendrites) and sharp bends (wavy dendrites) than control (J:72772)
• intranuclear inclusions are detected in the cytoplasm of cortical neurons and to a lesser degree in striatal neurons (J:72772)
• in a sample of five mice, 27-70% of large striatal neurons that labeled for huntingtin had inclusions (J:72772)
• nuclear inclusions are composed of granules and filaments as determined by electron microscopy (J:72772)
• in 11 of 14 mice, inclusions appeared in a few cells in the hippocampus, substantia nigra, cerebellum and brainstem (J:72772)
• intranuclear inclusions are detected in the cytoplasm of cortical neurons and to a lesser degree in striatal neurons (J:72772)
• in a sample of five mice, 27-70% of large striatal neurons that labeled for huntingtin had inclusions (J:72772)
• nuclear inclusions are composed of granules and filaments as determined by electron microscopy (J:72772)
• in 11 of 14 mice, inclusions appeared in a few cells in the hippocampus, substantia nigra, cerebellum and brainstem (J:72772)
• stimulation of corpus callosum slices evoked smaller EPSPs in striatal neurons as compared to control (J:72772)
• response to stimulus in striatal neurons results in a rightward shift in the input-output curve (EPSP amplitude) as compared to control (J:72772)
• stimulation of corpus callosum slices evoked smaller EPSPs in striatal neurons as compared to control (J:72772)
• response to stimulus in striatal neurons results in a rightward shift in the input-output curve (EPSP amplitude) as compared to control (J:72772)
• one population of mutant neurons (most affected) exhibit higher peak currents and current densities when induced by NMDA as well as an increase in calcium ion influx (J:72772)
• one population of mutant neurons (most affected) exhibit higher peak currents and current densities when induced by NMDA as well as an increase in calcium ion influx (J:72772)

behavior/neurological
• most mice exhibit clasping in the tail suspension test (J:72772)
• most mice exhibit clasping in the tail suspension test (J:72772)
• in rotarod performance (J:72772)
• in rotarod performance (J:72772)
• some mice exhibit an alteration in activity that is scored as hypo- or hyperactivity (J:72772)
• some mice exhibit an alteration in activity that is scored as hypo- or hyperactivity (J:72772)
• some mice exhibit an abnormal gait which could include wide-based gait, walking with an arched posture and slow gait (J:72772)
• some mice exhibit an abnormal gait which could include wide-based gait, walking with an arched posture and slow gait (J:72772)

Mouse Models of Human Disease
OMIM ID Ref(s)
Huntington Disease; HD 143100 J:72772


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
02/02/2016
MGI 6.02
The Jackson Laboratory