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Phenotypes associated with this allele
Allele Symbol
Allele Name
Allele ID
Tg(HDexon1)62Gpb
transgene insertion 62, Gillian Bates
MGI:2386951
Summary 11 genotypes
Jump to Allelic Composition Genetic Background Genotype ID
cx1
Sirt2tm1Gdon/Sirt2tm1Gdon
Tg(HDexon1)62Gpb/0
involves: 129P2/OlaHsd * C57BL/6 * CBA MGI:5467291
cx2
Supt4atm1Yty/Supt4a+
Tg(HDexon1)62Gpb/0
involves: 129S6/SvEvTac * C57BL/6 * CBA MGI:5763069
cx3
Tgm2tm1.1Rmgr/Tgm2+
Tg(HDexon1)62Gpb/0
involves: 129X1/SvJ * C57BL/6 * CBA MGI:3528067
cx4
Tgm2tm1.1Rmgr/Tgm2tm1.1Rmgr
Tg(HDexon1)62Gpb/0
involves: 129X1/SvJ * C57BL/6 * CBA MGI:3528066
tg5
Tg(HDexon1)62Gpb/0 B6CBA-Tg(HDexon1)62Gpb/1J MGI:3694681
tg6
Tg(HDexon1)62Gpb/0 B6CBA-Tg(HDexon1)62Gpb/3J MGI:5429322
tg7
Tg(HDexon1)62Gpb/0 B6.Cg-Tg(HDexon1)62Gpb/240 MGI:5429327
tg8
Tg(HDexon1)62Gpb/0 involves: C57BL/6 * C57BL/6JOlaHsd * CBA * CBA/CaOlaHsd MGI:5467290
tg9
Tg(HDexon1)62Gpb/0 involves: C57BL/6 * CBA MGI:3757568
tg10
Tg(HDexon1)62Gpb/0 involves: C57BL/6 * CBA/J MGI:2653604
tg11
Tg(HDexon1)62Gpb/0 involves: C57BL/6J * CBA/J MGI:3530234


Genotype
MGI:5467291
cx1
Allelic
Composition
Sirt2tm1Gdon/Sirt2tm1Gdon
Tg(HDexon1)62Gpb/0
Genetic
Background
involves: 129P2/OlaHsd * C57BL/6 * CBA
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Sirt2tm1Gdon mutation (0 available); any Sirt2 mutation (20 available)
Tg(HDexon1)62Gpb mutation (12 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
behavior/neurological
• as in Tg(HDexon1)62Gpb mice
• as in Tg(HDexon1)62Gpb mice

nervous system
• as in Tg(HDexon1)62Gpb mice




Genotype
MGI:5763069
cx2
Allelic
Composition
Supt4atm1Yty/Supt4a+
Tg(HDexon1)62Gpb/0
Genetic
Background
involves: 129S6/SvEvTac * C57BL/6 * CBA
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Supt4atm1Yty mutation (0 available); any Supt4a mutation (13 available)
Tg(HDexon1)62Gpb mutation (12 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
mortality/aging
• mice exhibit premature death compared with wild-type mice but improved survival compared with Tg(HDexon1)62Gpb mice

behavior/neurological
• impaired performance on a rotarod is delayed until 13 weeks of age compared with 10 weeks as in Tg(HDexon1)62Gpb mice
• improved beam walking compared with Tg(HDexon1)62Gpb mice

growth/size/body
• as in Tg(HDexon1)62Gpb mice




Genotype
MGI:3528067
cx3
Allelic
Composition
Tgm2tm1.1Rmgr/Tgm2+
Tg(HDexon1)62Gpb/0
Genetic
Background
involves: 129X1/SvJ * C57BL/6 * CBA
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Tg(HDexon1)62Gpb mutation (12 available)
Tgm2tm1.1Rmgr mutation (2 available); any Tgm2 mutation (37 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
nervous system
• mutants had more huntingtin protein aggregates within the cerebral cortex and striatum but no difference in the onset of motor dysfunction and the mean lifespan compared to Tg(HDexon1)62Gpb transgenic mice
• mutants had more huntingtin protein aggregates within the cerebral cortex and striatum but no difference in the onset of motor dysfunction and the mean lifespan compared to Tg(HDexon1)62Gpb transgenic mice




Genotype
MGI:3528066
cx4
Allelic
Composition
Tgm2tm1.1Rmgr/Tgm2tm1.1Rmgr
Tg(HDexon1)62Gpb/0
Genetic
Background
involves: 129X1/SvJ * C57BL/6 * CBA
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Tg(HDexon1)62Gpb mutation (12 available)
Tgm2tm1.1Rmgr mutation (2 available); any Tgm2 mutation (37 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
mortality/aging
• mean lifespan of mutants was increased by 19.7% compared Tg(HDexon1)62Gpb transgenic mice

behavior/neurological
• mutants showed a delayed onset of motor dysfunction compared to Tg(HDexon1)62Gpb transgenic mice at 9, 10, and 11 weeks of age

nervous system
• 10 week-old mutants had 30-35% more huntingtin protein aggregates within the cerebral cortex and striatum than Tg(HDexon1)62Gpb transgenic mice
• 10 week-old mutants had 30-35% more huntingtin protein aggregates within the cerebral cortex and striatum than Tg(HDexon1)62Gpb transgenic mice




Genotype
MGI:3694681
tg5
Allelic
Composition
Tg(HDexon1)62Gpb/0
Genetic
Background
B6CBA-Tg(HDexon1)62Gpb/1J
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Tg(HDexon1)62Gpb mutation (12 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
cardiovascular system
• mitochondria in cardiomyocytes examined at 12 weeks of age exhibit a circular appearance with a disruption of uniform densities
• significantly reduced at 12 weeks of age
• mice exhibit left ventricular dilation in comparison to controls
• detected as early as 8 weeks of age and reduced to 50% by 12 weeks of age
• mice exhibit progressive reduction in stroke volume, although there are no differences in heart rate
• transmitral flow velocity is altered as compared to controls
• E/A ratio is significantly decreased at all time points
• mice exhibit a decrease in passive ventricular filling and a significant increase in atrial-mediated left ventricle filling
• diminished left ventricular wall motion during systole
• left ventricle fractional shortening significantly impaired at 10 and 12 weeks of age
• diminished left ventricular wall motion during systole
• left ventricle fractional shortening significantly impaired at 10 and 12 weeks of age

growth/size/body
• mice exhibited less weight gain over time than controls

muscle
• mitochondria in cardiomyocytes examined at 12 weeks of age exhibit a circular appearance with a disruption of uniform densities
• diminished left ventricular wall motion during systole
• left ventricle fractional shortening significantly impaired at 10 and 12 weeks of age

behavior/neurological
• exhibit clasping of both fore and hind paws
• impaired mobility in home cages

nervous system
• 3-hydroxykynurenine (3-HK) levels are significantly elevated in the striatum at 4 weeks of age, a phenotype observed in Huntington disease patients
• 3-HK levels are significantly elevated in the cortex at 4 weeks of age, a phenotype observed in Huntington disease patients
• 3-HK levels are significantly elevated in the cerebellum at 4 weeks of age, a phenotype observed in Huntington disease patients
• striatal and cortical mitochondria are equally resistant to calcium at 8 and 12 weeks of age, while in controls, striatal mitochondria is more sensitive to calcium
• striatal and cortical neurons display more rapid and increased swelling to N-methyl-D-aspartate (NMDA) than controls, but not to alpha-amino-3-hydroxy-5-methyl-4-isoxazole propionic acid (AMPA) or kainate (KA), indicating increased sensitivity to NMDA
• intracellular recordings show that resting membrane potentials of striatal neurons are significantly more depolarized than in controls

Mouse Models of Human Disease
DO ID OMIM ID(s) Ref(s)
Huntington's disease DOID:12858 OMIM:143100
J:99425 , J:111237




Genotype
MGI:5429322
tg6
Allelic
Composition
Tg(HDexon1)62Gpb/0
Genetic
Background
B6CBA-Tg(HDexon1)62Gpb/3J
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Tg(HDexon1)62Gpb mutation (12 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
behavior/neurological
• increased preference for dark in dark/light choice test at 12 and 14 weeks of age
• beginning at 4 weeks of age
• mice exhibit a reduced latency to fall on rotarod test beginning at 4 weeks of age as compared to wild-type
• latency time decreases with age
• progressive deterioration of grip strength especially in males between 10-14 weeks of age
• mice rear less in the center of the open field test than controls at all ages in the light phase and by 6 weeks of age in the dark phase
• mice exhibit a progressive decrease in climbing activity starting at 4 weeks of age
• mice are hypoactive in the open field test in both the light and dark phases of the light cycle
• in the light phase females are less active at 8 and 14 weeks of age
• in the light phase males are less active starting at 6 weeks of age
• in the dark phase both sexes are hypoactive beginning at 12 weeks of age
• progressive hypoactivity is observed in the center of the open field beginning at 4 weeks of age
• wider base (separation between legs) than controls; females exhibit difference earlier than males
• decreased stride length (ipsilateral) observed at 8 weeks of age
• shorter splay length (contralateral) than controls

growth/size/body
• decrease in body weight is observed by 7 weeks in both genders

mortality/aging
• median survival of 113 days
• all mice die within 12 weeks of the first death

nervous system
• observed after 8 weeks of age




Genotype
MGI:5429327
tg7
Allelic
Composition
Tg(HDexon1)62Gpb/0
Genetic
Background
B6.Cg-Tg(HDexon1)62Gpb/240
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Tg(HDexon1)62Gpb mutation (12 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
behavior/neurological
• increased preference for dark in dark/light choice test at 14 weeks of age
• observed at 6 and 14 weeks of age in both genders
• mice exhibit a progressive impairment on the rotarod test beginning at 8 weeks of age
• progressive deterioration of grip strength especially in males at 14 weeks of age
• mice rear less in the center of the open field test than controls starting at 12 weeks of age in the light phase
• rearing is similar to controls in dark phase
• mice cover less total distance in light phase of open field test starting at 6 weeks of age in females and 8 weeks of age in males
• in dark phase, hypoactivity is significant at 6 weeks of age in both genders
• mice cover less distance in center starting at 12 weeks in light phase and 6 weeks in dark phase
• narrower base (separation between legs) than controls
• shorter splay length (contralateral) than controls at 14 weeks of age
• stride length (ipsilateral) is similar to controls

growth/size/body
• body weight decreases by 10 weeks of age in females and 9 weeks in males

mortality/aging
• median survival of 141 days for females and 179 days for males
• all mice die within 9 weeks of the first death

nervous system
• observed at 14 weeks of age, especially in females




Genotype
MGI:5467290
tg8
Allelic
Composition
Tg(HDexon1)62Gpb/0
Genetic
Background
involves: C57BL/6 * C57BL/6JOlaHsd * CBA * CBA/CaOlaHsd
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Tg(HDexon1)62Gpb mutation (12 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
behavior/neurological

nervous system




Genotype
MGI:3757568
tg9
Allelic
Composition
Tg(HDexon1)62Gpb/0
Genetic
Background
involves: C57BL/6 * CBA
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Tg(HDexon1)62Gpb mutation (12 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
nervous system
• reduction in brain weight by 5 weeks
• immunoreactive htt is detected in the striatum at 4.5 weeks
• immunoreactive htt is detected in the cortex beginning at 3.5 weeks
• striatal neurons have prominent and frequent indentations of the nuclear membrane and an apparent increase in the clustering and number of nuclear pores by 10-12 weeks of age
• inclusions are observed within neurons of cerebral cortex, striatum, cerebellum, spinal cord and to a much lesser degree in the hippocampus, thalamus, globus pallidus and substantia nigra
• inclusions appear in the cerebral cortex before they can be detected in the striatum
• inclusions are ubiquitinated by 5-6 weeks and can be detected by ultrastructural analysis by 8 weeks
• htt immunoreactive inclusions are seen in approximately 20% of neurons
• in the striatum, ultrastructural analysis of inclusions reveals a prominent, roughly circular, pale structure
• inclusions are granular with occasional filamentous structures around the periphery; they are larger than the nucleolus and occupy 1% of nuclear volume

growth/size/body
• progressive loss of body weight

Mouse Models of Human Disease
DO ID OMIM ID(s) Ref(s)
Huntington's disease DOID:12858 OMIM:143100
J:42085




Genotype
MGI:2653604
tg10
Allelic
Composition
Tg(HDexon1)62Gpb/0
Genetic
Background
involves: C57BL/6 * CBA/J
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Tg(HDexon1)62Gpb mutation (12 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
vision/eye
• retinal dysplasia covers more than 50% of the entire retina
• misplaced photoreceptor nuclei are seen in the segment layers at 10 weeks of age
• inner segment is reduced in thickness at 10 weeks of age
• seen at 10 weeks of age
• outer segment is reduced in thickness at 10 weeks of age
• seen at 10 weeks of age
• outer nuclear layer of the retina exhibits an irregular and wavy shape, disrupted with folds and whorls at 10 weeks of age
• misplaced photoreceptor nuclei are seen in the outer plexiform layer at 10 weeks of age
• however, no inner plexiform layer or ganglion cell layer abnormalities

nervous system
• misplaced photoreceptor nuclei are seen in the segment layers at 10 weeks of age
• inner segment is reduced in thickness at 10 weeks of age
• seen at 10 weeks of age
• outer segment is reduced in thickness at 10 weeks of age
• seen at 10 weeks of age
• nuclear inclusions are seen in the retina




Genotype
MGI:3530234
tg11
Allelic
Composition
Tg(HDexon1)62Gpb/0
Genetic
Background
involves: C57BL/6J * CBA/J
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Tg(HDexon1)62Gpb mutation (12 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
mortality/aging
• usually between 10 to 13 weeks of age
• mice can die suddenly

nervous system
• handling induced seizures that can be several minutes duration
• average of 19% smaller than wildtype

behavior/neurological
• dyskinesia of limbs when suspended by the tail
• progressive resting tremor in limbs, trunk and head
• involuntary jerky shudders
• mice lose balance when sitting on hind limbs, turning and grooming their backs
• onset of motor impairment as early as 4 weeks of age
• stereotypic repetitive stroking of the nose and face, hind limb kicking and scratching movement
• 2 distinct characteristic vocalizations observed
• handling induced seizures that can be several minutes duration

endocrine/exocrine glands
• islets exhibit huntingtin inclusions in 24% of cells by 12 weeks of age
• islets exhibit huntingtin inclusions in 19% of cells by 7 weeks of age, by week 12 frequency is greater than 95%
• islets are hypotrophic by 12 weeks, BrdU incorporation is reduced 6-fold
• significant reduction in insulin and somatostatin content by 12 weeks
• islets are smaller in size by 12 weeks
• islets contain few insulin secretory vesicles by 12 weeks
• by 12 weeks, no signs of apoptosis or necrosis are observed
• islets exhibit huntingtin inclusions in 6% of cells by 12 weeks of age
• small seminal ducts and gland size

growth/size/body
• body weight declines after 10 weeks
• body weight is normal at weaning
• with progression of phenotype up to 30% of body weight can be lost
• overall loss of muscle bulk observed

homeostasis/metabolism
• abnormal glucose homeostasis
• insulin secretion in response to glucose and KCl is decreased 4-fold and 2.5-fold, respectively, by 12 weeks of age
• identified at 11.5 weeks of age, insulin response absent

renal/urinary system

reproductive system
• small seminal ducts and gland size
• smaller size ovaries and uteri often observed

Mouse Models of Human Disease
DO ID OMIM ID(s) Ref(s)
Huntington's disease DOID:12858 OMIM:143100
J:36689





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last database update
10/19/2021
MGI 6.17
The Jackson Laboratory