Phenotypes associated with this allele

• Allele Symbol: Tg(Thy1-Sncg)HvP36Putt
• Allele Name: transgene insertion HvP36, Herman van der Putten
• Allele ID: MGI:4440514
• Summary: 3 genotypes

Jump to	Allelic Composition	Genetic Background	Genotype ID
tg1	Tg(Thy1-Sncg)HvP36Putt/Tg(Thy1-Sncg)HvP36Putt	C57BL/6-Tg(Thy1-Sncg)HvP36Putt	MGI:4440540
tg2	Tg(Thy1-Sncg)HvP36Putt/0	C57BL/6-Tg(Thy1-Sncg)HvP36Putt	MGI:4440541
tg3	Tg(Thy1-Sncg)HvP36Putt/?	C57BL/6-Tg(Thy1-Sncg)HvP36Putt	MGI:5431238

Genotype
MGI:4440540

tg1

• Allelic Composition: Tg(Thy1-Sncg)HvP36Putt/Tg(Thy1-Sncg)HvP36Putt
• Genetic Background: C57BL/6-Tg(Thy1-Sncg)HvP36Putt

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

mortality/aging

premature death ( J:147580 )

• mice do not survive beyond 16 months

behavior/neurological

limb grasping ( J:147580 )

• at 6 to 9 months

abnormal motor coordination/balance ( J:147580 )

impaired righting response ( J:147580 )

• following paresis

impaired balance ( J:147580 )

• earlier than 6 months

impaired coordination ( J:147580 )

• earlier than 6 months

hunched posture ( J:147580 )

• at 6 to 9 months

abnormal gait ( J:147580 )

• at 6 to 9 months

short stride length ( J:147580 )

• at 6 months

paralysis ( J:147580 )

• following paresis

paresis ( J:147580 )

• at 9 to 16 months, mice develop paresis unlike wild-type mice with earlier development in forelimbs than in hindlimbs

nervous system

abnormal nervous system morphology ( J:147580 )

• mice exhibit aggregation and fibrillation of gamma-synuclein in the nervous system unlike wild-type mice

• abnormal gamma-synuclein structures are most abundant in the spinal cord

astrocytosis ( J:147580 )

• in the spinal cord gray matter of severely affected and older mice

decreased motor neuron number ( J:147580 )

• at 12 months, motor neuron numbers in severely affected mice are less than 40% of wild-type

• motor neuron loss corresponds to severity of motor impairment

homeostasis/metabolism

amyloidosis ( J:147580 )

• in the spinal cord or brain tissue when mild clinical signs of pathology are apparent

• the number of inclusions increases as clinical phenotypes worsen

Genotype
MGI:4440541

tg2

• Allelic Composition: Tg(Thy1-Sncg)HvP36Putt/0
• Genetic Background: C57BL/6-Tg(Thy1-Sncg)HvP36Putt

behavior/neurological

abnormal motor coordination/balance ( J:147580 )

• mice develop motor dysfunction compared with wild-type mice with greater latency and slower progression than homozygous mice

impaired coordination ( J:147580 )

• after 18 months of age, mice exhibit impaired performance on a rotarod compared with wild-type mice

short stride length ( J:147580 )

• at 18 months, mice have shorter strides than wild-type mice

nervous system

astrocytosis ( J:147580 )

• in older mice

decreased motor neuron number ( J:147580 )

• motor neuron loss corresponds to severity of motor impairment

homeostasis/metabolism

amyloidosis ( J:147580 )

• in the spinal cord or brain tissue when mild clinical signs of pathology are apparent

• the number of inclusions increases as clinical phenotypes worsen

Genotype
MGI:5431238

tg3

• Allelic Composition: Tg(Thy1-Sncg)HvP36Putt/?
• Genetic Background: C57BL/6-Tg(Thy1-Sncg)HvP36Putt

nervous system

nervous system phenotype ( J:185793 )

N • retinal ganglion cells do not exhibit deterioration or loss despite overexpression of gamma-synuclein

gliosis ( J:185793 )

• gliosis is observed within motor regions of cortex

microgliosis ( J:185793 )

• observed in the trigeminal motor nucleus in affected 12 month old mice

astrocytosis ( J:185793 )

• observed in the trigeminal motor nucleus in affected 12 month old mice

decreased motor neuron number ( J:185793 )

• loss of motor neurons is observed in the trigeminal motor nucleus, but not in the facial and abducens motor nuclei

• neuron loss is observed in select upper and lower motor neuron populations of the motor cortex, and to a lesser degree in the somatosensory regions

• neuron loss is correlated with perikaryal and axonal accumulation of gamma-synuclein, accumulation of detergent-insoluble species and gliosis

abnormal myelin sheath morphology ( J:185793 )

• damage to myelin sheath is observed in substantial numbers of myelinated fibers from the sciatic nerve in severely affected mice

neuron degeneration ( J:185793 )

• selective neuronal dystrophy is observed in the cortex

abnormal trigeminal nerve morphology ( J:185793 )

• significant neuron loss is observed in affected 12 month old mice

• mice exhibit high levels of astrogliosis and microgliosis in the trigeminal motor nucleus

abnormal dorsal spinal root morphology ( J:185793 )

• some abnormal myelinated fibers and phagocytes are observed in the dorsal spinal root

• however, the majority of myelinated fibers are similar to control in number and condition

abnormal sciatic nerve morphology ( J:185793 )

• deterioration and loss of myelinated axons in severely affected mice

• reduction in total numbers of myelinated fibers in severely affected mice

• reduced levels of neurofilament proteins, alpha tubulin, and myelin basic protein are observed in severely affected mice

• active Wallerian-like degeneration of the sciatic nerve is observed

• mildly affected mice exhibit slight changes in morphology, but no loss of fibers

abnormal ventral spinal root morphology ( J:185793 )

• deterioration and loss of myelinated fibers in severely affected mice

• significant decrease in mean myelinated fiber size (49.5%) found in remaining myelinated fibers

• extensive phagocyte infiltration

hematopoietic system

microgliosis ( J:185793 )

• observed in the trigeminal motor nucleus in affected 12 month old mice

immune system

microgliosis ( J:185793 )

• observed in the trigeminal motor nucleus in affected 12 month old mice

Mouse Models of Human Disease		DO ID	OMIM ID(s)	Ref(s)
amyotrophic lateral sclerosis type 1		DOID:0060193	OMIM:105400	J:185793