Phenotypes associated with this allele

• Allele Symbol: Dync1h1^Cra1
• Allele Name: cramping 1
• Allele ID: MGI:2661085
• Summary: 4 genotypes

Jump to	Allelic Composition	Genetic Background	Genotype ID
hm1	Dync1h1^Cra1/Dync1h1^Cra1	C3HeB/FeJ-Dync1h1^Cra1	MGI:3722059
ht2	Dync1h1^Cra1/Dync1h1^+	C3HeB/FeJ-Dync1h1^Cra1	MGI:3721971
ht3	Dync1h1^Cra1/Dync1h1^+	involves: C3HeB/FeJ * C57BL/6	MGI:3722064
cx4	Dync1h1^Cra1/Dync1h1^+ Tg(SOD1*G93A)1Gur/?	involves: C3HeB/FeJ * C57BL/6 * SJL	MGI:3721969

Genotype
MGI:3722059

hm1

• Allelic Composition: Dync1h1^Cra1/Dync1h1^Cra1
• Genetic Background: C3HeB/FeJ-Dync1h1^Cra1

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

mortality/aging

neonatal lethality, complete penetrance ( J:83128 )

• mice die within 24 hours of birth

nervous system

neuronal intranuclear inclusions ( J:83128 )

• at E18.5, surviving neurons have inclusion bodies

decreased spinal cord ventral horn cell number ( J:83128 )

• at E18.5, mice have lost 80% of anterior horn cells

• apoptosis levels in the anterior horn cells is increased

behavior/neurological

aphagia ( J:83128 )

• at birth mice cannot feed

paralysis ( J:83128 )

• at birth mice cannot move

Genotype
MGI:3721971

ht2

• Allelic Composition: Dync1h1^Cra1/Dync1h1⁺
• Genetic Background: C3HeB/FeJ-Dync1h1^Cra1

nervous system

decreased motor neuron number ( J:83128 )

• alpha motor neurons in the spinal cord anterior horn are decreased in number at 16 and 19 months

motor neuron degeneration ( J:83128 )

behavior/neurological

impaired coordination ( J:83128 , J:107901 )

• latency to fall in a hanging testis reduced at 16 months of age (J:83128)

• mice show a reduced rotarod performance and reached latency to fall after 18 seconds on day 200 (J:107901)

decreased grip strength ( J:83128 )

• hindlimb and forelimb grip strength is reduced and 3 months and worsens at 16 months

muscle

muscle spasm ( J:83128 )

• mice exhibit cramping when hung by their tails in which the whole body would start to tremble, then the forelimbs cramped, the neck stretched, the mouth opened and the tongue stuck out

Genotype
MGI:3722064

ht3

• Allelic Composition: Dync1h1^Cra1/Dync1h1⁺
• Genetic Background: involves: C3HeB/FeJ * C57BL/6

muscle

muscle spasm ( J:83128 )

• Background Sensitivity: authors state that the phenotype observed is changed and milder than in Dync1h1^Cra1 homozygotes

Genotype
MGI:3721969

cx4

• Allelic Composition: Dync1h1^Cra1/Dync1h1⁺; Tg(SOD1*G93A)1Gur/?
• Genetic Background: involves: C3HeB/FeJ * C57BL/6 * SJL

mortality/aging

premature death ( J:107901 )

• mice reach end stage by day 167 +/-2.5 SEM (standard error of measurement)

nervous system

astrocytosis ( J:107901 )

• astrocytosis is evident in the cervical and lumbar spinal cord

abnormal spinal cord morphology ( J:107901 )

• degeneration of the motor neurons in the cervical and lumbar spinal cord at end stage with astrocytosis and vacuolization

decreased motor neuron number ( J:107901 )

• degeneration of the motor neurons in the cervical and lumbar spinal cord is visible at end stage

motor neuron degeneration ( J:107901 )

• degeneration of the motor neurons in the cervical and lumbar spinal cord is visible at end stage

behavior/neurological

decreased locomotor activity ( J:107901 )

• motor activity is less than in Tg(SOD1*G93A)1Gur mice but does not deteriorate as quickly

growth/size/body

decreased body weight ( J:107901 )

• weight reduction was not as severe as in Tg(SOD1*G93A)1Gur mice