Phenotypes Associated with This Genotype

Genotype
MGI:5306156

cn6

• Allelic Composition: Itgav^tm2Hyn/Itgav^tm2.1Hyn; Tg(Nes-cre)1Kln/0
• Genetic Background: involves: 129P2/OlaHsd * 129S4/SvJae * C57BL/6 * FVB * SJL

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

mortality/aging

increased susceptibility to bacterial infection induced morbidity/mortality ( J:94376 )

• mice have to be euthanized by 8 months due to urinary dysfunction and bacterial bladder infection

premature death ( J:94376 )

• some mice die by 4 weeks of age

• mice have to be euthanized by 8 months due to urinary dysfunction and bacterial bladder infection

nervous system

nervous system phenotype ( J:94376 )

N • mice do not exhibit peripheral nerve abnormalities

seizures ( J:94376 )

• more frequent by 6 months

convulsive seizures ( J:94376 )

abnormal brain vasculature morphology ( J:94376 )

• vascular abnormalities are initiated in the developing ganglionic eminences of the forebrain

• cerebral blood vessels are tortuous and distended compared to in control mice

intracranial hemorrhage ( J:94376 )

• microhemorrhage in the brain at 3 weeks

intracerebral hemorrhage ( J:94376 )

• at E14.5, mice develop small bilateral cerebral hemorrhages that become progressively more severe throughout embryogenesis unlike control mice

• less severe than in null mice

spinal hemorrhage ( J:94376 )

• microhemorrhage in the spinal cord at 3 weeks

abnormal glial cell morphology ( J:94376 )

• glial degeneration in the fasciculus gracilis white matter region

gliosis ( J:94376 )

abnormal embryonic/fetal subventricular zone morphology ( J:94376 )

• vascular abnormalities are initiated in the developing ganglionic eminences of the forebrain

axon degeneration ( J:94376 )

• in the fasciculus gracilis

axonal dystrophy ( J:94376 )

• in the fasciculus gracilis

demyelination ( J:94376 )

• in the spinal cord white matter

behavior/neurological

abnormal motor capabilities/coordination/movement ( J:94376 )

• by 2 to 3 weeks, some mice develop motor dysfunction

• by 2 to 3 months, all mice exhibit hind limb coordination defects

abnormal involuntary movement ( J:94376 )

• mice exhibit involuntary tail wriggling

ataxia ( J:94376 )

impaired coordination ( J:94376 )

• on a rotarod

impaired limb coordination ( J:94376 )

• mice partially drag hind limbs

abnormal posture ( J:94376 )

• rigid at 2 to 3 months

abnormal gait ( J:94376 )

• rigid at 2 to 3 months

• mice exhibit toe-walking

• mice partially drag hind limbs

paraparesis ( J:94376 )

• spastic paraparesis by 6 months

seizures ( J:94376 )

• more frequent by 6 months

convulsive seizures ( J:94376 )

cardiovascular system

abnormal brain vasculature morphology ( J:94376 )

• vascular abnormalities are initiated in the developing ganglionic eminences of the forebrain

• cerebral blood vessels are tortuous and distended compared to in control mice

intracranial hemorrhage ( J:94376 )

• microhemorrhage in the brain at 3 weeks

intracerebral hemorrhage ( J:94376 )

• at E14.5, mice develop small bilateral cerebral hemorrhages that become progressively more severe throughout embryogenesis unlike control mice

• less severe than in null mice

spinal hemorrhage ( J:94376 )

• microhemorrhage in the spinal cord at 3 weeks

muscle

muscle phenotype ( J:94376 )

N • mice do not develop hind limb muscular atrophy

muscle hypertonia ( J:94376 )

• in hind limb muscles at 2 to 3 months

renal/urinary system

abnormal renal/urinary system physiology ( J:94376 )

• mice have to be euthanized by 8 months due to urinary dysfunction and bacterial bladder infection

immune system

increased susceptibility to bacterial infection induced morbidity/mortality ( J:94376 )

• mice have to be euthanized by 8 months due to urinary dysfunction and bacterial bladder infection