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Spinal Muscular Atrophy
Smn1 KO - Loss of Function

Spinal Muscular Atrophy

  • Genotype

    Smn1 KO

  • Mechanism

    Loss of Function

  • Phenotype

    Locomotor activity, Fatigue, Motor coordination, Touch evoke escape response, Muscle rigidity and weakness

  • Biomarker

    Dystrophin , Myosin heavy chain , Creatine kinase

  • Gene Expression Markers

    hb9, isl1, SMN1/2, synaptophysin, SNAP25, myod, myogenin

  • Physiology Changes

    Posture, Reflex responses 

  • Histology Grading Endpoint

    Loss and degeneration of spinal motor neurons, Reduced motor neuron size, Fibrosis, Axonal degeneration

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