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Amyotrophic Lateral Sclerosis 

TDP 43 -/-

 Phenotype

  • Dysphagia

  • Respiratory Insufficiency 

  • Constricted GI

  • Aggression

  • Episodic Irritability with darting

  • Late Stage Social Isolation

  • Reduced distance travelled

  • Reduced time in motion

Histology

  • Loss of motor neurons in the spinal ventral horns

  • Loss of brainstem motor nuclei

  • Neuronal loss in motor cortex

  • Demyelination

  • Spongiosis

  • Astrogliosis

ALS TDP43

Biomarkers

  •  FUS

  • SPG11

  •  ZNF512B

  • SS18L1

  • EWSR1

  • SOD1

  • C9ORF72

  • CRP

  • pNfH

  •  CHIT

  •  IGF1

​

SOD-1 -/-

 Phenotype

  • Dysphagia

  • Respiratory Insufficiency 

  • Aggression

  • Episodic Irritability with darting

  • Late Stage Social Isolation

  • Adipose Tissue Loss

  • Reduced Spleen Size

  • Sparse Leukocyte count

  • Reduced distance travelled

  • Reduced time in motion

Histology

  • Loss of motor neurons in the spinal ventral horns

  • Loss of brainstem motor nuclei

  • Neuronal loss in motor cortex

  • Demyelination

  • Spongiosis

  • Astrogliosis

ALS SOD1

Biomarkers

  • Insulin Levels

  • FUS

  • SPG11

  •  ZNF512B

  • SS18L1

  • EWSR1

  • SOD1

  • C9ORF72

  • CRP

  • pNfH

  •  CHIT

  •  IGF1

​

Huntington’s Disease

Htt K33Q

 Phenotype

  • Presence of Chorea/Involuntary Jerk Movements

  • Lack of Spatial Memory

  • Negligible Avoidance Learning and Memory

  • Presence of Repetitive Pattern Psychosis

  • Early Latency to fall in Swim Tunnel

  • Marked Wall Butt Aggression Behaviour

  • Reduced Distance Travelled

  • Reduced Time in Motion

Histology

  • Striatal Neurodegeneration

  • Striatal Activated Microglia

  • Gliosis

  • Shrinkage or Loss of Caudate Nuclei

  • Loss of Neurons in  Cerebral cortex, Thalamus, Brainstem Nuclei and Cerebellum.

Huntington's Disease

Biomarkers

  • mHTT

  • BDNF

  • Nefl

  • Elevated Urea in Brain

  • PDE10A

  • Neuropeptide Y

  • IL-8

  • MCP-1

  • B3GAT1, Actn2, ADGRG1, TUBB2A, MTCO1P12, Scl45a3, Wt1, Pcdh20

Tauopathy

Tau SER282X -/-

 Phenotype

  • Lack of Exploratory Behaviour for New Context

  • Inability to Avoidance Learning and Memory

  • Presence of Repetitive Pattern Psychosis

  • Mirror Biting Aggressive Behaviour

  • Early Latency to fall in Swim Tunnel

  • Episodic Gait Freezing

  • Reduced Distance Travelled

  • Reduced Time in Motion

  • Low Survival Rates

Histology

  • Histological Scoring for Neurodegeneration  Tufted Astrocytes

  • Cortical Ballooned Cells

  • Gliosis

Tauopathy

Biomarkers

  • Total Tau, pTau

  • Nfl

  • Aβ

  • APP

  • Asparagine endopeptidase

  • Tau Fragments

Parkinson's

PINK1 -/- with asyn transplant

 Phenotype

  • Episodic Tremor

  • Gait Freezing

  • Stiff Struggling Motor behaviour

  • Reduced  Distance Travelled

  • Reduced or Time in Motion

  • Balance and Directional Gait loss

  • Failure to Avoidance Learning and Memory

  • Motor Co-ordination failure in Swim Tunnel

  • Skin Slough

  • Reduced Sleep Bouts

  • Episodic anxiety Darts

Histology

  •  No. of Dopaminergic Neurons per Field

  • Astrogliosis

  • Activated Microglia

  • Neuronal loss and neurofilament shrinkage in regions of Amygdala, Hypothalamus, Substantia nigra and Medullary tegmentum 

Parkinson's

Biomarkers

  • Orexin

  • ApoA1

  • Glial fibrillary acidic protein 

  • NfL

  • pTau

  • ROS

  • Uric Acid

  • Dopamine Receptor D2

  • aSyn

  • Glutamate Ionotropic Receptor AMPA type subunit 2

  • pGSK3β

  • PARK7

  • hif1α, catalase, SOD3, atp1a2a, fech, pax2a, notch1a, Caspase3

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