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Glycogenosis Type II (Pompe Disease)
  • Description
  • Signalment
  • Clinical Features
  • Neurolocalization
  • Genetics
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Glycogenosis Type II causes a deficiency in the enzyme acid a-glucosidase which causes defects in the catabolism of glycogen resulting in its accumulation in neuron lysosomes and cytoplasm.
Age of Onset: 4-12 months of age
Sex Predisposition: Any sex of animal can be affected
Clinical Course:
​
Clinical signs worsen over time
Clinical Signs:
Behavioral/Mental Awareness
“Apathetic habitus” 

Posture and Appearance
Loss of condition

Movement
Muscle Weakness

Special functions (e.g. respiration; urination)
Esophageal dilation induced vomiting
Clinical heart disease
Myocardial hypertrophy

Other
Dysphonia
Diffuse CNS
Autosomal recessive inheritance - Nonsense Mutation in the acid a-Glucosidase (GAA gene mutation)

For breed specific genetic testing, follow the links below:

https://embarkvet.com/products/dog-health/health-conditions/glycogen-storage-disease-type-ii-pompes-disease-gsd-ii/​
https://www.genomia.cz/en/test/gsdii/​
To read more about this disease click below:
References
Picture
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