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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

Cranial Nerves
Dysphonia

Special Functions
(e.g. respiration; urination)

Esophageal dilation induced vomiting

Other
Clinical heart disease
Myocardial hypertrophy​
Brain and spinal cord
Autosomal recessive inheritance of nonsense mutation in the acid a-glucosidase

For breed-specific genetic testing, click the link(s) below:

https://www.genomia.cz/en/test/gsdii/
To read more about this disease click below:
References
Picture
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