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Additionally, the Pcca -/- (A138T) mouse model, representing one of the gene mutations observed in human patients with a milder phenotype 38,49,56,57 , may not fully recapitulate the diverse conditions (various genotypes, disease severity, and metabolic crises) encountered in human PA patients
The third stage is the chronic phase of striatal atrophy, which can be characterized by persistent T2 hyperintensity in the nucleus accumbens and caudate nucleus on MRI [72]
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