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Literature summary for 3.1.2.22 extracted from

  • Macauley, S.L.; Wozniak, D.F.; Kielar, C.; Tan, Y.; Cooper, J.D.; Sands, M.S.
    Cerebellar pathology and motor deficits in the palmitoyl protein thioesterase 1-deficient mouse (2009), Exp. Neurol., 217, 124-135.
    View publication on PubMedView publication on EuropePMC
Search for more literature for 3.1.2.22

Organism

Organism UniProt Comment Textmining
Mus musculus
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Source Tissue

Source Tissue Comment Organism Textmining
cerebellum PPT1-/- cerebella appear smaller than aged matched wild-type littermates Mus musculus
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Synonyms

Synonyms Comment Organism
palmitoyl protein thioesterase 1
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 Mus musculus
PPT1
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 Mus musculus

General Information

General Information Comment Organism
malfunction PPT1-deficient mouse (PPT1-/-) mimics the clinical symptoms and underlying pathology of infantile neuronal ceroid lipofuscinosis: Purkinje cell loss beginning at 3 months, which correlates with changes in rotarod performance, an early stage reactive gliosis and a primary pathology in astrocytes, including changes in S100beta and GLAST expression, a late stage granule cell loss, microglial activation, and demyelination. Neuronal–glial interactions are the core pathology in the PPT1-/- cerebellum Mus musculus