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Global Brain Transcriptome Analysis of a Tpp1 Neuronal Ceroid Lipofuscinoses Mouse Model
In humans, homozygous mutations in the TPP1 gene results in loss of tripeptidyl peptidase 1 (TPP1) enzymatic activity, leading to late infantile neuronal ceroid lipofuscinoses disease. Using a mouse model that targets the Tpp1 gene and recapitulates the pathology and clinical features of the human d...
Autores principales: | Domowicz, Miriam S., Chan, Wen-Ching, Claudio-Vázquez, Patricia, Henry, Judith G., Ware, Christopher B., Andrade, Jorge, Dawson, Glyn, Schwartz, Nancy B. |
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Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
SAGE Publications
2019
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Materias: | |
Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6475859/ https://www.ncbi.nlm.nih.gov/pubmed/31003587 http://dx.doi.org/10.1177/1759091419843393 |
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