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Crystal Structure and Autoactivation Pathway of the Precursor Form of Human Tripeptidyl-peptidase 1, the Enzyme Deficient in Late Infantile Ceroid Lipofuscinosis
Late infantile neuronal ceroid lipofuscinosis is a fatal childhood neurological disorder caused by a deficiency in the lysosomal protease tripeptidyl-peptidase 1 (TPP1). TPP1 represents the only known mammalian member of the S53 family of serine proteases, a group characterized by a subtilisin-like...
Autores principales: | , , , , |
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Formato: | Texto |
Lenguaje: | English |
Publicado: |
American Society for Biochemistry and Molecular Biology
2009
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Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC2635056/ https://www.ncbi.nlm.nih.gov/pubmed/19038967 http://dx.doi.org/10.1074/jbc.M806943200 |