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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...

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Detalles Bibliográficos
Autores principales: Guhaniyogi, Jayita, Sohar, Istvan, Das, Kalyan, Stock, Ann M., Lobel, Peter
Formato: Texto
Lenguaje:English
Publicado: American Society for Biochemistry and Molecular Biology 2009
Materias:
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

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