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Identification of a Novel Acid Sphingomyelinase Activity Associated with Recombinant Human Acid Ceramidase

Acid ceramidase (AC) is a lysosomal enzyme required to hydrolyze ceramide to sphingosine by the removal of the fatty acid moiety. An inherited deficiency in this activity results in two disorders, Farber Lipogranulomatosis and spinal muscular atrophy with myoclonic epilepsy, leading to the accumulat...

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Detalles Bibliográficos
Autores principales: He, Xingxuan, Schuchman, Edward H.
Formato: Online Artículo Texto
Lenguaje:English
Publicado: MDPI 2023
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10669851/
https://www.ncbi.nlm.nih.gov/pubmed/38002305
http://dx.doi.org/10.3390/biom13111623