In a Model of Batten Disease, Palmitoyl Protein Thioesterase-1 Deficiency Is Associated with Brown Adipose Tissue and Thermoregulation Abnormalities

Infantile neuronal ceroid lipofuscinosis (INCL) is a fatal neurodegenerative disorder caused by a deficiency of palmitoyl-protein thioesterase-1 (PPT1). We have previously shown that children with INCL have increased risk of hypothermia during anesthesia and that PPT1-deficiency in mice is associate...

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Detalles Bibliográficos
Autores principales: Khaibullina, Alfia, Kenyon, Nicholas, Guptill, Virginia, Quezado, Martha M., Wang, Li, Koziol, Deloris, Wesley, Robert, Moya, Pablo R., Zhang, Zhongjian, Saha, Arjun, Mukherjee, Anil B., Quezado, Zenaide M.N.
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Public Library of Science 2012
Materias:
Research Article
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3490854/
https://www.ncbi.nlm.nih.gov/pubmed/23139814
http://dx.doi.org/10.1371/journal.pone.0048733

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