In a model of Batten disease, palmitoyl protein thioesterase-1 deficiency is associated with brown adipose tissue and thermoregulation abnormalities.

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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Main Authors: Alfia Khaibullina, Nicholas Kenyon, Virginia Guptill, Martha M Quezado, Li Wang, Deloris Koziol, Robert Wesley, Pablo R Moya, Zhongjian Zhang, Arjun Saha, Anil B Mukherjee, Zenaide M N Quezado
Format: Article
Language:English
Published: Public Library of Science (PLoS) 2012-01-01
Series:PLoS ONE
Online Access:http://europepmc.org/articles/PMC3490854?pdf=render

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http://europepmc.org/articles/PMC3490854?pdf=render

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