{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,6,5]],"date-time":"2026-06-05T04:53:30Z","timestamp":1780635210466,"version":"3.54.1"},"reference-count":41,"publisher":"Public Library of Science (PLoS)","issue":"11","license":[{"start":{"date-parts":[[2012,11,6]],"date-time":"2012-11-06T00:00:00Z","timestamp":1352160000000},"content-version":"unspecified","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/publicdomain\/zero\/1.0\/"}],"content-domain":{"domain":["www.plosone.org"],"crossmark-restriction":false},"short-container-title":["PLoS ONE"],"DOI":"10.1371\/journal.pone.0048733","type":"journal-article","created":{"date-parts":[[2012,11,6]],"date-time":"2012-11-06T22:00:02Z","timestamp":1352239202000},"page":"e48733","update-policy":"https:\/\/doi.org\/10.1371\/journal.pone.corrections_policy","source":"Crossref","is-referenced-by-count":15,"title":["In a Model of Batten Disease, Palmitoyl Protein Thioesterase-1 Deficiency Is Associated with Brown Adipose Tissue and Thermoregulation Abnormalities"],"prefix":"10.1371","volume":"7","author":[{"given":"Alfia","family":"Khaibullina","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Nicholas","family":"Kenyon","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Virginia","family":"Guptill","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Martha M.","family":"Quezado","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Li","family":"Wang","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Deloris","family":"Koziol","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Robert","family":"Wesley","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Pablo R.","family":"Moya","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Zhongjian","family":"Zhang","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Arjun","family":"Saha","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Anil B.","family":"Mukherjee","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Zenaide M.N.","family":"Quezado","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"340","published-online":{"date-parts":[[2012,11,6]]},"reference":[{"key":"ref1","doi-asserted-by":"crossref","first-page":"850","DOI":"10.1016\/j.bbadis.2006.06.010","article-title":"The neuronal ceroid-lipofuscinoses: from past to present","volume":"1762","author":"M Haltia","year":"2006","journal-title":"Biochim Biophys Acta"},{"key":"ref2","doi-asserted-by":"crossref","first-page":"S35","DOI":"10.1007\/s100720070038","article-title":"Neuronal ceroid lipofuscinoses in childhood","volume":"21","author":"P Santavuori","year":"2000","journal-title":"Neurol Sci"},{"key":"ref3","doi-asserted-by":"crossref","first-page":"443","DOI":"10.1016\/S1567-424X(09)70193-X","article-title":"Neuronal ceroid lipofuscinoses in childhood","volume":"53","author":"P Santavuori","year":"2000","journal-title":"Suppl Clin Neurophysiol"},{"key":"ref4","doi-asserted-by":"crossref","first-page":"61","DOI":"10.1111\/j.1750-3639.2004.tb00499.x","article-title":"Current state of clinical and morphological features in human NCL","volume":"14","author":"HH Goebel","year":"2004","journal-title":"Brain Pathol"},{"key":"ref5","doi-asserted-by":"crossref","first-page":"584","DOI":"10.1038\/376584a0","article-title":"Mutations in the palmitoyl protein thioesterase gene causing infantile neuronal ceroid lipofuscinosis","volume":"376","author":"J Vesa","year":"1995","journal-title":"Nature"},{"key":"ref6","doi-asserted-by":"crossref","first-page":"23212","DOI":"10.1016\/S0021-9258(17)31641-1","article-title":"Molecular cloning and expression of palmitoyl-protein thioesterase","volume":"269","author":"LA Camp","year":"1994","journal-title":"J Biol Chem"},{"key":"ref7","unstructured":"Hofmann SL, Peltonen L (2001) The neuronal ceroid lipofuscinosis. In: Scriver CR, Sly WS, Childs B, editors. The Metabolic & Molecular Bases of Inherited Disease. New York: McGraw-Hill. 3877\u20133894."},{"key":"ref8","doi-asserted-by":"crossref","first-page":"1580","DOI":"10.1093\/hmg\/ddl078","article-title":"Palmitoyl-protein thioesterase-1 deficiency leads to the activation of caspase-9 and contributes to rapid neurodegeneration in INCL","volume":"15","author":"SJ Kim","year":"2006","journal-title":"Hum Mol Genet"},{"key":"ref9","doi-asserted-by":"crossref","first-page":"1826","DOI":"10.1093\/hmg\/ddl105","article-title":"Endoplasmic reticulum stress-induced caspase-4 activation mediates apoptosis and neurodegeneration in INCL","volume":"15","author":"SJ Kim","year":"2006","journal-title":"Hum Mol Genet"},{"key":"ref10","doi-asserted-by":"crossref","first-page":"469","DOI":"10.1093\/hmg\/ddm324","article-title":"ER and oxidative stresses are common mediators of apoptosis in both neurodegenerative and non-neurodegenerative lysosomal storage disorders and are alleviated by chemical chaperones","volume":"17","author":"H Wei","year":"2008","journal-title":"Hum Mol Genet"},{"key":"ref11","doi-asserted-by":"crossref","first-page":"837","DOI":"10.1093\/hmg\/ddm029","article-title":"Production of lysophosphatidylcholine by cPLA2 in the brain of mice lacking PPT1 is a signal for phagocyte infiltration","volume":"16","author":"Z Zhang","year":"2007","journal-title":"Hum Mol Genet"},{"key":"ref12","doi-asserted-by":"crossref","first-page":"3075","DOI":"10.1172\/JCI33482","article-title":"Palmitoyl protein thioesterase-1 deficiency impairs synaptic vesicle recycling at nerve terminals, contributing to neuropathology in humans and mice","volume":"118","author":"SJ Kim","year":"2008","journal-title":"J Clin Invest"},{"key":"ref13","doi-asserted-by":"crossref","first-page":"1111","DOI":"10.1093\/hmg\/ddq555","article-title":"Disruption of adaptive energy metabolism and elevated ribosomal p-S6K1 levels contribute to INCL pathogenesis: partial rescue by resveratrol","volume":"20","author":"H Wei","year":"2011","journal-title":"Hum Mol Genet"},{"key":"ref14","doi-asserted-by":"crossref","first-page":"349","DOI":"10.1016\/j.cmet.2006.10.004","article-title":"Thermoregulatory and metabolic defects in Huntington\u2019s disease transgenic mice implicate PGC-1alpha in Huntington\u2019s disease neurodegeneration","volume":"4","author":"P Weydt","year":"2006","journal-title":"Cell Metab"},{"key":"ref15","doi-asserted-by":"crossref","first-page":"119","DOI":"10.1038\/ncb2012","article-title":"PINK1\/Parkin-mediated mitophagy is dependent on VDAC1 and p62\/SQSTM1","volume":"12","author":"S Geisler","year":"2010","journal-title":"Nat Cell Biol"},{"key":"ref16","doi-asserted-by":"crossref","first-page":"329","DOI":"10.1056\/NEJMra0909142","article-title":"Alzheimer\u2019s disease","volume":"362","author":"HW Querfurth","year":"2010","journal-title":"N Engl J Med"},{"key":"ref17","doi-asserted-by":"crossref","first-page":"372","DOI":"10.1213\/ane.0b013e3181aa6e95","article-title":"Children with infantile neuronal ceroid lipofuscinosis have an increased risk of hypothermia and bradycardia during anesthesia","volume":"109","author":"N Miao","year":"2009","journal-title":"Anesth Analg"},{"key":"ref18","doi-asserted-by":"crossref","first-page":"13566","DOI":"10.1073\/pnas.251485198","article-title":"Disruption of PPT1 or PPT2 causes neuronal ceroid lipofuscinosis in knockout mice","volume":"98","author":"P Gupta","year":"2001","journal-title":"Proc Natl Acad Sci U S A"},{"key":"ref19","doi-asserted-by":"crossref","first-page":"346","DOI":"10.1016\/j.nbd.2004.02.010","article-title":"Regional and cellular neuropathology in the palmitoyl protein thioesterase-1 null mutant mouse model of infantile neuronal ceroid lipofuscinosis","volume":"16","author":"E Bible","year":"2004","journal-title":"Neurobiol Dis"},{"key":"ref20","doi-asserted-by":"crossref","first-page":"145","DOI":"10.1152\/advan.00052.2006","article-title":"PGC-1alpha: a key regulator of energy metabolism","volume":"30","author":"H Liang","year":"2006","journal-title":"Adv Physiol Educ"},{"key":"ref21","doi-asserted-by":"crossref","first-page":"242","DOI":"10.1242\/jeb.050989","article-title":"Nonshivering thermogenesis and its adequate measurement in metabolic studies","volume":"214","author":"B Cannon","year":"2011","journal-title":"J Exp Biol"},{"key":"ref22","doi-asserted-by":"crossref","first-page":"277","DOI":"10.1152\/physrev.00015.2003","article-title":"Brown adipose tissue: function and physiological significance","volume":"84","author":"B Cannon","year":"2004","journal-title":"Physiol Rev"},{"key":"ref23","doi-asserted-by":"crossref","first-page":"337","DOI":"10.1093\/hmg\/ddi451","article-title":"Palmitoyl-protein thioesterase-1 deficiency mediates the activation of the unfolded protein response and neuronal apoptosis in INCL","volume":"15","author":"Z Zhang","year":"2006","journal-title":"Hum Mol Genet"},{"key":"ref24","unstructured":"Franklin KBJ, Paxinos G (2008) The mouse brain in stereotaxic coordinates. New York: Elsevier Inc. 360 p."},{"key":"ref25","doi-asserted-by":"crossref","first-page":"e36","DOI":"10.1093\/nar\/30.9.e36","article-title":"Relative expression software tool (REST) for group-wise comparison and statistical analysis of relative expression results in real-time PCR","volume":"30","author":"MW Pfaffl","year":"2002","journal-title":"Nucleic Acids Res"},{"key":"ref26","doi-asserted-by":"crossref","first-page":"82","DOI":"10.1016\/S0005-2728(00)00247-4","article-title":"UCP1: the only protein able to mediate adaptive non-shivering thermogenesis and metabolic inefficiency","volume":"1504","author":"J Nedergaard","year":"2001","journal-title":"Biochim Biophys Acta"},{"key":"ref27","doi-asserted-by":"crossref","first-page":"773","DOI":"10.1113\/expphysiol.2007.041848","article-title":"Central control of thermogenesis in mammals","volume":"93","author":"SF Morrison","year":"2008","journal-title":"Exp Physiol"},{"key":"ref28","doi-asserted-by":"crossref","first-page":"R127","DOI":"10.1152\/ajpregu.00427.2006","article-title":"Central efferent pathways mediating skin cooling-evoked sympathetic thermogenesis in brown adipose tissue","volume":"292","author":"K Nakamura","year":"2007","journal-title":"Am J Physiol Regul Integr Comp Physiol"},{"key":"ref29","doi-asserted-by":"crossref","first-page":"62","DOI":"10.1038\/nn2027","article-title":"A thermosensory pathway that controls body temperature","volume":"11","author":"K Nakamura","year":"2008","journal-title":"Nat Neurosci"},{"key":"ref30","doi-asserted-by":"crossref","first-page":"2611","DOI":"10.1113\/jphysiol.2008.152686","article-title":"Preoptic mechanism for cold-defensive responses to skin cooling","volume":"586","author":"K Nakamura","year":"2008","journal-title":"J Physiol"},{"key":"ref31","doi-asserted-by":"crossref","first-page":"S36","DOI":"10.1038\/ijo.2010.182","article-title":"Sympathetic and sensory innervation of brown adipose tissue","volume":"34","author":"TJ Bartness","year":"2010","journal-title":"Int J Obes (Lond)"},{"key":"ref32","doi-asserted-by":"crossref","first-page":"R776","DOI":"10.1152\/ajpregu.00614.2009","article-title":"Endogenous activation of spinal 5-hydroxytryptamine (5-HT) receptors contributes to the thermoregulatory activation of brown adipose tissue","volume":"298","author":"CJ Madden","year":"2010","journal-title":"Am J Physiol Regul Integr Comp Physiol"},{"key":"ref33","doi-asserted-by":"crossref","first-page":"5247","DOI":"10.1113\/jphysiol.2011.214445","article-title":"Postnatal loss of brainstem serotonin neurones compromises the ability of neonatal rats to survive episodic severe hypoxia","volume":"589","author":"KJ Cummings","year":"2011","journal-title":"J Physiol"},{"key":"ref34","doi-asserted-by":"crossref","first-page":"25073","DOI":"10.1074\/jbc.M000547200","article-title":"Thermogenic responses in brown fat cells are fully UCP1-dependent. UCP2 or UCP3 do not substitute for UCP1 in adrenergically or fatty scid-induced thermogenesis","volume":"275","author":"A Matthias","year":"2000","journal-title":"J Biol Chem"},{"key":"ref35","doi-asserted-by":"crossref","first-page":"38236","DOI":"10.1074\/jbc.M402375200","article-title":"Native UCP1 displays simple competitive kinetics between the regulators purine nucleotides and fatty acids","volume":"279","author":"IG Shabalina","year":"2004","journal-title":"J Biol Chem"},{"key":"ref36","doi-asserted-by":"crossref","first-page":"238","DOI":"10.1016\/j.cmet.2011.02.009","article-title":"New powers of brown fat: fighting the metabolic syndrome","volume":"13","author":"J Nedergaard","year":"2011","journal-title":"Cell Metab"},{"key":"ref37","doi-asserted-by":"crossref","first-page":"312","DOI":"10.1083\/jcb.41.1.312","article-title":"Ultrastructural and biochemical changes in brown fat in cold-exposed rats","volume":"41","author":"JF Thomsom","year":"1969","journal-title":"J Cell Biol"},{"key":"ref38","doi-asserted-by":"crossref","first-page":"78","DOI":"10.1016\/j.lfs.2011.04.014","article-title":"Effects of burn injury, cold stress and cutaneous wound injury on the morphology and energy metabolism of murine brown adipose tissue (BAT) in vivo","volume":"89","author":"EA Carter","year":"2011","journal-title":"Life Sci"},{"key":"ref39","doi-asserted-by":"crossref","first-page":"200","DOI":"10.1038\/nm.2297","article-title":"Brown adipose tissue activity controls triglyceride clearance","volume":"17","author":"A Bartelt","year":"2011","journal-title":"Nat Med"},{"key":"ref40","doi-asserted-by":"crossref","first-page":"52","DOI":"10.1016\/j.nbd.2007.06.012","article-title":"Palmitoyl protein thioesterase 1 (Ppt1)-deficient mouse neurons show alterations in cholesterol metabolism and calcium homeostasis prior to synaptic dysfunction","volume":"28","author":"L Ahtiainen","year":"2007","journal-title":"Neurobiol Dis"},{"key":"ref41","doi-asserted-by":"crossref","first-page":"35765","DOI":"10.1074\/jbc.M705124200","article-title":"Lysosomal dysfunction results in altered energy balance","volume":"282","author":"JC Woloszynek","year":"2007","journal-title":"J Biol Chem"}],"container-title":["PLoS ONE"],"original-title":[],"language":"en","link":[{"URL":"http:\/\/dx.plos.org\/10.1371\/journal.pone.0048733","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2022,1,30]],"date-time":"2022-01-30T23:29:27Z","timestamp":1643585367000},"score":1,"resource":{"primary":{"URL":"https:\/\/dx.plos.org\/10.1371\/journal.pone.0048733"}},"subtitle":[],"editor":[{"given":"Udai","family":"Pandey","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"editor"}]}],"short-title":[],"issued":{"date-parts":[[2012,11,6]]},"references-count":41,"journal-issue":{"issue":"11","published-online":{"date-parts":[[2012,11,6]]}},"URL":"https:\/\/doi.org\/10.1371\/journal.pone.0048733","relation":{},"ISSN":["1932-6203"],"issn-type":[{"value":"1932-6203","type":"electronic"}],"subject":[],"published":{"date-parts":[[2012,11,6]]}}}