{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,31]],"date-time":"2026-10-31T06:52:15Z","timestamp":1793429535600,"version":"4.5.0"},"reference-count":69,"publisher":"Springer Science and Business Media LLC","issue":"1","license":[{"start":{"date-parts":[[2015,1,31]],"date-time":"2015-01-31T00:00:00Z","timestamp":1422662400000},"content-version":"unspecified","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by\/4.0"}],"content-domain":{"domain":["link.springer.com"],"crossmark-restriction":false},"short-container-title":["acta neuropathol commun"],"published-print":{"date-parts":[[2015,12]]},"DOI":"10.1186\/s40478-014-0182-y","type":"journal-article","created":{"date-parts":[[2015,1,30]],"date-time":"2015-01-30T14:43:26Z","timestamp":1422629006000},"update-policy":"https:\/\/doi.org\/10.1007\/springer_crossmark_policy","source":"Crossref","is-referenced-by-count":79,"title":["LAMP-2 deficiency leads to hippocampal dysfunction but normal clearance of neuronal substrates of chaperone-mediated autophagy in a mouse model for Danon disease"],"prefix":"10.1186","volume":"3","author":[{"given":"Michelle","family":"Rothaug","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Stijn","family":"Stroobants","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Michaela","family":"Schweizer","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Judith","family":"Peters","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Friederike","family":"Zunke","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Mirka","family":"Allerding","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Rudi","family":"D\u2019Hooge","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Paul","family":"Saftig","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Judith","family":"Blanz","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"297","published-online":{"date-parts":[[2015,1,31]]},"reference":[{"key":"182_CR1","doi-asserted-by":"crossref","unstructured":"Wilke S, Krausze J, Bussow K (2012) Crystal structure of the conserved domain of the DC lysosomal associated membrane protein: implications for the lysosomal glycocalyx. BMC Biol 10:62. doi:10.1186\/1741-7007-10-62","DOI":"10.1186\/1741-7007-10-62"},{"issue":"6","key":"182_CR2","doi-asserted-by":"publisher","first-page":"603","DOI":"10.1007\/BF02400979","volume":"80","author":"WF Neiss","year":"1984","unstructured":"Neiss WF (1984) A coat of glycoconjugates on the inner surface of the lysosomal membrane in the rat kidney. Histochemistry 80(6):603\u2013608","journal-title":"Histochemistry"},{"issue":"43","key":"182_CR3","doi-asserted-by":"publisher","first-page":"31039","DOI":"10.1074\/jbc.274.43.31039","volume":"274","author":"R Kundra","year":"1999","unstructured":"Kundra R, Kornfeld S (1999) Asparagine-linked oligosaccharides protect Lamp-1 and Lamp-2 from intracellular proteolysis. J Biol Chem 274(43):31039\u201331046","journal-title":"J Biol Chem"},{"issue":"6798","key":"182_CR4","doi-asserted-by":"publisher","first-page":"902","DOI":"10.1038\/35022595","volume":"406","author":"Y Tanaka","year":"2000","unstructured":"Tanaka Y, Guhde G, Suter A, Eskelinen EL, Hartmann D, Lullmann-Rauch R, Janssen PM, Blanz J, von Figura K, Saftig P (2000) Accumulation of autophagic vacuoles and cardiomyopathy in LAMP-2-deficient mice. Nature 406(6798):902\u2013906, doi:10.1038\/35022595","journal-title":"Nature"},{"issue":"5\u20136","key":"182_CR5","doi-asserted-by":"publisher","first-page":"495","DOI":"10.1016\/j.mam.2006.08.005","volume":"27","author":"EL Eskelinen","year":"2006","unstructured":"Eskelinen EL (2006) Roles of LAMP-1 and LAMP-2 in lysosome biogenesis and autophagy. Mol Aspects Med 27(5\u20136):495\u2013502, doi:10.1016\/j.mam.2006.08.005","journal-title":"Mol Aspects Med"},{"issue":"1","key":"182_CR6","doi-asserted-by":"publisher","first-page":"475","DOI":"10.4049\/jimmunol.180.1.475","volume":"180","author":"W Beertsen","year":"2008","unstructured":"Beertsen W, Willenborg M, Everts V, Zirogianni A, Podschun R, Schroder B, Eskelinen EL, Saftig P (2008) Impaired phagosomal maturation in neutrophils leads to periodontitis in lysosomal-associated membrane protein-2 knockout mice. J Immunol 180(1):475\u2013482","journal-title":"J Immunol"},{"issue":"9","key":"182_CR7","doi-asserted-by":"publisher","first-page":"2153","DOI":"10.1111\/j.1462-5822.2007.00946.x","volume":"9","author":"MG Binker","year":"2007","unstructured":"Binker MG, Cosen-Binker LI, Terebiznik MR, Mallo GV, McCaw SE, Eskelinen EL, Willenborg M, Brumell JH, Saftig P, Grinstein S, Gray-Owen SD (2007) Arrested maturation of Neisseria-containing phagosomes in the absence of the lysosome-associated membrane proteins, LAMP-1 and LAMP-2. Cell Microbiol 9(9):2153\u20132166, doi:10.1111\/j.1462-5822.2007.00946","journal-title":"Cell Microbiol"},{"issue":"2","key":"182_CR8","doi-asserted-by":"publisher","first-page":"313","DOI":"10.1038\/sj.emboj.7601511","volume":"26","author":"KK Huynh","year":"2007","unstructured":"Huynh KK, Eskelinen EL, Scott CC, Malevanets A, Saftig P, Grinstein S (2007) LAMP proteins are required for fusion of lysosomes with phagosomes. EMBO J 26(2):313\u2013324, doi:10.1038\/sj.emboj.7601511","journal-title":"EMBO J"},{"issue":"7","key":"182_CR9","doi-asserted-by":"publisher","first-page":"3132","DOI":"10.1091\/mbc.E04-02-0103","volume":"15","author":"EL Eskelinen","year":"2004","unstructured":"Eskelinen EL, Schmidt CK, Neu S, Willenborg M, Fuertes G, Salvador N, Tanaka Y, Lullmann-Rauch R, Hartmann D, Heeren J, von Figura K, Knecht E, Saftig P (2004) Disturbed cholesterol traffic but normal proteolytic function in LAMP-1\/LAMP-2 double-deficient fibroblasts. Mol Biol Cell 15(7):3132\u20133145, doi:10.1091\/mbc.E04-02-0103E04-02-0103","journal-title":"Mol Biol Cell"},{"issue":"2","key":"182_CR10","doi-asserted-by":"publisher","first-page":"280","DOI":"10.1111\/j.1582-4934.2009.00973.x","volume":"15","author":"A Schneede","year":"2011","unstructured":"Schneede A, Schmidt CK, Holtta-Vuori M, Heeren J, Willenborg M, Blanz J, Domanskyy M, Breiden B, Brodesser S, Landgrebe J, Sandhoff K, Ikonen E, Saftig P, Eskelinen EL (2011) Role for LAMP-2 in endosomal cholesterol transport. J Cell Mol Med 15(2):280\u2013295, doi:10.1111\/j.1582-4934.2009","journal-title":"J Cell Mol Med"},{"issue":"2","key":"182_CR11","doi-asserted-by":"publisher","first-page":"757","DOI":"10.1006\/bbrc.1995.2528","volume":"215","author":"DS Konecki","year":"1995","unstructured":"Konecki DS, Foetisch K, Zimmer KP, Schlotter M, Lichter-Konecki U (1995) An alternatively spliced form of the human lysosome-associated membrane protein-2 gene is expressed in a tissue-specific manner. Biochem Biophys Res Commun 215(2):757\u2013767","journal-title":"Biochem Biophys Res Commun"},{"issue":"Pt 5","key":"182_CR12","doi-asserted-by":"crossref","first-page":"2093","DOI":"10.1242\/jcs.108.5.2093","volume":"108","author":"CL Hatem","year":"1995","unstructured":"Hatem CL, Gough NR, Fambrough DM (1995) Multiple mRNAs encode the avian lysosomal membrane protein LAMP-2, resulting in alternative transmembrane and cytoplasmic domains. J Cell Sci 108(Pt 5):2093\u20132100","journal-title":"J Cell Sci"},{"issue":"1","key":"182_CR13","doi-asserted-by":"publisher","first-page":"43","DOI":"10.1046\/j.1432-0436.1999.6510043.x","volume":"65","author":"U Lichter-Konecki","year":"1999","unstructured":"Lichter-Konecki U, Moter SE, Krawisz BR, Schlotter M, Hipke C, Konecki DS (1999) Expression patterns of murine lysosome-associated membrane protein 2 (Lamp-2) transcripts during morphogenesis. Differentiation 65(1):43\u201358, doi:10.1046\/j.1432-0436.1999.6510043","journal-title":"Differentiation"},{"issue":"5274","key":"182_CR14","doi-asserted-by":"publisher","first-page":"501","DOI":"10.1126\/science.273.5274.501","volume":"273","author":"AM Cuervo","year":"1996","unstructured":"Cuervo AM, Dice JF (1996) A receptor for the selective uptake and degradation of proteins by lysosomes. Science 273(5274):501\u2013503","journal-title":"Science"},{"issue":"Pt 24","key":"182_CR15","doi-asserted-by":"crossref","first-page":"4441","DOI":"10.1242\/jcs.113.24.4441","volume":"113","author":"AM Cuervo","year":"2000","unstructured":"Cuervo AM, Dice JF (2000) Unique properties of lamp2a compared to other lamp2 isoforms. J Cell Sci 113(Pt 24):4441\u20134450","journal-title":"J Cell Sci"},{"issue":"3","key":"182_CR16","doi-asserted-by":"publisher","first-page":"142","DOI":"10.1016\/j.tem.2009.10.003","volume":"21","author":"AM Cuervo","year":"2010","unstructured":"Cuervo AM (2010) Chaperone-mediated autophagy: selectivity pays off. Trends Endocrinol Metab 21(3):142\u2013150, doi:10.003S1043-2760(09)00162-3","journal-title":"Trends Endocrinol Metab"},{"issue":"5","key":"182_CR17","doi-asserted-by":"publisher","first-page":"571","DOI":"10.1016\/j.immuni.2005.03.009","volume":"22","author":"D Zhou","year":"2005","unstructured":"Zhou D, Li P, Lin Y, Lott JM, Hislop AD, Canaday DH, Brutkiewicz RR, Blum JS (2005) Lamp-2a facilitates MHC class II presentation of cytoplasmic antigens. Immunity 22(5):571\u2013581, doi:10.1016\/j.immuni.2005.03.009","journal-title":"Immunity"},{"key":"182_CR18","doi-asserted-by":"crossref","unstructured":"Valdor R, Mocholi E, Botbol Y, Guerrero-Ros I, Chandra D, Koga H, Gravekamp C, Cuervo AM, Macian F (2014) Chaperone-mediated autophagy regulates T cell responses through targeted degradation of negative regulators of T cell activation. Nat Immunol. doi:10.1038\/ni.3003","DOI":"10.1038\/ni.3003"},{"issue":"5688","key":"182_CR19","doi-asserted-by":"publisher","first-page":"1292","DOI":"10.1126\/science.1101738","volume":"305","author":"AM Cuervo","year":"2004","unstructured":"Cuervo AM, Stefanis L, Fredenburg R, Lansbury PT, Sulzer D (2004) Impaired degradation of mutant alpha-synuclein by chaperone-mediated autophagy. Science 305(5688):1292\u20131295, doi:10.1126\/science.1101738305\/5688\/1292","journal-title":"Science"},{"issue":"Pt 7","key":"182_CR20","doi-asserted-by":"publisher","first-page":"2130","DOI":"10.1093\/brain\/awt131","volume":"136","author":"M Xilouri","year":"2013","unstructured":"Xilouri M, Brekk OR, Landeck N, Pitychoutis PM, Papasilekas T, Papadopoulou-Daifoti Z, Kirik D, Stefanis L (2013) Boosting chaperone-mediated autophagy in vivo mitigates alpha-synuclein-induced neurodegeneration. Brain 136(Pt 7):2130\u20132146, doi:10.1093\/brain\/awt131 awt131","journal-title":"Brain"},{"issue":"1","key":"182_CR21","doi-asserted-by":"publisher","first-page":"29","DOI":"10.1016\/j.nbd.2010.07.006","volume":"43","author":"H Koga","year":"2011","unstructured":"Koga H, Cuervo AM (2011) Chaperone-mediated autophagy dysfunction in the pathogenesis of neurodegeneration. Neurobiol Dis 43(1):29\u201337, doi:10.1016\/j.nbd.2010.07.006","journal-title":"Neurobiol Dis"},{"issue":"10","key":"182_CR22","doi-asserted-by":"publisher","first-page":"e46834","DOI":"10.1371\/journal.pone.0046834","volume":"7","author":"L Qi","year":"2012","unstructured":"Qi L, Zhang XD, Wu JC, Lin F, Wang J, DiFiglia M, Qin ZH (2012) The role of chaperone-mediated autophagy in huntingtin degradation. PLoS One 7(10):e46834, doi:10.1371\/journal.pone.0046834","journal-title":"PLoS One"},{"issue":"12","key":"182_CR23","doi-asserted-by":"publisher","first-page":"531","DOI":"10.1016\/S0962-8924(00)01853-5","volume":"10","author":"AJ Tobin","year":"2000","unstructured":"Tobin AJ, Signer ER (2000) Huntington\u2019s disease: the challenge for cell biologists. Trends Cell Biol 10(12):531\u2013536","journal-title":"Trends Cell Biol"},{"issue":"12","key":"182_CR24","doi-asserted-by":"publisher","first-page":"3338","DOI":"10.1523\/JNEUROSCI.0285-07.2007","volume":"27","author":"M Periquet","year":"2007","unstructured":"Periquet M, Fulga T, Myllykangas L, Schlossmacher MG, Feany MB (2007) Aggregated alpha-synuclein mediates dopaminergic neurotoxicity in vivo. J Neurosci 27(12):3338\u20133346, doi:10.1523\/JNEUROSCI.0285-07.2007","journal-title":"J Neurosci"},{"issue":"1","key":"182_CR25","doi-asserted-by":"publisher","first-page":"51","DOI":"10.1212\/WNL.31.1.51","volume":"31","author":"MJ Danon","year":"1981","unstructured":"Danon MJ, Oh SJ, DiMauro S, Manaligod JR, Eastwood A, Naidu S, Schliselfeld LH (1981) Lysosomal glycogen storage disease with normal acid maltase. Neurology 31(1):51\u201357","journal-title":"Neurology"},{"issue":"6798","key":"182_CR26","doi-asserted-by":"publisher","first-page":"906","DOI":"10.1038\/35022604","volume":"406","author":"I Nishino","year":"2000","unstructured":"Nishino I, Fu J, Tanji K, Yamada T, Shimojo S, Koori T, Mora M, Riggs JE, Oh SJ, Koga Y, Sue CM, Yamamoto A, Murakami N, Shanske S, Byrne E, Bonilla E, Nonaka I, DiMauro S, Hirano M (2000) Primary LAMP-2 deficiency causes X-linked vacuolar cardiomyopathy and myopathy (Danon disease). Nature 406(6798):906\u2013910, doi:10.1038\/35022604","journal-title":"Nature"},{"issue":"7","key":"182_CR27","doi-asserted-by":"publisher","first-page":"407","DOI":"10.1038\/jhg.2012.72","volume":"57","author":"Z Cheng","year":"2012","unstructured":"Cheng Z, Fang Q (2012) Danon disease: focusing on heart. J Hum Genet 57(7):407\u2013410, doi:10.1038\/jhg.2012.72","journal-title":"J Hum Genet"},{"issue":"4","key":"182_CR28","doi-asserted-by":"publisher","first-page":"281","DOI":"10.1007\/s00395-006-0591-6","volume":"101","author":"J Stypmann","year":"2006","unstructured":"Stypmann J, Janssen PM, Prestle J, Engelen MA, Kogler H, Lullmann-Rauch R, Eckardt L, von Figura K, Landgrebe J, Mleczko A, Saftig P (2006) LAMP-2 deficient mice show depressed cardiac contractile function without significant changes in calcium handling. Basic Res Cardiol 101(4):281\u2013291, doi:10.1007\/s00395-006-0591-6","journal-title":"Basic Res Cardiol"},{"issue":"3","key":"182_CR29","doi-asserted-by":"publisher","first-page":"459","DOI":"10.1007\/s00401-012-1075-4","volume":"125","author":"A Furuta","year":"2013","unstructured":"Furuta A, Wakabayashi K, Haratake J, Kikuchi H, Kabuta T, Mori F, Tokonami F, Katsumi Y, Tanioka F, Uchiyama Y, Nishino I, Wada K (2013) Lysosomal storage and advanced senescence in the brain of LAMP-2-deficient Danon disease. Acta Neuropathol 125(3):459\u2013461, doi:10.1007\/s00401-012-1075-4","journal-title":"Acta Neuropathol"},{"issue":"1","key":"182_CR30","doi-asserted-by":"publisher","first-page":"37","DOI":"10.1016\/S1471-4914(00)01868-2","volume":"7","author":"P Saftig","year":"2001","unstructured":"Saftig P, Tanaka Y, Lullmann-Rauch R, von Figura K (2001) Disease model: LAMP-2 enlightens Danon disease. Trends Mol Med 7(1):37\u201339","journal-title":"Trends Mol Med"},{"issue":"5","key":"182_CR31","doi-asserted-by":"publisher","first-page":"935","DOI":"10.1038\/nprot.2012.038","volume":"7","author":"CY Hsu","year":"2012","unstructured":"Hsu CY, Uludag H (2012) A simple and rapid nonviral approach to efficiently transfect primary tissue-derived cells using polyethylenimine. Nat Protoc 7(5):935\u2013945, doi:10.1038\/nprot.2012.038","journal-title":"Nat Protoc"},{"issue":"5","key":"182_CR32","doi-asserted-by":"publisher","first-page":"1316","DOI":"10.1016\/j.pnpbp.2011.03.023","volume":"35","author":"A Naert","year":"2011","unstructured":"Naert A, Callaerts-Vegh Z, Moechars D, Meert T, D'Hooge R (2011) Vglut2 haploinsufficiency enhances behavioral sensitivity to MK-801 and amphetamine in mice. Prog Neuropsychopharmacol Biol Psychiatry 35(5):1316\u20131321, doi:10.1016\/j.pnpbp.2011.03.023","journal-title":"Prog Neuropsychopharmacol Biol Psychiatry"},{"issue":"22","key":"182_CR33","doi-asserted-by":"publisher","first-page":"3437","DOI":"10.1093\/hmg\/ddn237","volume":"17","author":"J Blanz","year":"2008","unstructured":"Blanz J, Stroobants S, Lullmann-Rauch R, Morelle W, Ludemann M, D'Hooge R, Reuterwall H, Michalski JC, Fogh J, Andersson C, Saftig P (2008) Reversal of peripheral and central neural storage and ataxia after recombinant enzyme replacement therapy in alpha-mannosidosis mice. Hum Mol Genet 17(22):3437\u20133445, doi:10.1093\/hmg\/ddn237","journal-title":"Hum Mol Genet"},{"issue":"3","key":"182_CR34","doi-asserted-by":"publisher","first-page":"306","DOI":"10.1002\/jnr.20650","volume":"82","author":"K Dobrenis","year":"2005","unstructured":"Dobrenis K, Chang HY, Pina-Benabou MH, Woodroffe A, Lee SC, Rozental R, Spray DC, Scemes E (2005) Human and mouse microglia express connexin36, and functional gap junctions are formed between rodent microglia and neurons. J Neurosci Res 82(3):306\u2013315, doi:10.1002\/jnr.20650","journal-title":"J Neurosci Res"},{"issue":"9","key":"182_CR35","doi-asserted-by":"publisher","first-page":"e45","DOI":"10.1093\/nar\/29.9.e45","volume":"29","author":"MW Pfaffl","year":"2001","unstructured":"Pfaffl MW (2001) A new mathematical model for relative quantification in real-time RT-PCR. Nucleic Acids Res 29(9):e45","journal-title":"Nucleic Acids Res"},{"issue":"43","key":"182_CR36","doi-asserted-by":"publisher","first-page":"15573","DOI":"10.1073\/pnas.1405700111","volume":"111","author":"M Rothaug","year":"2014","unstructured":"Rothaug M, Zunke F, Mazzulli JR, Schweizer M, Altmeppen H, Lullmann-Rauch R, Kallemeijn WW, Gaspar P, Aerts JM, Glatzel M, Saftig P, Krainc D, Schwake M, Blanz J (2014) LIMP-2 expression is critical for beta-glucocerebrosidase activity and alpha-synuclein clearance. Proc Natl Acad Sci U S A 111(43):15573\u201315578, doi:10.1073\/pnas.1405700111","journal-title":"Proc Natl Acad Sci U S A"},{"issue":"3","key":"182_CR37","doi-asserted-by":"publisher","first-page":"403","DOI":"10.4161\/auto.23002","volume":"9","author":"Y Fujiwara","year":"2013","unstructured":"Fujiwara Y, Furuta A, Kikuchi H, Aizawa S, Hatanaka Y, Konya C, Uchida K, Yoshimura A, Tamai Y, Wada K, Kabuta T (2013) Discovery of a novel type of autophagy targeting RNA. Autophagy 9(3):403\u2013409, doi:10.4161\/auto.2300223002","journal-title":"Autophagy"},{"issue":"1","key":"182_CR38","doi-asserted-by":"publisher","first-page":"1","DOI":"10.1016\/0014-4800(87)90026-8","volume":"46","author":"F Henell","year":"1987","unstructured":"Henell F, Berkenstam A, Ahlberg J, Glaumann H (1987) Degradation of short- and long-lived proteins in perfused liver and in isolated autophagic vacuoles--lysosomes. Exp Mol Pathol 46(1):1\u201314","journal-title":"Exp Mol Pathol"},{"issue":"6","key":"182_CR39","doi-asserted-by":"publisher","first-page":"1149","DOI":"10.1016\/j.cell.2007.10.035","volume":"131","author":"M Komatsu","year":"2007","unstructured":"Komatsu M, Waguri S, Koike M, Sou YS, Ueno T, Hara T, Mizushima N, Iwata J, Ezaki J, Murata S, Hamazaki J, Nishito Y, Iemura S, Natsume T, Yanagawa T, Uwayama J, Warabi E, Yoshida H, Ishii T, Kobayashi A, Yamamoto M, Yue Z, Uchiyama Y, Kominami E, Tanaka K (2007) Homeostatic levels of p62 control cytoplasmic inclusion body formation in autophagy-deficient mice. Cell 131(6):1149\u20131163, doi:10.1016\/j.cell.2007.10.035","journal-title":"Cell"},{"issue":"33","key":"182_CR40","doi-asserted-by":"publisher","first-page":"24131","DOI":"10.1074\/jbc.M702824200","volume":"282","author":"S Pankiv","year":"2007","unstructured":"Pankiv S, Clausen TH, Lamark T, Brech A, Bruun JA, Outzen H, Overvatn A, Bjorkoy G, Johansen T (2007) p62\/SQSTM1 binds directly to Atg8\/LC3 to facilitate degradation of ubiquitinated protein aggregates by autophagy. J Biol Chem 282(33):24131\u201324145, doi:10.1074\/jbc.M702824200","journal-title":"J Biol Chem"},{"issue":"6","key":"182_CR41","doi-asserted-by":"publisher","first-page":"527","DOI":"10.1177\/030098589703400601","volume":"34","author":"RD Jolly","year":"1997","unstructured":"Jolly RD, Walkley SU (1997) Lysosomal storage diseases of animals: an essay in comparative pathology. Vet Pathol 34(6):527\u2013548","journal-title":"Vet Pathol"},{"issue":"23","key":"182_CR42","doi-asserted-by":"publisher","first-page":"15619","DOI":"10.1074\/jbc.M800723200","volume":"283","author":"L Wang","year":"2008","unstructured":"Wang L, Harris TE, Lawrence JC Jr (2008) Regulation of proline-rich Akt substrate of 40 kDa (PRAS40) function by mammalian target of rapamycin complex 1 (mTORC1)-mediated phosphorylation. J Biol Chem 283(23):15619\u201315627, doi:10.1074\/jbc.M800723200","journal-title":"J Biol Chem"},{"issue":"6","key":"182_CR43","doi-asserted-by":"publisher","first-page":"903","DOI":"10.1016\/j.molcel.2007.03.003","volume":"25","author":"Y Sancak","year":"2007","unstructured":"Sancak Y, Thoreen CC, Peterson TR, Lindquist RA, Kang SA, Spooner E, Carr SA, Sabatini DM (2007) PRAS40 is an insulin-regulated inhibitor of the mTORC1 protein kinase. Mol Cell 25(6):903\u2013915, doi:10.1016\/j.molcel.2007.03.003","journal-title":"Mol Cell"},{"issue":"5910","key":"182_CR44","doi-asserted-by":"publisher","first-page":"124","DOI":"10.1126\/science.1166088","volume":"323","author":"Q Yang","year":"2009","unstructured":"Yang Q, She H, Gearing M, Colla E, Lee M, Shacka JJ, Mao Z (2009) Regulation of neuronal survival factor MEF2D by chaperone-mediated autophagy. Science 323(5910):124\u2013127, doi:10.1126\/science.1166088 323\/5910\/124","journal-title":"Science"},{"issue":"1","key":"182_CR45","doi-asserted-by":"publisher","first-page":"92","DOI":"10.1038\/cr.2013.153","volume":"24","author":"AM Cuervo","year":"2014","unstructured":"Cuervo AM, Wong E (2014) Chaperone-mediated autophagy: roles in disease and aging. Cell Res 24(1):92\u2013104, doi:10.1038\/cr.2013.153cr2013153","journal-title":"Cell Res"},{"issue":"6","key":"182_CR46","doi-asserted-by":"publisher","first-page":"647","DOI":"10.1038\/ncb2718","volume":"15","author":"C Settembre","year":"2013","unstructured":"Settembre C, De Cegli R, Mansueto G, Saha PK, Vetrini F, Visvikis O, Huynh T, Carissimo A, Palmer D, Klisch TJ, Wollenberg AC, Di Bernardo D, Chan L, Irazoqui JE, Ballabio A (2013) TFEB controls cellular lipid metabolism through a starvation-induced autoregulatory loop. Nat Cell Biol 15(6):647\u2013658, doi:10.1038\/ncb2718","journal-title":"Nat Cell Biol"},{"issue":"35","key":"182_CR47","doi-asserted-by":"publisher","first-page":"23542","DOI":"10.1074\/jbc.M801992200","volume":"283","author":"T Vogiatzi","year":"2008","unstructured":"Vogiatzi T, Xilouri M, Vekrellis K, Stefanis L (2008) Wild type alpha-synuclein is degraded by chaperone-mediated autophagy and macroautophagy in neuronal cells. J Biol Chem 283(35):23542\u201323556, doi:10.1074\/jbc.M801992200","journal-title":"J Biol Chem"},{"issue":"5","key":"182_CR48","doi-asserted-by":"publisher","first-page":"1079","DOI":"10.1038\/sj.emboj.7600576","volume":"24","author":"D Kasper","year":"2005","unstructured":"Kasper D, Planells-Cases R, Fuhrmann JC, Scheel O, Zeitz O, Ruether K, Schmitt A, Poet M, Steinfeld R, Schweizer M, Kornak U, Jentsch TJ (2005) Loss of the chloride channel ClC-7 leads to lysosomal storage disease and neurodegeneration. EMBO J 24(5):1079\u20131091, doi:10.1038\/sj.emboj.7600576","journal-title":"EMBO J"},{"issue":"1","key":"182_CR49","doi-asserted-by":"publisher","first-page":"86","DOI":"10.1111\/j.1750-3639.2004.tb00502.x","volume":"14","author":"HM Mitchison","year":"2004","unstructured":"Mitchison HM, Lim MJ, Cooper JD (2004) Selectivity and types of cell death in the neuronal ceroid lipofuscinoses. Brain Pathol 14(1):86\u201396","journal-title":"Brain Pathol"},{"issue":"4","key":"182_CR50","doi-asserted-by":"publisher","first-page":"574","DOI":"10.1111\/j.1471-4159.2011.07550.x","volume":"120","author":"H Zhu","year":"2012","unstructured":"Zhu H, Yoshimoto T, Imajo-Ohmi S, Dazortsava M, Mathivanan A, Yamashima T (2012) Why are hippocampal CA1 neurons vulnerable but motor cortex neurons resistant to transient ischemia? J Neurochem 120(4):574\u2013585, doi:10.1111\/j.1471-4159.2011.07550.x","journal-title":"J Neurochem"},{"issue":"7280","key":"182_CR51","doi-asserted-by":"publisher","first-page":"549","DOI":"10.1038\/nature08710","volume":"463","author":"T Kirkegaard","year":"2010","unstructured":"Kirkegaard T, Roth AG, Petersen NH, Mahalka AK, Olsen OD, Moilanen I, Zylicz A, Knudsen J, Sandhoff K, Arenz C, Kinnunen PK, Nylandsted J, Jaattela M (2010) Hsp70 stabilizes lysosomes and reverts Niemann-Pick disease-associated lysosomal pathology. Nature 463(7280):549\u2013553, doi:10.1038\/nature08710","journal-title":"Nature"},{"issue":"4","key":"182_CR52","doi-asserted-by":"publisher","first-page":"425","DOI":"10.1084\/jem.20040531","volume":"200","author":"J Nylandsted","year":"2004","unstructured":"Nylandsted J, Gyrd-Hansen M, Danielewicz A, Fehrenbacher N, Lademann U, Hoyer-Hansen M, Weber E, Multhoff G, Rohde M, Jaattela M (2004) Heat shock protein 70 promotes cell survival by inhibiting lysosomal membrane permeabilization. The Journal of experimental medicine 200(4):425\u2013435, doi:10.1084\/jem.20040531 jem.20040531","journal-title":"The Journal of experimental medicine"},{"issue":"11","key":"182_CR53","doi-asserted-by":"publisher","first-page":"1643","DOI":"10.4161\/auto.21654","volume":"8","author":"T Saha","year":"2012","unstructured":"Saha T (2012) LAMP2A overexpression in breast tumors promotes cancer cell survival via chaperone-mediated autophagy. Autophagy 8(11):1643\u20131656, doi:10.4161\/auto.21654","journal-title":"Autophagy"},{"issue":"6","key":"182_CR54","doi-asserted-by":"publisher","first-page":"563","DOI":"10.1097\/GIM.0b013e31820ad795","volume":"13","author":"D Boucek","year":"2011","unstructured":"Boucek D, Jirikowic J, Taylor M (2011) Natural history of Danon disease. Genet Med 13(6):563\u2013568, doi:10.1097\/GIM.0b013e31820ad795","journal-title":"Genet Med"},{"issue":"10","key":"182_CR55","doi-asserted-by":"publisher","first-page":"830","DOI":"10.1007\/s10038-007-0184-8","volume":"52","author":"MR Taylor","year":"2007","unstructured":"Taylor MR, Ku L, Slavov D, Cavanaugh J, Boucek M, Zhu X, Graw S, Carniel E, Barnes C, Quan D, Prall R, Lovell MA, Mierau G, Ruegg P, Mandava N, Bristow MR, Towbin JA, Mestroni L (2007) Danon disease presenting with dilated cardiomyopathy and a complex phenotype. J Hum Genet 52(10):830\u2013835, doi:10.1007\/s10038-007-0184-8","journal-title":"J Hum Genet"},{"issue":"4","key":"182_CR56","doi-asserted-by":"publisher","first-page":"535","DOI":"10.1016\/j.molcel.2010.08.004","volume":"39","author":"U Bandyopadhyay","year":"2010","unstructured":"Bandyopadhyay U, Sridhar S, Kaushik S, Kiffin R, Cuervo AM (2010) Identification of regulators of chaperone-mediated autophagy. Mol Cell 39(4):535\u2013547, doi:10.1016\/j.molcel.2010.08.004S1097-2765(10)00614-3","journal-title":"Mol Cell"},{"issue":"1","key":"182_CR57","doi-asserted-by":"publisher","first-page":"95","DOI":"10.1023\/A:1021831621210","volume":"4","author":"JI Wolfsdorf","year":"2003","unstructured":"Wolfsdorf JI, Weinstein DA (2003) Glycogen storage diseases. Rev Endocr Metab Disord 4(1):95\u2013102, doi:5109165","journal-title":"Rev Endocr Metab Disord"},{"issue":"7","key":"182_CR58","doi-asserted-by":"publisher","first-page":"521","DOI":"10.1016\/j.nmd.2008.04.010","volume":"18","author":"MC Malicdan","year":"2008","unstructured":"Malicdan MC, Noguchi S, Nonaka I, Saftig P, Nishino I (2008) Lysosomal myopathies: an excessive build-up in autophagosomes is too much to handle. Neuromuscul Disord 18(7):521\u2013529, doi:10.1016\/j.nmd.2008.04.010 S0960-8966(08)00106-5","journal-title":"Neuromuscul Disord"},{"issue":"9","key":"182_CR59","doi-asserted-by":"publisher","first-page":"3355","DOI":"10.1091\/mbc.E02-02-0114","volume":"13","author":"EL Eskelinen","year":"2002","unstructured":"Eskelinen EL, Illert AL, Tanaka Y, Schwarzmann G, Blanz J, Von Figura K, Saftig P (2002) Role of LAMP-2 in lysosome biogenesis and autophagy. Mol Biol Cell 13(9):3355\u20133368, doi:10.1091\/mbc.E02-02-0114","journal-title":"Mol Biol Cell"},{"issue":"7","key":"182_CR60","doi-asserted-by":"publisher","first-page":"1521","DOI":"10.1093\/hmg\/ddr590","volume":"21","author":"O Criado","year":"2012","unstructured":"Criado O, Aguado C, Gayarre J, Duran-Trio L, Garcia-Cabrero AM, Vernia S, San Millan B, Heredia M, Roma-Mateo C, Mouron S, Juana-Lopez L, Dominguez M, Navarro C, Serratosa JM, Sanchez M, Sanz P, Bovolenta P, Knecht E, Rodriguez De Cordoba S (2012) Lafora bodies and neurological defects in malin-deficient mice correlate with impaired autophagy. Hum Mol Genet 21(7):1521\u20131533, doi:10.1093\/hmg\/ddr590ddr590","journal-title":"Hum Mol Genet"},{"issue":"1","key":"182_CR61","doi-asserted-by":"publisher","first-page":"175","DOI":"10.1093\/hmg\/ddr452","volume":"21","author":"R Puri","year":"2012","unstructured":"Puri R, Suzuki T, Yamakawa K, Ganesh S (2012) Dysfunctions in endosomal-lysosomal and autophagy pathways underlie neuropathology in a mouse model for Lafora disease. Hum Mol Genet 21(1):175\u2013184, doi:10.1093\/hmg\/ddr452","journal-title":"Hum Mol Genet"},{"issue":"11","key":"182_CR62","doi-asserted-by":"publisher","first-page":"667","DOI":"10.1002\/emmm.201100174","volume":"3","author":"J Valles-Ortega","year":"2011","unstructured":"Valles-Ortega J, Duran J, Garcia-Rocha M, Bosch C, Saez I, Pujadas L, Serafin A, Canas X, Soriano E, Delgado-Garcia JM, Gruart A, Guinovart JJ (2011) Neurodegeneration and functional impairments associated with glycogen synthase accumulation in a mouse model of Lafora disease. EMBO Mol Med 3(11):667\u2013681, doi:10.1002\/emmm.201100174","journal-title":"EMBO Mol Med"},{"key":"182_CR63","doi-asserted-by":"publisher","first-page":"177","DOI":"10.3389\/fnagi.2014.00177","volume":"6","author":"JA Lim","year":"2014","unstructured":"Lim JA, Li L, Raben N (2014) Pompe disease: from pathophysiology to therapy and back again. Front Aging Neurosci 6:177, doi:10.3389\/fnagi.2014.00177","journal-title":"Front Aging Neurosci"},{"issue":"7","key":"182_CR64","doi-asserted-by":"publisher","first-page":"1083","DOI":"10.1083\/jcb.200909067","volume":"187","author":"LM Thompson","year":"2009","unstructured":"Thompson LM, Aiken CT, Kaltenbach LS, Agrawal N, Illes K, Khoshnan A, Martinez-Vincente M, Arrasate M, O'Rourke JG, Khashwji H, Lukacsovich T, Zhu YZ, Lau AL, Massey A, Hayden MR, Zeitlin SO, Finkbeiner S, Green KN, LaFerla FM, Bates G, Huang L, Patterson PH, Lo DC, Cuervo AM, Marsh JL, Steffan JS (2009) IKK phosphorylates Huntingtin and targets it for degradation by the proteasome and lysosome. The Journal of cell biology 187(7):1083\u20131099, doi:10.1083\/jcb.200909067","journal-title":"The Journal of cell biology"},{"issue":"50","key":"182_CR65","doi-asserted-by":"publisher","first-page":"18492","DOI":"10.1523\/JNEUROSCI.3219-11.2011","volume":"31","author":"H Koga","year":"2011","unstructured":"Koga H, Martinez-Vicente M, Arias E, Kaushik S, Sulzer D, Cuervo AM (2011) Constitutive upregulation of chaperone-mediated autophagy in Huntington's disease. J Neurosci 31(50):18492\u201318505, doi:10.1523\/JNEUROSCI. 3219-11.2011","journal-title":"J Neurosci"},{"key":"182_CR66","doi-asserted-by":"crossref","unstructured":"Massey AC, Kaushik S, Sovak G, Kiffin R, Cuervo AM (2006) Consequences of the selective blockage of chaperone-mediated autophagy. Proc Natl Acad Sci U S A 103 (15):5805-5810. doi:0507436103","DOI":"10.1073\/pnas.0507436103"},{"key":"182_CR67","doi-asserted-by":"publisher","first-page":"20","DOI":"10.1186\/2047-9158-3-20","volume":"3","author":"G Wang","year":"2014","unstructured":"Wang G, Mao Z (2014) Chaperone-mediated autophagy: roles in neurodegeneration. Transl Neurodegener 3:20, doi:10.1186\/2047-9158-3-20","journal-title":"Transl Neurodegener"},{"issue":"9","key":"182_CR68","doi-asserted-by":"publisher","first-page":"959","DOI":"10.1038\/nm.1851","volume":"14","author":"C Zhang","year":"2008","unstructured":"Zhang C, Cuervo AM (2008) Restoration of chaperone-mediated autophagy in aging liver improves cellular maintenance and hepatic function. Nature medicine 14(9):959\u2013965, doi:10.1038\/nm.1851","journal-title":"Nature medicine"},{"issue":"5939","key":"182_CR69","doi-asserted-by":"crossref","first-page":"473","DOI":"10.1126\/science.1174447","volume":"325","author":"M Sardiello","year":"2009","unstructured":"Sardiello M, Palmieri M, di Ronza A, Medina DL, Valenza M, Gennarino VA, Di Malta C, Donaudy F, Embrione V, Polishchuk RS, Banfi S, Parenti G, Cattaneo E, Ballabio A (2009) A gene network regulating lysosomal biogenesis and function. Science 325(5939):473\u2013477, doi:10.1126\/science.1174447","journal-title":"Science"}],"container-title":["Acta Neuropathologica Communications"],"original-title":[],"language":"en","link":[{"URL":"http:\/\/link.springer.com\/content\/pdf\/10.1186\/s40478-014-0182-y.pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"http:\/\/link.springer.com\/article\/10.1186\/s40478-014-0182-y\/fulltext.html","content-type":"text\/html","content-version":"vor","intended-application":"text-mining"},{"URL":"http:\/\/link.springer.com\/content\/pdf\/10.1186\/s40478-014-0182-y","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"},{"URL":"https:\/\/link.springer.com\/content\/pdf\/10.1186\/s40478-014-0182-y.pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2021,9,2]],"date-time":"2021-09-02T07:49:18Z","timestamp":1630568958000},"score":1,"resource":{"primary":{"URL":"https:\/\/actaneurocomms.biomedcentral.com\/articles\/10.1186\/s40478-014-0182-y"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2015,1,31]]},"references-count":69,"journal-issue":{"issue":"1","published-print":{"date-parts":[[2015,12]]}},"alternative-id":["182"],"URL":"https:\/\/doi.org\/10.1186\/s40478-014-0182-y","relation":{},"ISSN":["2051-5960"],"issn-type":[{"value":"2051-5960","type":"electronic"}],"subject":[],"published":{"date-parts":[[2015,1,31]]},"assertion":[{"value":"21 November 2014","order":1,"name":"received","label":"Received","group":{"name":"ArticleHistory","label":"Article History"}},{"value":"30 December 2014","order":2,"name":"accepted","label":"Accepted","group":{"name":"ArticleHistory","label":"Article History"}},{"value":"31 January 2015","order":3,"name":"first_online","label":"First Online","group":{"name":"ArticleHistory","label":"Article History"}}],"article-number":"6"}}