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Volumn , Issue , 2012, Pages 331-354

Lysosome storage disorders on the brain: The autophagy lysosome pathway contributes to disease pathophysiology and may be utilized for therapeutic benefit

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EID: 84971216516     PISSN: None     EISSN: None     Source Type: Book    
DOI: 10.1142/9789814350457_0014     Document Type: Chapter
Times cited : (1)

References (83)
  • 1
    • 1042288280 scopus 로고    scopus 로고
    • The intracellular location and function of proteins of neuronal ceroid lipofuscinoses
    • J. Ezaki and E. Kominami, The intracellular location and function of proteins of neuronal ceroid lipofuscinoses, Brain Pathol 14: 77-85 (2004).
    • (2004) Brain Pathol , vol.14 , pp. 77-85
    • Ezaki, J.1    Kominami, E.2
  • 2
    • 28244435957 scopus 로고    scopus 로고
    • Cathepsin deficiency as a model for neuronal ceroid lipofuscinoses
    • J. J. Shacka and K. A. Roth, Cathepsin deficiency as a model for neuronal ceroid lipofuscinoses, Am J Pathol 167: 1473-1476 (2005).
    • (2005) Am J Pathol , vol.167 , pp. 1473-1476
    • Shacka, J.J.1    Roth, K.A.2
  • 3
    • 34548059220 scopus 로고    scopus 로고
    • Cathepsin D deficiency and NCL/Batten disease: There’s more to death than apoptosis
    • J. J. Shacka and K. A. Roth, Cathepsin D deficiency and NCL/Batten disease: there’s more to death than apoptosis, Autophagy 3: 474-476 (2007).
    • (2007) Autophagy , vol.3 , pp. 474-476
    • Shacka, J.J.1    Roth, K.A.2
  • 4
    • 77950675049 scopus 로고    scopus 로고
    • Neuronopathic Gaucher disease in the mouse: Viable combined selective saposin C deficiency and mutant glucocerebrosidase (V394L) mice with glucosylsphingosine and glucosylceramide accumulation and progressive neurological deficits
    • Y. Sun, B. Liou, H. Ran, M. R. Skelton, M. T. Williams, C. V. Vorhees, K. Kitatani, Y. A. Hannun, D. P. Witte, Y. H. Xu and G. A. Grabowski, Neuronopathic Gaucher disease in the mouse: viable combined selective saposin C deficiency and mutant glucocerebrosidase (V394L) mice with glucosylsphingosine and glucosylceramide accumulation and progressive neurological deficits, Hum Mol Genet 19: 1088-1097 (2010).
    • (2010) Hum Mol Genet , vol.19 , pp. 1088-1097
    • Sun, Y.1    Liou, B.2    Ran, H.3    Skelton, M.R.4    Williams, M.T.5    Vorhees, C.V.6    Kitatani, K.7    Hannun, Y.A.8    Witte, D.P.9    Xu, Y.H.10    Grabowski, G.A.11
  • 5
    • 77955447007 scopus 로고    scopus 로고
    • Impaired autophagosomes and lysosomes in neuronopathic Gaucher disease
    • Y. Sun and G. A. Grabowski, Impaired autophagosomes and lysosomes in neuronopathic Gaucher disease, Autophagy 6: 648-649 (2010).
    • (2010) Autophagy , vol.6 , pp. 648-649
    • Sun, Y.1    Grabowski, G.A.2
  • 7
    • 67849118764 scopus 로고    scopus 로고
    • Cystinosis of the brain and spinal cord with associated vasculopathy
    • J. R. Berger, D. A. Dillon, B. A. Young, S. J. Goldstein and P. Nelson, Cystinosis of the brain and spinal cord with associated vasculopathy, J Neurol Sci 284: 182-185 (2009).
    • (2009) J Neurol Sci , vol.284 , pp. 182-185
    • Berger, J.R.1    Dillon, D.A.2    Young, B.A.3    Goldstein, S.J.4    Nelson, P.5
  • 8
    • 36148991943 scopus 로고    scopus 로고
    • Cell-autonomous death of cerebellar purkinje neurons with autophagy in Niemann-Pick type C disease
    • D. C. Ko, L. Milenkovic, S. M. Beier, H. Manuel, J. Buchanan and M. P. Scott, Cell-autonomous death of cerebellar purkinje neurons with autophagy in Niemann-Pick type C disease, PLoS Genet 1: 81-95 (2005).
    • (2005) Plos Genet , vol.1 , pp. 81-95
    • Ko, D.C.1    Milenkovic, L.2    Beier, S.M.3    Manuel, H.4    Buchanan, J.5    Scott, M.P.6
  • 11
    • 33845343984 scopus 로고    scopus 로고
    • Sphingolipid metabolism diseases
    • T. Kolter and K. Sandhoff, Sphingolipid metabolism diseases, Biochim Biophys Acta 1758: 2057-2079 (2006).
    • (2006) Biochim Biophys Acta , vol.1758 , pp. 2057-2079
    • Kolter, T.1    Sandhoff, K.2
  • 15
    • 76249099881 scopus 로고    scopus 로고
    • Abnormal autophagy, ubiquitination, inflammation and apoptosis are dependent upon lysosomal storage and are useful biomarkers of mucopolysaccharidosis VI
    • A. Tessitore, M. Pirozzi and A. Auricchio, Abnormal autophagy, ubiquitination, inflammation and apoptosis are dependent upon lysosomal storage and are useful biomarkers of mucopolysaccharidosis VI, Pathogenetics 2: 4 (2009).
    • (2009) Pathogenetics , vol.2 , pp. 4
    • Tessitore, A.1    Pirozzi, M.2    Auricchio, A.3
  • 19
    • 70350618320 scopus 로고    scopus 로고
    • Inhibition of autophagosome formation restores mitochondrial function in mucolipidosis II and III skin fibroblasts
    • T. Otomo, K. Higaki, E. Nanba, K. Ozono and N. Sakai, Inhibition of autophagosome formation restores mitochondrial function in mucolipidosis II and III skin fibroblasts, Mol Genet Metab 98: 393-399 (2009).
    • (2009) Mol Genet Metab , vol.98 , pp. 393-399
    • Otomo, T.1    Higaki, K.2    Nanba, E.3    Ozono, K.4    Sakai, N.5
  • 20
    • 54049156405 scopus 로고    scopus 로고
    • The type IV mucolipidosis-associated protein TRPML1 is an endolysosomal iron release channel
    • X. P. Dong, X. Cheng, E. Mills, M. Delling, F. Wang, T. Kurz and H. Xu, The type IV mucolipidosis-associated protein TRPML1 is an endolysosomal iron release channel, Nature 455: 992-996 (2008).
    • (2008) Nature , vol.455 , pp. 992-996
    • Dong, X.P.1    Cheng, X.2    Mills, E.3    Delling, M.4    Wang, F.5    Kurz, T.6    Xu, H.7
  • 21
    • 78049405392 scopus 로고    scopus 로고
    • Zinc dyshomeostasis is linked with the loss of mucolipidosis IV-associated TRPML1 ion channel
    • J. L. Eichelsdoerfer, J. A. Evans, S. A. Slaugenhaupt and M. P. Cuajungco, Zinc dyshomeostasis is linked with the loss of mucolipidosis IV-associated TRPML1 ion channel, J Biol Chem 285: 34304-34308 (2010).
    • (2010) J Biol Chem , vol.285 , pp. 34304-34308
    • Eichelsdoerfer, J.L.1    Evans, J.A.2    Slaugenhaupt, S.A.3    Cuajungco, M.P.4
  • 24
    • 43949112966 scopus 로고    scopus 로고
    • LAMP-2: A control step for phagosome and autophagosome maturation
    • P. Saftig, W. Beertsen and E. L. Eskelinen, LAMP-2: a control step for phagosome and autophagosome maturation, Autophagy 4: 510-512 (2008).
    • (2008) Autophagy , vol.4 , pp. 510-512
    • Saftig, P.1    Beertsen, W.2    Eskelinen, E.L.3
  • 26
    • 77953057020 scopus 로고    scopus 로고
    • A 13-year-old girl with proximal weakness and hypertrophic cardiomyopathy with Danon disease
    • H. Kim, A. Cho, B. C. Lim, M. J. Kim, K. J. Kim, I. Nishino, Y. S. Hwang and J. H. Chae, A 13-year-old girl with proximal weakness and hypertrophic cardiomyopathy with Danon disease, Muscle Nerve 41: 879-882 (2010).
    • (2010) Muscle Nerve , vol.41 , pp. 879-882
    • Kim, H.1    Cho, A.2    Lim, B.C.3    Kim, M.J.4    Kim, K.J.5    Nishino, I.6    Hwang, Y.S.7    Chae, J.H.8
  • 27
    • 62949091373 scopus 로고    scopus 로고
    • Autophagy: A lysosomal degradation pathway with a central role in health and disease
    • E. L. Eskelinen and P. Saftig, Autophagy: a lysosomal degradation pathway with a central role in health and disease, Biochim Biophys Acta 1793: 664-673 (2009).
    • (2009) Biochim Biophys Acta , vol.1793 , pp. 664-673
    • Eskelinen, E.L.1    Saftig, P.2
  • 28
    • 48249103491 scopus 로고    scopus 로고
    • Neurodegenerative lysosomal disorders — a continuum from development to late age
    • R. A. Nixon, D. S. Yang and J. H. Lee, Neurodegenerative lysosomal disorders — a continuum from development to late age, Autophagy 4: 590-599 (2008).
    • (2008) Autophagy , vol.4 , pp. 590-599
    • Nixon, R.A.1    Yang, D.S.2    Lee, J.H.3
  • 31
    • 38949108670 scopus 로고    scopus 로고
    • Guidelines for the use and interpretation of assays for monitoring autophagy in higher eukaryotes
    • D. J. Klionsky, H. Abeliovich, P. Agostinis, D. K. Agrawal, et al., Guidelines for the use and interpretation of assays for monitoring autophagy in higher eukaryotes, Autophagy 4: 151-175 (2007).
    • (2007) Autophagy , vol.4 , pp. 151-175
    • Klionsky, D.J.1    Abeliovich, H.2    Agostinis, P.3    Agrawal, D.K.4
  • 33
    • 57049094929 scopus 로고    scopus 로고
    • Suppression of autophagy in skeletal muscle uncovers the accumulation of ubiquitinated proteins and their potential role in muscle damage in Pompe disease
    • N. Raben, V. Hill, L. Shea, S. Takikita, R. Baum, N. Mizushima, E. Ralston and P. Plotz, Suppression of autophagy in skeletal muscle uncovers the accumulation of ubiquitinated proteins and their potential role in muscle damage in Pompe disease, Hum Mol Genet 17: 3897-3908 (2008).
    • (2008) Hum Mol Genet , vol.17 , pp. 3897-3908
    • Raben, N.1    Hill, V.2    Shea, L.3    Takikita, S.4    Baum, R.5    Mizushima, N.6    Ralston, E.7    Plotz, P.8
  • 34
    • 34948909145 scopus 로고    scopus 로고
    • Abnormal chaperone-mediated autophagy (CMA) in cardiomyocytes of a boy with Danon disease
    • A. Fidzianska, E. Walczak and M. Walski, Abnormal chaperone-mediated autophagy (CMA) in cardiomyocytes of a boy with Danon disease, Folia Neuropathol 45: 133-139 (2007).
    • (2007) Folia Neuropathol , vol.45 , pp. 133-139
    • Fidzianska, A.1    Walczak, E.2    Walski, M.3
  • 35
    • 78549273390 scopus 로고    scopus 로고
    • Macroautophagy is not directly involved in the metabolism of amyloid precursor protein
    • B. Boland, D. A. Smith, D. Mooney, S. S. Jung, D. M. Walsh and F. M. Platt, Macroautophagy is not directly involved in the metabolism of amyloid precursor protein, J Biol Chem 285: 37415-37428 (2010).
    • (2010) J Biol Chem , vol.285 , pp. 37415-37428
    • Boland, B.1    Smith, D.A.2    Mooney, D.3    Jung, S.S.4    Walsh, D.M.5    Platt, F.M.6
  • 39
    • 27944504351 scopus 로고    scopus 로고
    • P62/SQSTM1 forms protein aggregates degraded by autophagy and has a protective effect on huntingtin-induced cell death
    • G. Bj0rk0y, T. Lamark, A. Brech, H. Outzen, M. Perander, A. 0vervatn, H. Stenmark and T. Johansen, p62/SQSTM1 forms protein aggregates degraded by autophagy and has a protective effect on huntingtin-induced cell death, J Cell Biol 171: 603-614 (2005).
    • (2005) J Cell Biol , vol.171 , pp. 603-614
    • Bj0rk0y, G.1    Lamark, T.2    Brech, A.3    Outzen, H.4    Perander, M.5    Overvatn, A.6    Stenmark, H.7    Johansen, T.8
  • 40
    • 77958185918 scopus 로고    scopus 로고
    • Saposin C mutations in Gaucher disease patients resulting in lysosomal lipid accumulation, saposin C deficiency, but normal prosaposin processing and sorting
    • A. M. Vaccaro, M. Motta, M. Tatti, S. Scarpa, L. Masuelli, M. Bhat, M. T. Vanier, A. Tylki-Szymanska and R. Salvioli, Saposin C mutations in Gaucher disease patients resulting in lysosomal lipid accumulation, saposin C deficiency, but normal prosaposin processing and sorting, Hum Mol Genet 19: 2987-2997 (2010).
    • (2010) Hum Mol Genet , vol.19 , pp. 2987-2997
    • Vaccaro, A.M.1    Motta, M.2    Tatti, M.3    Scarpa, S.4    Masuelli, L.5    Bhat, M.6    Vanier, M.T.7    Tylki-Szymanska, A.8    Salvioli, R.9
  • 41
    • 50249095558 scopus 로고    scopus 로고
    • Mucolipidosis type IV: The importance of functional lysosomes for efficient autophagy
    • S. Vergarajauregui and R. Puertollano, Mucolipidosis type IV: the importance of functional lysosomes for efficient autophagy, Autophagy 4: 832-834 (2008).
    • (2008) Autophagy , vol.4 , pp. 832-834
    • Vergarajauregui, S.1    Puertollano, R.2
  • 42
    • 34548860918 scopus 로고    scopus 로고
    • Cholesterol accumulation is associated with lysosomal dysfunction and autophagic stress in Npc1 -/- mouse brain
    • G. Liao, Y. Yao, J. Liu, Z. Yu, S. Cheung, A. Xie, X. Liang and X. Bi, Cholesterol accumulation is associated with lysosomal dysfunction and autophagic stress in Npc1 -/- mouse brain, Am J Pathol 171: 962-975 (2007).
    • (2007) Am J Pathol , vol.171 , pp. 962-975
    • Liao, G.1    Yao, Y.2    Liu, J.3    Yu, Z.4    Cheung, S.5    Xie, A.6    Liang, X.7    Bi, X.8
  • 45
    • 34447302577 scopus 로고    scopus 로고
    • Autophagy in Niemann-Pick C disease is dependent upon Beclin-1 and responsive to lipid trafficking defects
    • C. D. Pacheco, R. Kunkel and A. P. Lieberman, Autophagy in Niemann-Pick C disease is dependent upon Beclin-1 and responsive to lipid trafficking defects, Hum Mol Genet 16: 1495-1503 (2007).
    • (2007) Hum Mol Genet , vol.16 , pp. 1495-1503
    • Pacheco, C.D.1    Kunkel, R.2    Lieberman, A.P.3
  • 46
    • 35848967804 scopus 로고    scopus 로고
    • How to interpret LC3 immunoblotting
    • N. Mizushima and T. Yoshimori, How to interpret LC3 immunoblotting, Autophagy 3: 542-545 (2007).
    • (2007) Autophagy , vol.3 , pp. 542-545
    • Mizushima, N.1    Yoshimori, T.2
  • 48
    • 2442522365 scopus 로고    scopus 로고
    • Salicylihalamide A inhibits the V0 sector of the V-ATPase through a mechanism distinct from bafilomycin A1
    • X. S. Xie, D. Padron, X. Liao, J. Wang, M. G. Roth and J. K. de Brabander, Salicylihalamide A inhibits the V0 sector of the V-ATPase through a mechanism distinct from bafilomycin A1, J Biol Chem 279: 19755-19763 (2004).
    • (2004) J Biol Chem , vol.279 , pp. 19755-19763
    • Xie, X.S.1    Padron, D.2    Liao, X.3    Wang, J.4    Roth, M.G.5    De Brabander, J.K.6
  • 50
  • 51
    • 24644471460 scopus 로고    scopus 로고
    • Cell death pathways in juvenile Batten disease
    • D. A. Persaud-Sawin and R. M. Boustany, Cell death pathways in juvenile Batten disease, Apoptosis 10: 973-985 (2005).
    • (2005) Apoptosis , vol.10 , pp. 973-985
    • Persaud-Sawin, D.A.1    Boustany, R.M.2
  • 52
    • 78651265495 scopus 로고    scopus 로고
    • Sphingosine-1-phosphate phosphohydrolase-1 regulates ER stress-induced autophagy
    • S. Lepine, J. C. Allegood, M. Park, P. Dent, S. Milstien and S. Spiegel, Sphingosine-1-phosphate phosphohydrolase-1 regulates ER stress-induced autophagy, Cell Death Differ 18: 350-361 (2010).
    • (2010) Cell Death Differ , vol.18 , pp. 350-361
    • Lepine, S.1    Allegood, J.C.2    Park, M.3    Dent, P.4    Milstien, S.5    Spiegel, S.6
  • 54
    • 69349090907 scopus 로고    scopus 로고
    • The cellular pathways of neuronal autophagy and their implication in neurodegenerative diseases
    • Z. Yue, L. Friedman, M. Komatsu and K. Tanaka, The cellular pathways of neuronal autophagy and their implication in neurodegenerative diseases, Biochim Biophys Acta 1793: 1496-1507 (2009).
    • (2009) Biochim Biophys Acta , vol.1793 , pp. 1496-1507
    • Yue, Z.1    Friedman, L.2    Komatsu, M.3    Tanaka, K.4
  • 57
    • 0023760665 scopus 로고
    • The new stereological tools: Disector, fractionator, nucleator and point sampled intercepts and their use in pathological research and diagnosis
    • H. J. Gundersen, P. Bagger, T. F. Bendtsen, S. M. Evans, et al., The new stereological tools: disector, fractionator, nucleator and point sampled intercepts and their use in pathological research and diagnosis, APMIS 96: 857-881 (1988).
    • (1988) APMIS , vol.96 , pp. 857-881
    • Gundersen, H.J.1    Bagger, P.2    Bendtsen, T.F.3    Evans, S.M.4
  • 58
    • 67349202845 scopus 로고    scopus 로고
    • Allopregnanolone treatment delays cholesterol accumulation and reduces autophagic/lysosomal dysfunction and inflammation in Npc1-/- mouse brain
    • G. Liao, S. Cheung, J. Galeano, A. X. Ji, Q. Qin and X. Bi, Allopregnanolone treatment delays cholesterol accumulation and reduces autophagic/lysosomal dysfunction and inflammation in Npc1-/- mouse brain, Brain Res 1270: 140-151 (2009).
    • (2009) Brain Res , vol.1270 , pp. 140-151
    • Liao, G.1    Cheung, S.2    Galeano, J.3    Ji, A.X.4    Qin, Q.5    Bi, X.6
  • 59
    • 77951217000 scopus 로고    scopus 로고
    • Dual role of 3-methyladenine in modulation of autophagy via different temporal patterns of inhibition on class I and III phosphoinositide 3-kinase
    • Y. T. Wu, H. L. Tan, G. Shui, C. Bauvy, Q. Huang, M. R. Wenk, C. N. Ong, P. Codogno and H. M. Shen, Dual role of 3-methyladenine in modulation of autophagy via different temporal patterns of inhibition on class I and III phosphoinositide 3-kinase, J Biol Chem 285: 10850-10861 (2010).
    • (2010) J Biol Chem , vol.285 , pp. 10850-10861
    • Wu, Y.T.1    Tan, H.L.2    Shui, G.3    Bauvy, C.4    Huang, Q.5    Wenk, M.R.6    Ong, C.N.7    Codogno, P.8    Shen, H.M.9
  • 60
    • 33646419529 scopus 로고    scopus 로고
    • Over-expression of CLN3P, the Batten disease protein, inhibits PANDER-induced apoptosis in neuroblastoma cells: Further evidence that CLN3P has anti-apoptotic properties
    • S. B. Narayan, D. Rakheja, J. V. Pastor, K. Rosenblatt, S. R. Greene, J. Yang, B. A. Wolf and M. J. Bennett, Over-expression of CLN3P, the Batten disease protein, inhibits PANDER-induced apoptosis in neuroblastoma cells: Further evidence that CLN3P has anti-apoptotic properties, Mol Genet Metab 88: 178-183 (2006).
    • (2006) Mol Genet Metab , vol.88 , pp. 178-183
    • Narayan, S.B.1    Rakheja, D.2    Pastor, J.V.3    Rosenblatt, K.4    Greene, S.R.5    Yang, J.6    Wolf, B.A.7    Bennett, M.J.8
  • 62
    • 74849083093 scopus 로고    scopus 로고
    • Lysosomal enhancement: A CLEAR answer to cellular degradative needs
    • M. Sardiello and A. Ballabio, Lysosomal enhancement: a CLEAR answer to cellular degradative needs, Cell Cycle 8: 4021-4022 (2009).
    • (2009) Cell Cycle , vol.8 , pp. 4021-4022
    • Sardiello, M.1    Ballabio, A.2
  • 64
    • 39449083733 scopus 로고    scopus 로고
    • Antioxidants and free radical scavengers for the treatment of stroke, traumatic brain injury and aging
    • J. E. Slemmer, J. J. Shacka, M. I. Sweeney and J. T. Weber, Antioxidants and free radical scavengers for the treatment of stroke, traumatic brain injury and aging, Curr Med Chem 15: 404-414 (2008).
    • (2008) Curr Med Chem , vol.15 , pp. 404-414
    • Slemmer, J.E.1    Shacka, J.J.2    Sweeney, M.I.3    Weber, J.T.4
  • 65
    • 58849163098 scopus 로고    scopus 로고
    • Oxidative stress and autophagy in the regulation of lysosome-dependent neuron death
    • V. N. Pivtoraiko, S. L. Stone, K. A. Roth and J. J. Shacka, Oxidative stress and autophagy in the regulation of lysosome-dependent neuron death, Antioxid Redox Signal 11: 481-496 (2009).
    • (2009) Antioxid Redox Signal , vol.11 , pp. 481-496
    • Pivtoraiko, V.N.1    Stone, S.L.2    Roth, K.A.3    Shacka, J.J.4
  • 66
    • 34250016055 scopus 로고    scopus 로고
    • Apoptotic neuronal death in Parkinson’s disease: Involvement of nitric oxide
    • S. Singh and M. Dikshit, Apoptotic neuronal death in Parkinson’s disease: involvement of nitric oxide, Brain Res Rev 54: 233-250 (2007).
    • (2007) Brain Res Rev , vol.54 , pp. 233-250
    • Singh, S.1    Dikshit, M.2
  • 67
    • 0028929548 scopus 로고
    • Neurofibrillary tangles in Niemann-Pick disease type C
    • S. Love, L. R. Bridges and C. P. Case, Neurofibrillary tangles in Niemann-Pick disease type C, Brain 118(Pt 1): 119-129 (1995).
    • (1995) Brain , vol.118 , pp. 119-129
    • Love, S.1    Bridges, L.R.2    Case, C.P.3
  • 68
    • 56349119573 scopus 로고    scopus 로고
    • Motor deficit in a Drosophila model of mucolipidosis type IV due to defective clearance of apoptotic cells
    • K. Venkatachalam, A. A. Long, R. Elsaesser, D. Nikolaeva, K. Broadie and C. Montell, Motor deficit in a Drosophila model of mucolipidosis type IV due to defective clearance of apoptotic cells, Cell 135: 838-851 (2008).
    • (2008) Cell , vol.135 , pp. 838-851
    • Venkatachalam, K.1    Long, A.A.2    Elsaesser, R.3    Nikolaeva, D.4    Broadie, K.5    Montell, C.6
  • 70
    • 60549091933 scopus 로고    scopus 로고
    • Tau deletion exacerbates the phenotype of Niemann-Pick type C mice and implicates autophagy in pathogenesis
    • C. D. Pacheco, M. J. Elrick and A. P. Lieberman, Tau deletion exacerbates the phenotype of Niemann-Pick type C mice and implicates autophagy in pathogenesis, Hum Mol Genet 18: 956-965 (2009).
    • (2009) Hum Mol Genet , vol.18 , pp. 956-965
    • Pacheco, C.D.1    Elrick, M.J.2    Lieberman, A.P.3
  • 73
    • 34248593481 scopus 로고    scopus 로고
    • Altered regulation of phosphatidylinositol 3-kinase signaling in cathep-sin D-deficient brain
    • K. C. Walls, B. J. Klocke, P. Saftig, M. Shibata, Y. Uchiyama, K. A. Roth and J. J. Shacka, Altered regulation of phosphatidylinositol 3-kinase signaling in cathep-sin D-deficient brain, Autophagy 3: 222-229 (2007).
    • (2007) Autophagy , vol.3 , pp. 222-229
    • Walls, K.C.1    Klocke, B.J.2    Saftig, P.3    Shibata, M.4    Uchiyama, Y.5    Roth, K.A.6    Shacka, J.J.7
  • 74
    • 33847215777 scopus 로고    scopus 로고
    • Cathepsin D deficiency induces persistent neurodegeneration in the absence of Bax-dependent apoptosis
    • J. J. Shacka, B. J. Klocke, C. Young, M. Shibata, J. W. Olney, Y. Uchiyama, P. Saftig and K. A. Roth, Cathepsin D deficiency induces persistent neurodegeneration in the absence of Bax-dependent apoptosis, J Neurosci 27: 2081-2090 (2007).
    • (2007) J Neurosci , vol.27 , pp. 2081-2090
    • Shacka, J.J.1    Klocke, B.J.2    Young, C.3    Shibata, M.4    Olney, J.W.5    Uchiyama, Y.6    Saftig, P.7    Roth, K.A.8
  • 75
    • 51549106105 scopus 로고    scopus 로고
    • Cathepsin D is the main lysosomal enzyme involved in the degradation of alpha-synuclein and generation of its carboxy-terminally truncated species
    • D. Sevlever, P. Jiang and S. H. Yen, Cathepsin D is the main lysosomal enzyme involved in the degradation of alpha-synuclein and generation of its carboxy-terminally truncated species, Biochemistry 47: 9678-9687 (2008).
    • (2008) Biochemistry , vol.47 , pp. 9678-9687
    • Sevlever, D.1    Jiang, P.2    Yen, S.H.3
  • 76
    • 58149215720 scopus 로고    scopus 로고
    • Regulation of neuronal survival factor MEF2D by chaperone-mediated autophagy
    • Q. Yang, H. She, M. Gearing, E. Colla, M. Lee, J. J. Shacka and Z. Mao, Regulation of neuronal survival factor MEF2D by chaperone-mediated autophagy, Science 323: 124-127 (2009).
    • (2009) Science , vol.323 , pp. 124-127
    • Yang, Q.1    She, H.2    Gearing, M.3    Colla, E.4    Lee, M.5    Shacka, J.J.6    Mao, Z.7
  • 79
    • 79952619654 scopus 로고    scopus 로고
    • Accumulation and distribution of alpha-synuclein and ubiquitin in the CNS of Gaucher disease mouse models
    • Y. H. Xu, Y. Sun, H. Ran, B. Quinn, D. Witte and G. A. Grabowski, Accumulation and distribution of alpha-synuclein and ubiquitin in the CNS of Gaucher disease mouse models, Mol Genet Metab 102: 436-447 (2011).
    • (2011) Mol Genet Metab , vol.102 , pp. 436-447
    • Xu, Y.H.1    Sun, Y.2    Ran, H.3    Quinn, B.4    Witte, D.5    Grabowski, G.A.6
  • 80
    • 78149410222 scopus 로고    scopus 로고
    • Glucocerebrosidase is present in alpha-synuclein inclusions in Lewy body disorders
    • O. Goker-Alpan, B. K. Stubblefield, B. I. Giasson and E. Sidransky, Glucocerebrosidase is present in alpha-synuclein inclusions in Lewy body disorders, Acta Neuropathol 120: 641-649 (2010).
    • (2010) Acta Neuropathol , vol.120 , pp. 641-649
    • Goker-Alpan, O.1    Stubblefield, B.K.2    Giasson, B.I.3    Sidransky, E.4
  • 81
    • 38449091923 scopus 로고    scopus 로고
    • The autophagy-lysosomal degradation pathway: Role in neurodegenerative disease and therapy
    • J. J. Shacka, K. A. Roth and J. Zhang, The autophagy-lysosomal degradation pathway: role in neurodegenerative disease and therapy, Front Biosci 13: 718-736 (2008).
    • (2008) Front Biosci , vol.13 , pp. 718-736
    • Shacka, J.J.1    Roth, K.A.2    Zhang, J.3
  • 83
    • 0029970783 scopus 로고    scopus 로고
    • Amyloidogenic processing of human amyloid precursor protein in hippocampal neurons devoid of cathepsin D
    • P. Saftig, C. Peters, K. von Figura, K. Craessaerts, F. Van Leuven and B. De Strooper, Amyloidogenic processing of human amyloid precursor protein in hippocampal neurons devoid of cathepsin D, J Biol Chem 271: 27241-27244 (1996).
    • (1996) J Biol Chem , vol.271 , pp. 27241-27244
    • Saftig, P.1    Peters, C.2    Von Figura, K.3    Craessaerts, K.4    Van Leuven, F.5    De Strooper, B.6


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