Published in Methods Enzymol on January 01, 2009
Autophagy modulation as a potential therapeutic target for diverse diseases. Nat Rev Drug Discov (2012) 5.26
Control of autophagy as a therapy for neurodegenerative disease. Nat Rev Neurol (2011) 2.38
Lysosomal proteolysis inhibition selectively disrupts axonal transport of degradative organelles and causes an Alzheimer's-like axonal dystrophy. J Neurosci (2011) 2.09
The cell biology of disease: lysosomal storage disorders: the cellular impact of lysosomal dysfunction. J Cell Biol (2012) 1.93
Control of autophagy maturation by acid sphingomyelinase in mouse coronary arterial smooth muscle cells: protective role in atherosclerosis. J Mol Med (Berl) (2014) 1.59
Autophagy, a guardian against neurodegeneration. Semin Cell Dev Biol (2010) 1.45
Transcription factor EB (TFEB) is a new therapeutic target for Pompe disease. EMBO Mol Med (2013) 1.44
Suppression of autophagy permits successful enzyme replacement therapy in a lysosomal storage disorder--murine Pompe disease. Autophagy (2010) 1.38
Involvement of the Toll-like receptor 4 pathway and use of TNF-alpha antagonists for treatment of the mucopolysaccharidoses. Proc Natl Acad Sci U S A (2009) 1.37
Photoimmunotherapy: comparative effectiveness of two monoclonal antibodies targeting the epidermal growth factor receptor. Mol Oncol (2014) 1.22
Pathophysiology of neuropathic lysosomal storage disorders. J Inherit Metab Dis (2010) 1.13
Macroautophagy is defective in mucolipin-1-deficient mouse neurons. Neurobiol Dis (2010) 1.07
Pompe disease: from pathophysiology to therapy and back again. Front Aging Neurosci (2014) 1.06
Fiber type conversion by PGC-1α activates lysosomal and autophagosomal biogenesis in both unaffected and Pompe skeletal muscle. PLoS One (2010) 1.03
Beclin 1 is required for neuron viability and regulates endosome pathways via the UVRAG-VPS34 complex. PLoS Genet (2014) 0.99
Differences in the predominance of lysosomal and autophagic pathologies between infants and adults with Pompe disease: implications for therapy. Mol Genet Metab (2010) 0.93
ICP0 dismantles microtubule networks in herpes simplex virus-infected cells. PLoS One (2010) 0.93
Impaired parkin-mediated mitochondrial targeting to autophagosomes differentially contributes to tissue pathology in lysosomal storage diseases. Hum Mol Genet (2012) 0.92
PGC-1α modulates denervation-induced mitophagy in skeletal muscle. Skelet Muscle (2015) 0.91
The virulent Wolbachia strain wMelPop increases the frequency of apoptosis in the female germline cells of Drosophila melanogaster. BMC Microbiol (2012) 0.90
Skeletal muscle pathology of infantile Pompe disease during long-term enzyme replacement therapy. Orphanet J Rare Dis (2013) 0.88
The value of muscle biopsies in Pompe disease: identifying lipofuscin inclusions in juvenile- and adult-onset patients. Acta Neuropathol Commun (2014) 0.87
The role of TRAIL in mediating autophagy in myositis skeletal muscle: a potential nonimmune mechanism of muscle damage. Arthritis Rheum (2011) 0.87
Photodynamic Efficiency: From Molecular Photochemistry to Cell Death. Int J Mol Sci (2015) 0.87
Lysosomal storage diseases and the heat shock response: convergences and therapeutic opportunities. J Lipid Res (2014) 0.82
The mucolipidosis IV Ca2+ channel TRPML1 (MCOLN1) is regulated by the TOR kinase. Biochem J (2015) 0.82
Defective autophagosome trafficking contributes to impaired autophagic flux in coronary arterial myocytes lacking CD38 gene. Cardiovasc Res (2014) 0.82
Drosophila GGA model: an ultimate gateway to GGA analysis. Traffic (2011) 0.81
Therapeutic targeting of autophagy in cardiovascular disease. J Mol Cell Cardiol (2015) 0.79
Crosstalk between autophagy and apoptosis in RAW 264.7 macrophages infected with ectromelia orthopoxvirus. Viral Immunol (2013) 0.77
Autophagy induction targeting mTORC1 enhances Mycobacterium tuberculosis replication in HIV co-infected human macrophages. Sci Rep (2016) 0.77
Myotubularin family phosphatase ceMTM3 is required for muscle maintenance by preventing excessive autophagy in Caenorhabditis elegans. BMC Cell Biol (2012) 0.77
WITHDRAWN: Clearance of lysosomal glycogen accumulation by Transcription factor EB (TFEB) in muscle cells from lysosomal alpha-glucosidase deficient mice. Biochem Biophys Res Commun (2013) 0.75
Coenzyme Q10 partially restores pathological alterations in a macrophage model of Gaucher disease. Orphanet J Rare Dis (2017) 0.75
Defective Autophagy, Mitochondrial Clearance and Lipophagy in Niemann-Pick Type B Lymphocytes. PLoS One (2016) 0.75
RACK1 depletion in the ribosome induces selective translation for non-canonical autophagy. Cell Death Dis (2017) 0.75
Lysosomal Storage Diseases-Regulating Neurodegeneration. J Exp Neurosci (2016) 0.75
Fluorogenic Substrates for Visualizing Acidic Organelle Enzyme Activities. PLoS One (2016) 0.75
Modulation of mTOR signaling as a strategy for the treatment of Pompe disease. EMBO Mol Med (2017) 0.75
Interplay between Autophagy, Exosomes and HIV-1 Associated Neurological Disorders: New Insights for Diagnosis and Therapeutic Applications. Viruses (2017) 0.75
Regulation of the autophagy system during chronic contractile activity-induced muscle adaptations. Physiol Rep (2017) 0.75
NOVEL METHOD FOR DETECTION OF GLYCOGEN IN CELLS. Glycobiology (2017) 0.75
LC3, a mammalian homologue of yeast Apg8p, is localized in autophagosome membranes after processing. EMBO J (2000) 41.48
Suppression of basal autophagy in neural cells causes neurodegenerative disease in mice. Nature (2006) 24.39
p62/SQSTM1 binds directly to Atg8/LC3 to facilitate degradation of ubiquitinated protein aggregates by autophagy. J Biol Chem (2007) 21.73
Loss of autophagy in the central nervous system causes neurodegeneration in mice. Nature (2006) 21.40
Guidelines for the use and interpretation of assays for monitoring autophagy in higher eukaryotes. Autophagy (2007) 20.92
Impairment of starvation-induced and constitutive autophagy in Atg7-deficient mice. J Cell Biol (2005) 18.60
Autophagosome formation: core machinery and adaptations. Nat Cell Biol (2007) 13.59
Autophagy in metazoans: cell survival in the land of plenty. Nat Rev Mol Cell Biol (2005) 7.11
Niemann-Pick C1 disease gene: homology to mediators of cholesterol homeostasis. Science (1997) 6.66
Accumulation of autophagic vacuoles and cardiomyopathy in LAMP-2-deficient mice. Nature (2000) 6.34
Primary LAMP-2 deficiency causes X-linked vacuolar cardiomyopathy and myopathy (Danon disease). Nature (2000) 5.71
Role for Rab7 in maturation of late autophagic vacuoles. J Cell Sci (2004) 5.19
Identification of HE1 as the second gene of Niemann-Pick C disease. Science (2000) 5.08
Macroautophagy--a novel Beta-amyloid peptide-generating pathway activated in Alzheimer's disease. J Cell Biol (2005) 4.91
Murine model of Niemann-Pick C disease: mutation in a cholesterol homeostasis gene. Science (1997) 4.55
The apoptosis/autophagy paradox: autophagic vacuolization before apoptotic death. J Cell Sci (2005) 3.93
Rab7 is required for the normal progression of the autophagic pathway in mammalian cells. J Cell Sci (2004) 3.66
A block of autophagy in lysosomal storage disorders. Hum Mol Genet (2007) 3.28
Multiple sulfatase deficiency is caused by mutations in the gene encoding the human C(alpha)-formylglycine generating enzyme. Cell (2003) 2.93
Disruption of autophagy at the maturation step by the carcinogen lindane is associated with the sustained mitogen-activated protein kinase/extracellular signal-regulated kinase activity. Cancer Res (2006) 2.87
Cathepsin D deficiency underlies congenital human neuronal ceroid-lipofuscinosis. Brain (2006) 2.85
The multiple sulfatase deficiency gene encodes an essential and limiting factor for the activity of sulfatases. Cell (2003) 2.71
Analysis of mitochondrial morphology and function with novel fixable fluorescent stains. J Histochem Cytochem (1996) 2.61
Isolation of autophagic vacuoles from rat liver: morphological and biochemical characterization. J Cell Biol (1982) 2.60
Participation of autophagy in storage of lysosomes in neurons from mouse models of neuronal ceroid-lipofuscinoses (Batten disease). Am J Pathol (2005) 2.56
Class III phosphoinositide 3-kinase--Beclin1 complex mediates the amino acid-dependent regulation of autophagy in C2C12 myotubes. Biochem J (2003) 2.45
A view of acidic intracellular compartments. J Cell Biol (1988) 2.35
Autophagy is disrupted in a knock-in mouse model of juvenile neuronal ceroid lipofuscinosis. J Biol Chem (2006) 2.34
Disturbed cholesterol traffic but normal proteolytic function in LAMP-1/LAMP-2 double-deficient fibroblasts. Mol Biol Cell (2004) 2.29
Role of LAMP-2 in lysosome biogenesis and autophagy. Mol Biol Cell (2002) 2.25
Targeted disruption of the acid alpha-glucosidase gene in mice causes an illness with critical features of both infantile and adult human glycogen storage disease type II. J Biol Chem (1998) 2.24
Cathepsin D deficiency induces lysosomal storage with ceroid lipofuscin in mouse CNS neurons. J Neurosci (2000) 2.21
Enzyme replacement therapy in the mouse model of Pompe disease. Mol Genet Metab (2003) 2.17
Lysosomal glycogen storage disease with normal acid maltase. Neurology (1981) 2.13
TRP-ML1 regulates lysosomal pH and acidic lysosomal lipid hydrolytic activity. J Biol Chem (2005) 1.96
Dysfunction of endocytic and autophagic pathways in a lysosomal storage disease. Ann Neurol (2006) 1.92
Autophagy and mistargeting of therapeutic enzyme in skeletal muscle in Pompe disease. Mol Ther (2006) 1.88
Analysis of GLUT4 distribution in whole skeletal muscle fibers: identification of distinct storage compartments that are recruited by insulin and muscle contractions. J Cell Biol (1998) 1.83
Cell-autonomous death of cerebellar purkinje neurons with autophagy in Niemann-Pick type C disease. PLoS Genet (2005) 1.77
Cathepsin D deficiency is associated with a human neurodegenerative disorder. Am J Hum Genet (2006) 1.76
Oxidative stress, accumulation of biological 'garbage', and aging. Antioxid Redox Signal (2006) 1.74
Autophagy in Niemann-Pick C disease is dependent upon Beclin-1 and responsive to lipid trafficking defects. Hum Mol Genet (2007) 1.73
NPC1 and NPC2 regulate cellular cholesterol homeostasis through generation of low density lipoprotein cholesterol-derived oxysterols. J Biol Chem (2003) 1.69
A mouse model for mucopolysaccharidosis type III A (Sanfilippo syndrome). Glycobiology (1999) 1.67
A lysosomal storage disorder in mice characterized by a dual deficiency of sphingomyelinase and glucocerebrosidase. Biochim Biophys Acta (1980) 1.63
Mitochondrial aberrations in mucolipidosis Type IV. J Biol Chem (2006) 1.61
Deconstructing Pompe disease by analyzing single muscle fibers: to see a world in a grain of sand... Autophagy (2007) 1.60
Mucolipidosis type IV. Mol Genet Metab (2001) 1.56
Autophagy, mitochondria and cell death in lysosomal storage diseases. Autophagy (2007) 1.55
Lysosomal storage diseases as disorders of autophagy. Autophagy (2007) 1.46
Cholesterol accumulation is associated with lysosomal dysfunction and autophagic stress in Npc1 -/- mouse brain. Am J Pathol (2007) 1.46
Neurologic, gastric, and opthalmologic pathologies in a murine model of mucolipidosis type IV. Am J Hum Genet (2007) 1.44
A novel mechanism for the facilitation of theta-induced long-term potentiation by brain-derived neurotrophic factor. J Neurosci (2004) 1.42
Cln3(Deltaex7/8) knock-in mice with the common JNCL mutation exhibit progressive neurologic disease that begins before birth. Hum Mol Genet (2002) 1.41
Pathogenic mechanisms in lysosomal disease: a reappraisal of the role of the lysosome. Acta Paediatr Suppl (2007) 1.38
The genetic spectrum of human neuronal ceroid-lipofuscinoses. Brain Pathol (2004) 1.30
Membrane trafficking and mitochondrial abnormalities precede subunit c deposition in a cerebellar cell model of juvenile neuronal ceroid lipofuscinosis. BMC Neurosci (2004) 1.27
Systemic inflammation and neurodegeneration in a mouse model of multiple sulfatase deficiency. Proc Natl Acad Sci U S A (2007) 1.25
Accumulation of glycosphingolipids in Niemann-Pick C disease disrupts endosomal transport. J Biol Chem (2004) 1.22
Enhanced autophagy and mitochondrial aberrations in murine G(M1)-gangliosidosis. Biochem Biophys Res Commun (2008) 1.21
Cellular mechanism of U18666A-mediated apoptosis in cultured murine cortical neurons: bridging Niemann-Pick disease type C and Alzheimer's disease. Cell Signal (2006) 1.16
The organization of the Golgi complex and microtubules in skeletal muscle is fiber type-dependent. J Neurosci (1999) 1.13
Autophagic vacuolar myopathies. Curr Neurol Neurosci Rep (2003) 1.13
Cell and gene-based therapies for the lysosomal storage diseases. Curr Gene Ther (2006) 1.10
Measurement of vacuolar pH and cytoplasmic calcium in living cells using fluorescence microscopy. Methods Enzymol (1989) 1.09
Lipid trafficking defects increase Beclin-1 and activate autophagy in Niemann-Pick type C disease. Autophagy (2007) 1.05
A novel missense mutation in lysosomal sulfamidase is the basis of MPS III A in a spontaneous mouse mutant. Glycobiology (2001) 1.02
Follow-up study of subunit c of mitochondrial ATP synthase (SCMAS) in Batten disease and in unrelated lysosomal disorders. Acta Neuropathol (1997) 1.01
Insights into the diagnosis and treatment of lysosomal storage diseases. Arch Neurol (2003) 0.98
Sphingomyelinosis, a new mutation in the mouse: a model of Niemann-Pick disease in humans. J Hered (1982) 0.97
Autophagic-lysosomal dysfunction and neurodegeneration in Niemann-Pick Type C mice: lipid starvation or indigestion? Autophagy (2007) 0.97
Beta-galactosidase-deficient mouse as an animal model for GM1-gangliosidosis. Glycoconj J (1997) 0.93
Tripeptidyl peptidase I, the late infantile neuronal ceroid lipofuscinosis gene product, initiates the lysosomal degradation of subunit c of ATP synthase. J Biochem (2000) 0.90
Danon disease as a cause of autophagic vacuolar myopathy. Congenit Heart Dis (2008) 0.87
Development of lysosomal storage in mice with targeted disruption of the beta-galactosidase gene: a model of human G(M1)-gangliosidosis. Brain Dev (2001) 0.82
Cathepsin D deficiency and NCL/Batten disease: there's more to death than apoptosis. Autophagy (2007) 0.82
Enhanced autoantigen expression in regenerating muscle cells in idiopathic inflammatory myopathy. J Exp Med (2005) 2.82
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 (2008) 2.15
Acid alpha-glucosidase deficiency (glycogenosis type II, Pompe disease). Curr Mol Med (2002) 1.82
Activation of the endoplasmic reticulum stress response in autoimmune myositis: potential role in muscle fiber damage and dysfunction. Arthritis Rheum (2005) 1.67
Dysferlin deficiency enhances monocyte phagocytosis: a model for the inflammatory onset of limb-girdle muscular dystrophy 2B. Am J Pathol (2008) 1.65
Therapeutic approaches in glycogen storage disease type II/Pompe Disease. Neurotherapeutics (2008) 1.22
N-terminal RASSF family: RASSF7-RASSF10. Epigenetics (2011) 1.17
Lower corneal hysteresis is associated with more rapid glaucomatous visual field progression. J Glaucoma (2012) 1.15
When more is less: excess and deficiency of autophagy coexist in skeletal muscle in Pompe disease. Autophagy (2009) 0.99
KIBRA gene methylation is associated with unfavorable biological prognostic parameters in chronic lymphocytic leukemia. Epigenetics (2012) 0.90
Autoantigens signal through chemokine receptors: uveitis antigens induce CXCR3- and CXCR5-expressing lymphocytes and immature dendritic cells to migrate. Blood (2005) 0.89
Autoantigens act as tissue-specific chemoattractants. J Leukoc Biol (2005) 0.85
Ganglion impar blockade: a review. Curr Pain Headache Rep (2013) 0.85
RASSF6 exhibits promoter hypermethylation in metastatic melanoma and inhibits invasion in melanoma cells. Epigenetics (2014) 0.84
Impaired organization and function of myofilaments in single muscle fibers from a mouse model of Pompe disease. J Appl Physiol (1985) (2010) 0.84
Chemokine receptors on dendritic cells promote autoimmune reactions. Arthritis Res (2002) 0.84
The human acid alpha-glucosidase gene is a novel target of the Notch-1/Hes-1 signaling pathway. J Biol Chem (2002) 0.80
β-lactam allergy: clinical implications and costs. Clin Mol Allergy (2013) 0.75
Helios gene gun particle delivery for therapy of acid maltase deficiency. DNA Cell Biol (2002) 0.75