Standout Papers
- Mitochondrial ATP synthase subunit c storage in the ceroid‐lipofuscinoses (Batten disease) (1992)
- Characterization of the expressed genes for subunit c of mitochondrial ATP synthase in sheep with ceroid lipofuscinosis (1993)
- The sequence of the major protein stored in ovine ceroid lipofuscinosis is identical with that of the dicyclohexylcarbodiimide-reactive proteolipid of mitochondrial ATP synthase (1990)
Citation Impact
Citing Papers
You say lipofuscin, we say ceroid: Defining autofluorescent storage material
2006
The sequences of human and bovine genes of the phosphate carrier from mitochondria contain evidence of alternatively spliced forms.
1994 StandoutNobel
Lysosomes in iron metabolism, ageing and apoptosis
2008
Sequences of members of the human gene family for the c subunit of mitochondrial ATP synthase
1993 StandoutNobel
Invited Review Free Radicals in Disease Processes: A Compilation of Cause and Consequence
1993
Gene activities that mediate increased life span of C. elegans insulin-like signaling mutants
2007 StandoutNobel
Free radicals, antioxidants, and human disease: curiosity, cause, or consequence?
1994 Standout
A Family of Basic Amino Acid Transporters of the Vacuolar Membrane from Saccharomyces cerevisiae
2004 StandoutNobel
Autophagy, organelles and ageing
2007
Neuronal ceroid lipofuscinosis protein CLN3 interacts with motor proteins and modifies location of late endosomal compartments
2012
Targeted Disruption of the Cln3 Gene Provides a Mouse Model for Batten Disease
1999
Clarifying lysosomal storage diseases
2011
The Role of Autophagy, Mitophagy and Lysosomal Functions in Modulating Bioenergetics and Survival in the Context of Redox and Proteotoxic Damage: Implications for Neurodegenerative Diseases
2016
Biochemical basis of lipofuscin, ceroid, and age pigment-like fluorophores
1996
The role of autophagy in neurodegenerative disease
2013 Standout
The CLN9 Protein, a Regulator of Dihydroceramide Synthase
2005
Prevalence of Lysosomal Storage Disorders
1999 Standout
Selective Transport of α-Mannosidase by Autophagic Pathways
2010 StandoutNobel
Pigments in Aging: An Overview
2002
Autophagy Is Disrupted in a Knock-in Mouse Model of Juvenile Neuronal Ceroid Lipofuscinosis
2006
Mitochondrial Dysfunction and Neurodegeneration in Lysosomal Storage Disorders
2017
Selective Transport of α-Mannosidase by Autophagic Pathways
2010 StandoutNobel
Principles of bioactive lipid signalling: lessons from sphingolipids
2008 Standout
Vascular Endothelial Growth Factor Regulates Endothelial Cell Survival through the Phosphatidylinositol 3′-Kinase/Akt Signal Transduction Pathway
1998 Standout
Lipofuscin: mechanisms of age-related accumulation and influence on cell function12 1Guest Editor: Rajindar S. Sohal 2This article is part of a series of reviews on “Oxidative Stress and Aging.” The full list of papers may be found on the homepage of the journal.
2002
The morphology of lipopigment in rat Purkenje neurons after chronic acetyl-l-carnitine administration: A reduction in aging-related changes
1992
Involvement of two different cell death pathways in retinal atrophy of cathepsin D-deficient mice
2003
Microglia processes block the spread of damage in the brain and require functional chloride channels
2009
Lysosomal metabolism of glycoproteins
2005
Participation of Autophagy in Storage of Lysosomes in Neurons from Mouse Models of Neuronal Ceroid-Lipofuscinoses (Batten Disease)
2005
Lysosomal storage disorders
2005
Mitochondria-Anchored Receptor Atg32 Mediates Degradation of Mitochondria via Selective Autophagy
2009 StandoutNobel
Decreased proteolysis caused by protein aggregates, inclusion bodies, plaques, lipofuscin, ceroid, and ‘aggresomes’ during oxidative stress, aging, and disease
2004
CLASSIFICATION AND STAGING OF MELANOMA
1998
Chapter 15 Simultaneous Measurement of Cell Cycle and Apoptotic Cell Death
1998
Aging as a catabolic malfunction
2004
Autophagy in the Pathogenesis of Disease
2008 Standout
Batten disease: Past, present, and future
1988
Catabolism of N-glycoproteins in mammalian cells: Molecular mechanisms and genetic disorders related to the processes
2016
Gangliosides as apoptotic signals in ER stress response
2006
Understanding the Odd Science of Aging
2005 Standout
Mitochondrial Membrane Permeabilization in Cell Death
2007 Standout
Lipofuscin
2003
The comparative biology of neuromelanin and lipofuscin in the human brain
2008
Neuronal ceroid-lipofuscinoses in childhood
1988
Loss of autophagy in the central nervous system causes neurodegeneration in mice
2006 StandoutNature
Lipofuscin
2007
Autophagy in cell death: an innocent convict?
2005 Standout
Batten's disease: Clues to neuronal protein catabolism in lysosomes
2000
Repertoires of Autophagy in the Pathogenesis of Ocular Diseases
2015 Standout
4-hydroxynonenal: A specific indicator for canine neuronal-retinal ceroidosis
1988
Oxidative Stress and the Eye
2008
Protein Sequence Editing of SKN-1A/Nrf1 by Peptide:N-Glycanase Controls Proteasome Gene Expression
2019 StandoutNobel
Autophagy-related Protein 8 (Atg8) Family Interacting Motif in Atg3 Mediates the Atg3-Atg8 Interaction and Is Crucial for the Cytoplasm-to-Vacuole Targeting Pathway
2010 StandoutNobel
Chemistry and biochemistry of 4-hydroxynonenal, malonaldehyde and related aldehydes
1991 Standout
Redox Activity Within the Lysosomal Compartment: Implications for Aging and Apoptosis
2009
Oxidative Stress, Accumulation of Biological 'Garbage', and Aging
2006
Mouse model for the lysosomal disorder galactosialidosis and correction of the phenotype with overexpressing erythroid precursor cells.
1995 StandoutNobel
Association of Mutations in a Lysosomal Protein with Classical Late-Infantile Neuronal Ceroid Lipofuscinosis
1997 Science
Oxidative damage and mitochondrial decay in aging.
1994 Standout
α-d-mannosidase of Saccharomyces cerevisiae Characterization and modulation of activity
1978
Autophagy, mitochondria and oxidative stress: cross-talk and redox signalling
2011 Standout
Genes required for completion of import of proteins into the endoplasmic reticulum in yeast.
1984 StandoutNobel
The Free Radical Theory of Aging Matures
1998 Standout
Prognostic Factors Analysis of 17,600 Melanoma Patients: Validation of the American Joint Committee on Cancer Melanoma Staging System
2001 Standout
Bone marrow transplantation corrects the enzyme defect in neurons of the central nervous system in a lysosomal storage disease.
1994
Lysosomal activity regulatesCaenorhabditis elegansmitochondrial dynamics through vitamin B12 metabolism
2020 StandoutNobel
Intracellular protein topogenesis
1980 StandoutNobel
Lysosomal cathepsin D mediates endogenous mucin glycodomain catabolism in mammals
2022 StandoutNobel
Degradable Controlled-Release Polymers and Polymeric Nanoparticles: Mechanisms of Controlling Drug Release
2016 Standout
Aggregation-Induced Emission: Together We Shine, United We Soar!
2015 Standout
Mitochondrial Reactive Oxygen Species (ROS) and ROS-Induced ROS Release
2014 Standout
Fluorescence Lifetime Measurements and Biological Imaging
2010 Standout
Using the shortwave infrared to image middle ear pathologies
2016 StandoutNobel
A role in vacuolar arginine transport for yeast Btn1p and for human CLN3, the protein defective in Batten disease
2003
Early stages in the yeast secretory pathway are required for transport of carboxypeptidase Y to the vacuole
1982 StandoutNobel
Lysosomal alpha-D-mannosidase of rat liver. Purification and comparison with the golgi and cytosolic alpha-D-mannosidases
1978
How neuroinflammation contributes to neurodegeneration
2016 StandoutScience
Fluorescent Sensors for Measuring Metal Ions in Living Systems
2014 Standout
Gene dosage-dependent secretion of yeast vacuolar carboxypeptidase Y.
1986 StandoutNobel
Effect of isomers of swainsonine on glycosidase activity and glycoprotein processing
1987 StandoutNobel
Biosynthesis of N- and O-linked oligosaccharides of the low density lipoprotein receptor.
1983 StandoutNobel
Expression and Activity of L-Myc in Normal Mouse Development
1996
Invertase signal and mature sequence substitutions that delay intercompartmental transport of active enzyme.
1985 StandoutNobel
Escape of mitochondrial DNA to the nucleus in ymel yeast is mediated by vacuolar-dependent turnover of abnormal mitochondrial compartments
1998
Morphological, Biochemical and Molecular Biology Approaches for the Diagnosis of Lysosomal Storage Diseases
2000
Mutants of Saccharomyces cerevisiae with defective vacuolar function
1988 StandoutNobel
Biosynthesis of lysosomal enzymes in fibroblasts. Phosphorylation of mannose residues.
1980
Purification and characterization of alpha-D-mannosidase from rat liver golgi membranes.
1977
Advances in age pigment research
1991
Promotion of direct reprogramming by transformation-deficient Myc
2010 StandoutNobel
Biosynthesis of lysosomal enzymes in fibroblasts. Synthesis as precursors of higher molecular weight.
1980 Standout
Autophagy as a Regulated Pathway of Cellular Degradation
2000 StandoutScience
A cell nanoinjector based on carbon nanotubes
2007 StandoutNobel
A New α-d-Mannosidase Occurring in Golgi Membranes
1973
Biochemical requirements for the targeting and fusion of ER-derived transport vesicles with purified yeast Golgi membranes.
1996 StandoutNobel
Hyperspectral imaging of bruises in the SWIR spectral region
2012
Neuronal pigmented autophagic vacuoles: lipofuscin, neuromelanin, and ceroid as macroautophagic responses during aging and disease
2008
Physiology of Microglia
2011 Standout
Solubilization and purification of alpha-mannosidase, a marker enzyme of vacuolar membranes in Saccharomyces cerevisiae.
1988 StandoutNobel
Works of R.D. Jolly being referenced
Effect of cisternal sulfamidase delivery in MPS IIIA Huntaway dogs—A proof of principle study
2009
Mitochondrial ATP synthase subunit c storage in the ceroid‐lipofuscinoses (Batten disease)
1992 StandoutNobel
Comparative biology of the neuronal ceroid‐lipofuscinoses (NCL): An overview
1995
The analytical approach to the nature of lipofuscin (age pigment)
2002
Ovine ceroid-lipofuscinosis I: Lipopigment composition is indicative of a lysosomal proteinosis
1988
Lipopigments in Veterinary Pathology: Pathogenesis and Terminology
1990
Mitochondrial dysfunction in the neuronal ceroid-lipofuscinoses (Batten disease)
2002
Glycoconjugates in Storage Cytosomes from Ceroid-Lipofuscinosis (Batten’s Disease) and in Lipofuscin from Old-Age Brain
1990
Apoptosis as the Mechanism of Neurodegeneration in Batten's Disease
1996
The mannosidoses and ceroid-lipofuscinoses: Experimental studies on two types of storage disease*
1997
LIPOFUSCIN IN BOVINE MUSCLE AND BRAIN: A MODEL FOR STUDYING AGE PIGMENT
1995
Ovine ceroid-lipofuscinosis II: Pathologic changes interpreted in light of biochemical observations
1988
The neuronal ceroid‐Iipofuscinoses (Batten disease): comparative aspects
1995
Sheep and other animals with ceroid‐lipofuscinoses: Their relevance to Batten disease
1992
Analysis of dolichyl pyrophosphoryl oligosaccharides in purified storage cytosomes from ovine ceroid-lipofuscinosis
1989
Anomalies of Mitochondrial ATP Synthase Regulation in Four Different Types of Neuronal Ceroid Lipofuscinosis
1999
Lysosomal Storage Diseases of Animals: An Essay in Comparative Pathology
1997
Autofluorescence emission spectra of neuronal lipopigment in animal and human ceroidoses (ceroid-lipofuscinoses)
1982
OVINE CEROID‐LIPOFUSCINOSIS: A MODEL OF BATTEN'S DISEASE
1980
Accumulation of sphingolipid activator proteins (SAPs) A and D in granular osmiophilic deposits in miniature Schnauzer dogs with ceroid‐lipofuscinosis
1997
Ovine neuronal ceroid lipofuscinosis: a large animal model syntenic with the human neuronal ceroid lipofuscinosis variant CLN6.
1998
Deficiency of α-mannosidase in Angus cattle. An inherited lysosomal storage disease
1972
Ceroid, lipofuscin and the ceroid‐lipofuscinoses (Batten Disease)
1993
Characterization of the expressed genes for subunit c of mitochondrial ATP synthase in sheep with ceroid lipofuscinosis
1993 StandoutNobel
A Urinary Pentasaccharide in Bovine Mannosidosis
1975
The sequence of the major protein stored in ovine ceroid lipofuscinosis is identical with that of the dicyclohexylcarbodiimide-reactive proteolipid of mitochondrial ATP synthase
1990 StandoutNobel
Enzyme Replacement Therapy—An Experiment of Nature in a Chimeric Mannosidosis Calf
1976
Mannosidosis in Angus cattle. The enzymic defect
1974
The catabolism of mammalian glycoproteins. Comparison of the storage products in bovine, feline and human mannosidosis
1983
The objective interpretation of histopathological data: An application to the ageing of ovine bruises
1986
Ceroid lipofuscinosis in sheep. I. Bis(monoacylglycero)phosphate, dolichol, ubiquinone, phospholipids, fatty acids, and fluorescence in liver lipopigment lipids.
1986
The nature of the residual α-mannosidase in plasma in bovine mannosidosis
1976
Ovine Ceroid Lipofuscinosis
1989
Ceroid lipofuscinosis in sheep. II. The major component of the lipopigment in liver, kidney, pancreas, and brain is low molecular weight protein.
1986
Bovine ceroid-lipofuscinosis (Batten's disease): The major component stored is the DCCD-reactive proteolipid, subunit c, of mitochondrial ATP synthase
1991