Standout Papers
Citation Impact
Citing Papers
The sequences of human and bovine genes of the phosphate carrier from mitochondria contain evidence of alternatively spliced forms.
1994 StandoutNobel
Chapter 5 Assaying mitochondrial respiratory complex activity in mitochondria isolated from human cells and tissues
2001
Sequences of members of the human gene family for the c subunit of mitochondrial ATP synthase
1993 StandoutNobel
Short-Term Effects of Nose-Only Cigarette Smoke Exposure on Glutathione Redox Homeostasis, Cytochrome P450 1A1/2 and Respiratory Enzyme Activities in Mice Tissues
2013 Standout
A Family of Basic Amino Acid Transporters of the Vacuolar Membrane from Saccharomyces cerevisiae
2004 StandoutNobel
Circulating mitochondrial DAMPs cause inflammatory responses to injury
2010 StandoutNature
The mitochondrial UPR: mechanisms, physiological functions and implications in ageing
2017
Mitochondrial diabetes mellitus: a review
1995
Beyond Cholesterol
1989 Standout
The First Nuclear-Encoded Complex I Mutation in a Patient with Leigh Syndrome
1998
Autophagy Is Disrupted in a Knock-in Mouse Model of Juvenile Neuronal Ceroid Lipofuscinosis
2006
Mitochondrial Dysfunction and Neurodegeneration in Lysosomal Storage Disorders
2017
The deoxyguanosine kinase gene is mutated in individuals with depleted hepatocerebral mitochondrial DNA
2001
Involvement of two different cell death pathways in retinal atrophy of cathepsin D-deficient mice
2003
Mitochondria: In Sickness and in Health
2012 Standout
Participation of Autophagy in Storage of Lysosomes in Neurons from Mouse Models of Neuronal Ceroid-Lipofuscinoses (Batten Disease)
2005
Lysosomal storage disorders
2005
Mitochondrial Respiratory Chain Deficiency inCaenorhabditis elegans Results in Developmental Arrest and Increased Life Span
2001
TRP channels as cellular sensors
2003 StandoutNature
Autophagy in the Pathogenesis of Disease
2008 Standout
SURF1, encoding a factor involved in the biogenesis of cytochrome c oxidase, is mutated in Leigh syndrome
1998
Immunohistochemical localization of growth factors in fetal wound healing
1991
Clinical features of melas and mitochondrial DNA mutations
1995
RPE lipofuscin and its role in retinal pathobiology
2005
Wnt/β-Catenin Signaling in Development and Disease
2006 Standout
Loss of autophagy in the central nervous system causes neurodegeneration in mice
2006 StandoutNature
The mitochondrial genome: structure, transcription, translation and replication
1999
A systematic RNAi screen identifies a critical role for mitochondria in C. elegans longevity
2002 StandoutNobel
Batten's disease: Clues to neuronal protein catabolism in lysosomes
2000
Repertoires of Autophagy in the Pathogenesis of Ocular Diseases
2015 Standout
Mucolipidosis type IV is caused by mutations in a gene encoding a novel transient receptor potential channel
2000
Wnt signalling in stem cells and cancer
2005 StandoutNature
Identification of a prion protein epitope modulating transmission of bovine spongiform encephalopathy prions to transgenic mice
1997 StandoutNobel
Coated pits, coated vesicles, and receptor-mediated endocytosis
1979 StandoutNatureNobel
Demonstration of low density lipoprotein receptors in mouse teratocarcinoma stem cells and description of a method for producing receptor-deficient mutant mice
1979 StandoutNobel
In Situ Detection of β-Galactosidase in Lenses of Transgenic Mice with a γ-Crystallin/ lacZ Gene
1987 Science
Genetics of the LDL receptor: Evidence that the mutations affecting binding and internalization are allelic
1977 StandoutNobel
OVINE CEROID‐LIPOFUSCINOSIS: A MODEL OF BATTEN'S DISEASE
1980
Lysosomal activity regulatesCaenorhabditis elegansmitochondrial dynamics through vitamin B12 metabolism
2020 StandoutNobel
Transient activation of β-catenin signalling in adult mouse epidermis is sufficient to induce new hair follicles but continuous activation is required to maintain hair follicle tumours
2004
Localization of low density lipoprotein receptors on plasma membrane of normal human fibroblasts and their absence in cells from a familial hypercholesterolemia homozygote.
1976 StandoutNobel
Lysosomal cathepsin D mediates endogenous mucin glycodomain catabolism in mammals
2022 StandoutNobel
Aggregation-Induced Emission: Together We Shine, United We Soar!
2015 Standout
Enzyme deficiency in cholesteryl ester storage disease
1972
Immunocytochemical visualization of coated pits and vesicles in human fibroblasts: Relation to low density lipoprotein receptor distribution
1978 StandoutNobel
Receptor-dependent hydrolysis of cholesteryl esters contained in plasma low density lipoprotein.
1975 StandoutNobel
A role in vacuolar arginine transport for yeast Btn1p and for human CLN3, the protein defective in Batten disease
2003
Evidence for regulation of 3-hydroxy-3-methylglutaryl coenzyme A reductase activity and cholesterol synthesis in nonhepatic tissues of rat.
1976 StandoutNobel
Restoration of a regulatory response to low density lipoprotein in acid lipase-deficient human fibroblasts.
1976
The Caenorhabditis elegans ARIP-4 DNA helicase couples mitochondrial surveillance to immune, detoxification, and antiviral pathways
2022 StandoutNobel
Wound Healing--Aiming for Perfect Skin Regeneration
1997 StandoutScience
Mitochondrial Diseases in Man and Mouse
1999 StandoutScience
Replacement of endogenous cholesteryl esters of low density lipoprotein with exogenous cholesteryl linoleate. Reconstitution of a biologically active lipoprotein particle.
1978 StandoutNobel
Prions
1998 StandoutNobel
Role of the coated endocytic vesicle in the uptake of receptor-bound low density lipoprotein in human fibroblasts
1977 StandoutNobel
Solubilization of the low density lipoprotein receptor
1979 StandoutNobel
Characterization of the expressed genes for subunit c of mitochondrial ATP synthase in sheep with ceroid lipofuscinosis
1993 StandoutNobel
A mutation that impairs the ability of lipoprotein receptors to localise in coated pits on the cell surface of human fibroblasts
1977 StandoutNatureNobel
Squalene synthetase activity in human fibroblasts: Regulation via the low density lipoprotein receptor
1979 StandoutNobel
Heterozygous familial hypercholesterolemia: Failure of normal allele to compensate for mutant allele at a regulated genetic locus
1976 StandoutNobel
Morphological, Biochemical and Molecular Biology Approaches for the Diagnosis of Lysosomal Storage Diseases
2000
CHOLESTEROL METABOLISM IN THE MACROPHAGE
1972
Inhibition of cholesteryl ester formation in human fibroblasts by an analogue of 7-ketocholesterol and by progesterone
1978 StandoutNobel
Replacement of neutral lipids of low density lipoprotein with esters of long chain unsaturated fatty acids.
1979 StandoutNobel
Deficient Activity of Hepatic Acid Lipase in Cholesterol Ester Storage Disease
1972 Science
Receptor-Mediated Control of Cholesterol Metabolism
1976 StandoutScienceNobel
Precise targeting of the pathology of the sialoglycoprotein, PrP, and vacuolar degeneration in mouse scrapie
1989
Degradation of cationized low density lipoprotein and regulation of cholesterol metabolism in homozygous familial hypercholesterolemia fibroblasts.
1976 StandoutNobel
Assay, Kinetics, and Lysosomal Localization of an Acid Cholesteryl Esterase in Rabbit Aortic Smooth Muscle Cells
1974 StandoutNobel
Binding site on macrophages that mediates uptake and degradation of acetylated low density lipoprotein, producing massive cholesterol deposition
1979 StandoutNobel
Autophagy as a Regulated Pathway of Cellular Degradation
2000 StandoutScience
Induction of 3-hydroxy-3-methylglutaryl coenzyme A reductase activity in human fibroblasts incubated with compactin (ML-236B), a competitive inhibitor of the reductase
1978 StandoutNobel
Receptor-mediated uptake of low density lipoprotein reconstituted with 25-hydroxycholesteryl oleate suppresses 3-hydroxy-3-methylglutaryl-coenzyme A reductase and inhibits growth of human fibroblasts
1978 StandoutNobel
Inherited prion diseases.
1994 StandoutNobel
Introduction of a lacZ reporter gene into the mouse int-2 locus by homologous recombination.
1990 StandoutNobel
Lipoprotein-mediated regulation of 3-hydroxy-3-methylglutaryl coenzyme A reductase activity and cholesteryl ester metabolism in the adrenal gland of the rat.
1977 StandoutNobel
Prion protein preamyloid and amyloid deposits in Gerstmann-Sträussler-Scheinker disease, Indiana kindred.
1992 StandoutNobel
Works of Brian Lake being referenced
Targeted Disruption of the Cln3 Gene Provides a Mouse Model for Batten Disease
1999
Mitochondrial ATP synthase subunit c storage in the ceroid‐lipofuscinoses (Batten disease)
1992 StandoutNobel
Mucolipidosis type IV Presentation of a mild variant
1992
Bone Marrow Transplantation in Late Infantile Batten Disease and Juvenile Batten Disease
1997
A new point mutation associated with mitochondrial encephalomyopathy
1993
Deficiency of respiratory chain complex I is a common cause of leigh disease
1996
Neutral lipid storage disease. Case report and lipid studies
1994
A Murine Model for Juvenile NCL: Gene Targeting of MouseCln3
1999
Liver failure associated with mitochondrial DNA depletion
1998
Lysosomal storage of subunit c of mitochondrial ATP synthase in Batten's disease (ceroid-lipofuscinosis)
1991
Histochemistry in Pathology
1990
Early-juvenile Batten's disease — A recognisable sub-group distinct from other forms of Batten's disease
1978
An improved method for the detection of ?-galactosidase activity, and its application to G Mi -gangliosidosis and mucopolysaccharidosis
1974
Wolman's disease: Deficiency of E600-resistant acid esterase activity with storage of lipids in lysosomes
1970
Deficiency of an Acid Lipase in Wolman's Disease
1969 Nature
Immunocytochemistry. Modern methods and applications
1988