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Variation in the Biochemical/Biophysical Properties of Mutant Superoxide Dismutase 1 Enzymes and the Rate of Disease Progression in Familial Amyotrophic Lateral Sclerosis Kindreds

Human molecular genetics, 1999-08, Vol.8 (8), p.1451-1460

1999

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Intranuclear Inclusions and Neuritic Aggregates in Transgenic Mice Expressing a Mutant N-Terminal Fragment of Huntingtin

Human molecular genetics, 1999-03, Vol.8 (3), p.397-407

1999

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Alzheimer's-Type Amyloidosis in Transgenic Mice Impairs Survival of Newborn Neurons Derived from Adult Hippocampal Neurogenesis

The Journal of neuroscience, 2007-06, Vol.27 (25), p.6771-6780

2007

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ALZHEIMER'S DISEASE: Genetic Studies and Transgenic Models

Annual review of genetics, 1998-01, Vol.32 (1), p.461-493

1998

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ALS-Linked SOD1 Mutant G85R Mediates Damage to Astrocytes and Promotes Rapidly Progressive Disease with SOD1-Containing Inclusions

Neuron (Cambridge, Mass.), 1997-02, Vol.18 (2), p.327-338

1997

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Evidence for synthesis of scrapie prion proteins in the endocytic pathway

The Journal of biological chemistry, 1992-08, Vol.267 (23), p.16188-16199

1992

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Truncated N-Terminal Fragments of Huntingtin with Expanded Glutamine Repeats form Nuclear and Cytoplasmic Aggregates in Cell Culture

Human molecular genetics, 1998-05, Vol.7 (5), p.783-790

1998

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Superoxide Dismutase 1 with Mutations Linked to Familial Amyotrophic Lateral Sclerosis Possesses Significant Activity

Proceedings of the National Academy of Sciences - PNAS, 1994-08, Vol.91 (17), p.8292-8296

1994

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Mutations Associated with Amyotrophic Lateral Sclerosis Convert Superoxide Dismutase from an Antiapoptotic Gene to a Proapoptotic Gene: Studies in Yeast and Neural Cells

Proceedings of the National Academy of Sciences - PNAS, 1995-03, Vol.92 (7), p.3024-3028

1995

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Superoxide Dismutase is an Abundant Component in Cell Bodies, Dendrites, and Axons of Motor Neurons and in a Subset of Other Neurons

Proceedings of the National Academy of Sciences - PNAS, 1995-02, Vol.92 (4), p.954-958

1995

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Scrapie-infected murine neuroblastoma cells produce protease-resistant prion proteins

Journal of Virology, 1988-05, Vol.62 (5), p.1558-1564

1988

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Decreased expression of striatal signaling genes in a mouse model of Huntington's disease

Human molecular genetics, 2000-05, Vol.9 (9), p.1259-1271

2000

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Rapid anterograde axonal transport of the cellular prion glycoprotein in the peripheral and central nervous systems

The Journal of biological chemistry, 1994-05, Vol.269 (20), p.14711-14714

1994

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Scrapie prion protein contains a phosphatidylinositol glycolipid

Cell, 1987-10, Vol.51 (2), p.229-240

1987

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Scrapie and cellular prion proteins differ in their kinetics of synthesis and topology in cultured cells

The Journal of cell biology, 1990-03, Vol.110 (3), p.743-752

1990

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Accelerated Amyloid Deposition in the Brains of Transgenic Mice Coexpressing Mutant Presenilin 1 and Amyloid Precursor Proteins

Neuron (Cambridge, Mass.), 1997-10, Vol.19 (4), p.939-945

1997

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Synthesis and trafficking of prion proteins in cultured cells

Molecular biology of the cell, 1992-08, Vol.3 (8), p.851-863

1992

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Effects of PS1 deficiency on membrane protein trafficking in neurons

Neuron (Cambridge, Mass.), 1998-11, Vol.21 (5), p.1213-1221

1998

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Creatine Increases Survival and Delays Motor Symptoms in a Transgenic Animal Model of Huntington's Disease

Neurobiology of disease, 2001-06, Vol.8 (3), p.479-491

2001

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Distinct Behavioral and Neuropathological Abnormalities in Transgenic Mouse Models of HD and DRPLA

Neurobiology of disease, 2001-06, Vol.8 (3), p.405-418

2001

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Genetic Neurodegenerative Diseases: The Human Illness and Transgenic Models

Science (American Association for the Advancement of Science), 1998-11, Vol.282 (5391), p.1079-1083

1998

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An adverse property of a familial ALS-linked SOD1 mutation causes motor neuron disease characterized by vacuolar degeneration of mitochondria

Neuron (Cambridge, Mass.), 1995-06, Vol.14 (6), p.1105-1116

1995

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Endoproteolysis of Presenilin 1 and Accumulation of Processed Derivatives In Vivo

Neuron (Cambridge, Mass.), 1996-07, Vol.17 (1), p.181-190

1996

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Differential release of cellular and scrapie prion proteins from cellular membranes by phosphatidylinositol-specific phospholipase C

Biochemistry (Easton), 1990-06, Vol.29 (22), p.5405-5412

1990

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Acquisition of Protease Resistance by Prion Proteins in Scrapie-Infected Cells does not Require Asparagine-Linked Glycosylation

Proceedings of the National Academy of Sciences - PNAS, 1990-11, Vol.87 (21), p.8262-8266

1990

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Variation in the Biochemical/Biophysical Properties of Mutant Superoxide Dismutase 1 Enzymes and the Rate of Disease Progression in Familial Amyotrophic Lateral Sclerosis Kindreds

Intranuclear Inclusions and Neuritic Aggregates in Transgenic Mice Expressing a Mutant N-Terminal Fragment of Huntingtin

Alzheimer's-Type Amyloidosis in Transgenic Mice Impairs Survival of Newborn Neurons Derived from Adult Hippocampal Neurogenesis

ALZHEIMER'S DISEASE: Genetic Studies and Transgenic Models

ALS-Linked SOD1 Mutant G85R Mediates Damage to Astrocytes and Promotes Rapidly Progressive Disease with SOD1-Containing Inclusions

Evidence for synthesis of scrapie prion proteins in the endocytic pathway

Truncated N-Terminal Fragments of Huntingtin with Expanded Glutamine Repeats form Nuclear and Cytoplasmic Aggregates in Cell Culture

Superoxide Dismutase 1 with Mutations Linked to Familial Amyotrophic Lateral Sclerosis Possesses Significant Activity

Mutations Associated with Amyotrophic Lateral Sclerosis Convert Superoxide Dismutase from an Antiapoptotic Gene to a Proapoptotic Gene: Studies in Yeast and Neural Cells

Superoxide Dismutase is an Abundant Component in Cell Bodies, Dendrites, and Axons of Motor Neurons and in a Subset of Other Neurons

Scrapie-infected murine neuroblastoma cells produce protease-resistant prion proteins

Decreased expression of striatal signaling genes in a mouse model of Huntington's disease

Rapid anterograde axonal transport of the cellular prion glycoprotein in the peripheral and central nervous systems

Scrapie prion protein contains a phosphatidylinositol glycolipid

Scrapie and cellular prion proteins differ in their kinetics of synthesis and topology in cultured cells

Accelerated Amyloid Deposition in the Brains of Transgenic Mice Coexpressing Mutant Presenilin 1 and Amyloid Precursor Proteins

Synthesis and trafficking of prion proteins in cultured cells

Effects of PS1 deficiency on membrane protein trafficking in neurons

Creatine Increases Survival and Delays Motor Symptoms in a Transgenic Animal Model of Huntington's Disease

Distinct Behavioral and Neuropathological Abnormalities in Transgenic Mouse Models of HD and DRPLA

Genetic Neurodegenerative Diseases: The Human Illness and Transgenic Models

An adverse property of a familial ALS-linked SOD1 mutation causes motor neuron disease characterized by vacuolar degeneration of mitochondria

Endoproteolysis of Presenilin 1 and Accumulation of Processed Derivatives In Vivo

Differential release of cellular and scrapie prion proteins from cellular membranes by phosphatidylinositol-specific phospholipase C

Acquisition of Protease Resistance by Prion Proteins in Scrapie-Infected Cells does not Require Asparagine-Linked Glycosylation

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