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Pharmacological Chaperones as Therapeutics for Lysosomal Storage Diseases

Journal of medicinal chemistry, 2013-04, Vol.56 (7), p.2705-2725

2013

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Oral Migalastat HCl Leads to Greater Systemic Exposure and Tissue Levels of Active α-Galactosidase A in Fabry Patients when Co-Administered with Infused Agalsidase

PloS one, 2015-08, Vol.10 (8), p.e0134341-e0134341

2015

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The pharmacological chaperone AT2220 increases the specific activity and lysosomal delivery of mutant acid alpha-glucosidase, and promotes glycogen reduction in a transgenic mouse model of Pompe disease

PloS one, 2014-07, Vol.9 (7), p.e102092

2014

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In vivo editing of lung stem cells for durable gene correction in mice

Science (American Association for the Advancement of Science), 2024-06, Vol.384 (6701), p.1196-1202

2024

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A GCase Chaperone Improves Motor Function in a Mouse Model of Synucleinopathy

Neurotherapeutics, 2014-10, Vol.11 (4), p.840-856

2014

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The Iminosugar Isofagomine Increases the Activity of N370S Mutant Acid β-Glucosidase in Gaucher Fibroblasts by Several Mechanisms

Proceedings of the National Academy of Sciences - PNAS, 2006-09, Vol.103 (37), p.13813-13818

2006

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pharmacological chaperone isofagomine increases the activity of the Gaucher disease L444P mutant form of β-glucosidase

The FEBS journal, 2010-04, Vol.277 (7), p.1618-1638

2010

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Lysosomal dysfunction in a mouse model of Sandhoff disease leads to accumulation of ganglioside-bound amyloid-β peptide

The Journal of neuroscience, 2012-04, Vol.32 (15), p.5223-5236

2012

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Structure of acid β-glucosidase with pharmacological chaperone provides insight into Gaucher disease

Nature chemical biology, 2007-02, Vol.3 (2), p.101-107

2007

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Characterization of two molluscan crystal-modulating biomineralization proteins and identification of putative mineral binding domains

Biopolymers, 2003-12, Vol.70 (4), p.522-533

2003

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Cellular and tissue localization of globotriaosylceramide in Fabry disease

Virchows Archiv : an international journal of pathology, 2007-10, Vol.451 (4), p.823-834

2007

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Bringing dead proteins back to life

eLife, 2014-02, Vol.3, p.e02189-e02189

2014

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Structural characterization of the N-terminal mineral modification domains from the molluscan crystal-modulating biomineralization proteins, AP7 and AP24

Biopolymers, 2004-08, Vol.74 (5), p.363-376

2004

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Model peptide studies of sequence regions in the elastomeric biomineralization protein, Lustrin A. I. The C-domain consensus-PG-, -NVNCT-motif

Biopolymers, 2002-05, Vol.63 (6), p.358-369

2002

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Characterization of a Ca(II)-, Mineral-Interactive Polyelectrolyte Sequence from the Adhesive Elastomeric Biomineralization Protein Lustrin A

Langmuir, 2003-10, Vol.19 (22), p.9373-9381

2003

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New pathway links γ-secretase to inflammation and memory while sparing notch

Annals of neurology, 2011-01, Vol.69 (1), p.5-7

2011

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Identification of a "glycine-loop"-like coiled structure in the 34 AA Pro,Gly,Met repeat domain of the biomineral-associated protein, PM27

Biopolymers, 2002-12, Vol.65 (5), p.362-372

2002

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New pathway links [gamma]-secretase to inflammation and memory while sparing notch

Annals of neurology, 2011-01, Vol.69 (1), p.5

2011

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Extracellular Matrix Assembly in Diatoms (Bacillariophyceae): I. A Model of Adhesives Based on Chemical Characterization and Localization of Polysaccharides from the Marine Diatom Achnanthes longipes and Other Diatoms

Plant physiology (Bethesda), 1997-04, Vol.113 (4), p.1059-1069

1997

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A STUDY OF CELL WALL AND FLAGELLA FORMATION DURING CELL DIVISION IN THE SCALY GREEN ALGA, SCHERFFELIA DUBIA (CHLOROPHYTA)

Journal of phycology, 2004-10, Vol.40 (5), p.895-910

2004

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Structural Analyses of Polyelectrolyte Sequence Domains within the Adhesive Elastomeric Biomineralization Protein Lustrin A

Langmuir, 2002-12, Vol.18 (25), p.9901-9906

2002

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Structure-Function Studies of the Lustrin A Polyelectrolyte Domains, RKSY and D4

Connective tissue research, 2003, Vol.44 (1), p.10-15

2003

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Characterization of a Ca(II)-, Mineral-Interactive Polyelectrolyte Sequence from the Adhesive Elastomeric Biomineralization Protein, Lustrin A

Langmuir, 2004-01, Vol.20 (1), p.277-277

2004

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Extracellular matrix assembly in diatoms (Bacillariophyceae). III. Organization of fucoglucuronogalactans within the adhesive stalks of Achnanthes longipes

Plant physiology (Bethesda), 1998-04, Vol.116 (4), p.1431-1441

1998

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Pharmacological Chaperones as Therapeutics for Lysosomal Storage Diseases

Oral Migalastat HCl Leads to Greater Systemic Exposure and Tissue Levels of Active α-Galactosidase A in Fabry Patients when Co-Administered with Infused Agalsidase

The pharmacological chaperone AT2220 increases the specific activity and lysosomal delivery of mutant acid alpha-glucosidase, and promotes glycogen reduction in a transgenic mouse model of Pompe disease

In vivo editing of lung stem cells for durable gene correction in mice

A GCase Chaperone Improves Motor Function in a Mouse Model of Synucleinopathy

The Iminosugar Isofagomine Increases the Activity of N370S Mutant Acid β-Glucosidase in Gaucher Fibroblasts by Several Mechanisms

pharmacological chaperone isofagomine increases the activity of the Gaucher disease L444P mutant form of β-glucosidase

Lysosomal dysfunction in a mouse model of Sandhoff disease leads to accumulation of ganglioside-bound amyloid-β peptide

Structure of acid β-glucosidase with pharmacological chaperone provides insight into Gaucher disease

Characterization of two molluscan crystal-modulating biomineralization proteins and identification of putative mineral binding domains

Cellular and tissue localization of globotriaosylceramide in Fabry disease

Bringing dead proteins back to life

Structural characterization of the N-terminal mineral modification domains from the molluscan crystal-modulating biomineralization proteins, AP7 and AP24

Model peptide studies of sequence regions in the elastomeric biomineralization protein, Lustrin A. I. The C-domain consensus-PG-, -NVNCT-motif

Characterization of a Ca(II)-, Mineral-Interactive Polyelectrolyte Sequence from the Adhesive Elastomeric Biomineralization Protein Lustrin A

New pathway links γ-secretase to inflammation and memory while sparing notch

Identification of a "glycine-loop"-like coiled structure in the 34 AA Pro,Gly,Met repeat domain of the biomineral-associated protein, PM27

New pathway links [gamma]-secretase to inflammation and memory while sparing notch

Extracellular Matrix Assembly in Diatoms (Bacillariophyceae): I. A Model of Adhesives Based on Chemical Characterization and Localization of Polysaccharides from the Marine Diatom Achnanthes longipes and Other Diatoms

A STUDY OF CELL WALL AND FLAGELLA FORMATION DURING CELL DIVISION IN THE SCALY GREEN ALGA, SCHERFFELIA DUBIA (CHLOROPHYTA)

Structural Analyses of Polyelectrolyte Sequence Domains within the Adhesive Elastomeric Biomineralization Protein Lustrin A

Structure-Function Studies of the Lustrin A Polyelectrolyte Domains, RKSY and D4

Characterization of a Ca(II)-, Mineral-Interactive Polyelectrolyte Sequence from the Adhesive Elastomeric Biomineralization Protein, Lustrin A

Extracellular matrix assembly in diatoms (Bacillariophyceae). III. Organization of fucoglucuronogalactans within the adhesive stalks of Achnanthes longipes

Oral Migalastat HCl Leads to Greater Systemic Exposure and Tissue Levels of Active [alpha]-Galactosidase A in Fabry Patients when Co-Administered with Infused Agalsidase

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