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Targeting Cystathionine Beta-Synthase Misfolding in Homocystinuria by Small Ligands: State of the Art and Future Directions

Current drug targets, 2016-01, Vol.17 (13), p.1455

2016

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Structural insight into the molecular mechanism of allosteric activation of human cystathionine β-synthase by S-adenosylmethionine

Proceedings of the National Academy of Sciences - PNAS, 2014-09, Vol.111 (37), p.E3845-E3852

2014

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Human cystathionine β-synthase (CBS) contains two classes of binding sites for S-adenosylmethionine (SAM): complex regulation of CBS activity and stability by SAM

Biochemical journal, 2013-01, Vol.449 (1), p.109-121

2013

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Structural basis of regulation and oligomerization of human cystathionine β-synthase, the central enzyme of transsulfuration

Proceedings of the National Academy of Sciences - PNAS, 2013-10, Vol.110 (40), p.E3790-E3799

2013

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Domain organization, catalysis and regulation of eukaryotic cystathionine beta-synthases

PloS one, 2014-08, Vol.9 (8), p.e105290

2014

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Identification of Cystathionine β-Synthase Inhibitors Using a Hydrogen Sulfide Selective Probe

Angewandte Chemie (International ed.), 2013-04, Vol.52 (17), p.4641-4644

2013

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Identification of Cystathionine [beta]-Synthase Inhibitors Using a Hydrogen Sulfide Selective Probe

Angewandte Chemie (International ed.), 2013-04, Vol.52 (17), p.4641-4644

International ed. in English, 2013

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Cystathionine beta-synthase null homocystinuric mice fail to exhibit altered hemostasis or lowering of plasma homocysteine in response to betaine treatment

Molecular genetics and metabolism, 2010-10, Vol.101 (2-3), p.163-171

2010

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Brain phenotype of transgenic mice overexpressing cystathionine β-synthase

PloS one, 2012-01, Vol.7 (1), p.e29056-e29056

2012

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Import of TAT-Conjugated Propionyl Coenzyme A Carboxylase Using Models of Propionic Acidemia

Molecular and cellular biology, 2018-03, Vol.38 (6)

2018

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Enzyme replacement therapy prevents loss of bone and fat mass in murine homocystinuria

Human mutation, 2018-02, Vol.39 (2), p.210-218

2018

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Kinetic stability of cystathionine beta-synthase can be modulated by structural analogs of S-adenosylmethionine: Potential approach to pharmacological chaperone therapy for homocystinuria

Biochimie, 2016-07, Vol.126, p.6-13

2016

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Behavior, body composition, and vascular phenotype of homocystinuric mice on methionine‐restricted diet or enzyme replacement therapy

The FASEB journal, 2019-11, Vol.33 (11), p.12477-12486

2019

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Rescue of Cystathionine β-Synthase (CBS) Mutants with Chemical Chaperones: PURIFICATION AND CHARACTERIZATION OF EIGHT CBS MUTANT ENZYMES

The Journal of biological chemistry, 2010-05, Vol.285 (21), p.15866-15873

2010

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Biogenesis of Hydrogen Sulfide and Thioethers by Cystathionine Beta-Synthase

Antioxidants & redox signaling, 2018-02, Vol.28 (4), p.311

2018

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Cystathionine beta-synthase: structure, function, regulation, and location of homocystinuria-causing mutations

The Journal of biological chemistry, 2004-07, Vol.279 (29), p.29871-29874

2004

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Engineering and Characterization of an Enzyme Replacement Therapy for Classical Homocystinuria

Biomacromolecules, 2017-06, Vol.18 (6), p.1747-1761

2017

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Enzyme replacement with PEGylated cystathionine β-synthase ameliorates homocystinuria in murine model

The Journal of clinical investigation, 2016-06, Vol.126 (6), p.2372-2384

2016

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Enzyme Replacement Therapy Ameliorates Multiple Symptoms of Murine Homocystinuria

Molecular therapy, 2018-03, Vol.26 (3), p.834-844

2018

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Enzyme replacement prevents neonatal death, liver damage, and osteoporosis in murine homocystinuria

The FASEB journal, 2017-12, Vol.31 (12), p.5495-5506

2017

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Long-term sex-biased correction of circulating propionic acidemia disease markers by adeno-associated virus vectors

Human gene therapy, 2015-03, Vol.26 (3), p.153

2015

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Characterization of Four Variant Forms of Human Propionyl-CoA Carboxylase Expressed in Escherichia coli

The Journal of biological chemistry, 2005-07, Vol.280 (30), p.27719-27727

2005

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Folding and activity of mutant cystathionine β-synthase depends on the position and nature of the purification tag: Characterization of the R266K CBS mutant

Protein expression and purification, 2012-04, Vol.82 (2), p.317-324

2012

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Pharmacokinetics and pharmacodynamics of PEGylated truncated human cystathionine beta-synthase for treatment of homocystinuria

Life sciences (1973), 2018-05, Vol.200, p.15-25

2018

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Crystal structure of cystathionine β-synthase from honeybee Apis mellifera

Journal of structural biology, 2018-04, Vol.202 (1), p.82-93

2018

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Targeting Cystathionine Beta-Synthase Misfolding in Homocystinuria by Small Ligands: State of the Art and Future Directions

Structural insight into the molecular mechanism of allosteric activation of human cystathionine β-synthase by S-adenosylmethionine

Human cystathionine β-synthase (CBS) contains two classes of binding sites for S-adenosylmethionine (SAM): complex regulation of CBS activity and stability by SAM

Structural basis of regulation and oligomerization of human cystathionine β-synthase, the central enzyme of transsulfuration

Domain organization, catalysis and regulation of eukaryotic cystathionine beta-synthases

Identification of Cystathionine β-Synthase Inhibitors Using a Hydrogen Sulfide Selective Probe

Identification of Cystathionine [beta]-Synthase Inhibitors Using a Hydrogen Sulfide Selective Probe

Cystathionine beta-synthase null homocystinuric mice fail to exhibit altered hemostasis or lowering of plasma homocysteine in response to betaine treatment

Brain phenotype of transgenic mice overexpressing cystathionine β-synthase

Import of TAT-Conjugated Propionyl Coenzyme A Carboxylase Using Models of Propionic Acidemia

Enzyme replacement therapy prevents loss of bone and fat mass in murine homocystinuria

Kinetic stability of cystathionine beta-synthase can be modulated by structural analogs of S-adenosylmethionine: Potential approach to pharmacological chaperone therapy for homocystinuria

Behavior, body composition, and vascular phenotype of homocystinuric mice on methionine‐restricted diet or enzyme replacement therapy

Rescue of Cystathionine β-Synthase (CBS) Mutants with Chemical Chaperones: PURIFICATION AND CHARACTERIZATION OF EIGHT CBS MUTANT ENZYMES

Biogenesis of Hydrogen Sulfide and Thioethers by Cystathionine Beta-Synthase

Cystathionine beta-synthase: structure, function, regulation, and location of homocystinuria-causing mutations

Engineering and Characterization of an Enzyme Replacement Therapy for Classical Homocystinuria

Enzyme replacement with PEGylated cystathionine β-synthase ameliorates homocystinuria in murine model

Enzyme Replacement Therapy Ameliorates Multiple Symptoms of Murine Homocystinuria

Enzyme replacement prevents neonatal death, liver damage, and osteoporosis in murine homocystinuria

Long-term sex-biased correction of circulating propionic acidemia disease markers by adeno-associated virus vectors

Characterization of Four Variant Forms of Human Propionyl-CoA Carboxylase Expressed in Escherichia coli

Folding and activity of mutant cystathionine β-synthase depends on the position and nature of the purification tag: Characterization of the R266K CBS mutant

Pharmacokinetics and pharmacodynamics of PEGylated truncated human cystathionine beta-synthase for treatment of homocystinuria

Crystal structure of cystathionine β-synthase from honeybee Apis mellifera

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