Vascular complications of cystathionine β-synthase deficiency: Future directions for homocysteine-to-hydrogen sulfide research

RS Beard Jr, SE Bearden - American Journal of Physiology …, 2011 - journals.physiology.org
Homocysteine (Hcy), a cardiovascular and neurovascular disease risk factor, is converted to
hydrogen sulfide (H2S) through the transsulfuration pathway. H2S has attracted …

Imbalance of Homocysteine and H2S: Significance, Mechanisms, and Therapeutic Promise in Vascular Injury

Q Yang, GW He - Oxidative Medicine and Cellular Longevity, 2019 - Wiley Online Library
While the role of hyperhomocysteinemia in cardiovascular pathogenesis continuously draws
attention, deficiency of hydrogen sulfide (H2S) has been growingly implicated in …

[HTML][HTML] Cystathionine β-synthase regulates endothelial function via protein S-sulfhydration

S Saha, PK Chakraborty, X Xiong, SKD Dwivedi… - The FASEB …, 2016 - ncbi.nlm.nih.gov
Deficiencies of the human cystathionine β-synthase (CBS) enzyme are characterized by a
plethora of vascular disorders and hyperhomocysteinemia. However, several clinical trials …

Extracellular transsulfuration generates hydrogen sulfide from homocysteine and protects endothelium from redox stress

SE Bearden, RS Beard Jr… - American Journal of …, 2010 - journals.physiology.org
Homocysteine, a cardiovascular and neurocognitive disease risk factor, is converted to
hydrogen sulfide, a cardiovascular and neuronal protectant, through the transsulfuration …

[HTML][HTML] H2S biogenesis by human cystathionine γ-lyase leads to the novel sulfur metabolites lanthionine and homolanthionine and is responsive to the grade of …

T Chiku, D Padovani, W Zhu, S Singh, V Vitvitsky… - Journal of Biological …, 2009 - ASBMB
Although there is a growing recognition of the significance of hydrogen sulfide (H 2 S) as a
biological signaling molecule involved in vascular and nervous system functions, its …

[HTML][HTML] Homocysteine: a sulph'rous fire

KA Hajjar - The Journal of Clinical Investigation, 2001 - Am Soc Clin Investig
HC metabolism and vascular dysfunction. HC is formed upon demethylation of methionine
via S-adenosylmethionine (AdoMet) and S-adenosylhomocysteine (AdoHC). HC is …

Perturbations in homocysteine-linked redox homeostasis in a murine model for hyperhomocysteinemia

V Vitvitsky, S Dayal, S Stabler, Y Zhou… - American Journal …, 2004 - journals.physiology.org
Elevated plasma levels of homocysteine are a risk factor for cardiovascular diseases, neural
tube defects, and Alzheimer's disease. The transsulfuration pathway converts homocysteine …

A 31 bp VNTR in the cystathionine β-synthase (CBS) gene is associated with reduced CBS activity and elevated post-load homocysteine levels

KJA Lievers, LAJ Kluijtmans, SG Heil… - European Journal of …, 2001 - nature.com
Molecular defects in genes encoding enzymes involved in homocysteine metabolism may
account for mild hyperhomocysteinaemia, an independent and graded risk factor for …

Vascular outcome in patients with homocystinuria due to cystathionine β-synthase deficiency treated chronically: a multicenter observational study

S Yap, GHJ Boers, B Wilcken, DEL Wilcken… - … , and vascular biology, 2001 - Am Heart Assoc
An inborn error of metabolism, homocystinuria due to cystathionine β-synthase deficiency,
results in markedly elevated levels of circulating homocysteine. Premature vascular events …

Mutations in cystathionine β‐synthase or methylenetetrahydrofolate reductase gene increase N‐homocysteinylated protein levels in humans

H Jakubowski, GHJ Boers, KA Strauss - The FASEB Journal, 2008 - Wiley Online Library
Severely elevated plasma homocysteine (Hcy) levels observed in genetic disorders of Hcy
metabolism are associated with pathologies in multiple organs and lead to premature death …