Physiological roles of hydrogen sulfide in mammalian cells, tissues, and organs

G Cirino, C Szabo… - Physiological …, 2023 - journals.physiology.org
Over the last two decades, hydrogen sulfide (H2S) has emerged as an endogenous
regulator of a broad range of physiological functions. H2S belongs to the class of molecules …

Guidelines for the diagnosis and management of cystathionine beta-synthase deficiency

AAM Morris, V Kožich, S Santra, G Andria… - Journal of inherited …, 2017 - Springer
Cystathionine beta-synthase (CBS) deficiency is a rare inherited disorder in the methionine
catabolic pathway, in which the impaired synthesis of cystathionine leads to accumulation of …

Cystathionine-β-synthase: molecular regulation and pharmacological inhibition

K Zuhra, F Augsburger, T Majtan, C Szabo - Biomolecules, 2020 - mdpi.com
Cystathionine-β-synthase (CBS), the first (and rate-limiting) enzyme in the transsulfuration
pathway, is an important mammalian enzyme in health and disease. Its biochemical …

Reprogrammed transsulfuration promotes basal-like breast tumor progression via realigning cellular cysteine persulfidation

K Erdélyi, T Ditrói, HJ Johansson… - Proceedings of the …, 2021 - National Acad Sciences
Basal-like breast cancer (BLBC) is the most aggressive subtype of breast tumors with poor
prognosis and limited molecular-targeted therapy options. We show that BLBC cells have a …

Hypermethioninemias of genetic and non‐genetic origin: a review

SH Mudd - American Journal of Medical Genetics Part C …, 2011 - Wiley Online Library
This review covers briefly the major conditions, genetic and non‐genetic, sometimes leading
to abnormally elevated methionine, with emphasis on recent developments. A major aim is …

[HTML][HTML] Homocystinuria caused by cystathionine beta-synthase deficiency

SJ Sacharow, JD Picker, HL Levy - 2017 - europepmc.org
Homocystinuria caused by cystathionine β-synthase (CBS) deficiency is characterized by
involvement of the eye (ectopia lentis and/or severe myopia), skeletal system (excessive …

Cysteine and obesity: consistency of the evidence across epidemiologic, animal and cellular studies

AK Elshorbagy, V Kozich, AD Smith… - Current Opinion in …, 2012 - journals.lww.com
Cysteine and obesity: consistency of the evidence across epi... : Current Opinion in Clinical
Nutrition & Metabolic Care Cysteine and obesity: consistency of the evidence across …

Performance of expanded newborn screening in Norway supported by post-analytical bioinformatics tools and rapid second-tier DNA analyses

T Tangeraas, I Sæves, C Klingenberg… - International journal of …, 2020 - mdpi.com
In 2012, the Norwegian newborn screening program (NBS) was expanded (eNBS) from
screening for two diseases to that for 23 diseases (20 inborn errors of metabolism, IEMs) …

[HTML][HTML] Cystathionine β-synthase overexpression drives metastatic dissemination in pancreatic ductal adenocarcinoma via inducing epithelial-to-mesenchymal …

Á Czikora, K Erdélyi, T Ditrói, N Szántó, EP Jurányi… - Redox Biology, 2022 - Elsevier
Pancreatic ductal adenocarcinoma (PDAC) is one of the deadliest of all cancer types with a
constant rise in global incidence. Therefore, better understanding of PDAC biology, in order …

Dopamine treatment attenuates acute kidney injury in a rat model of deep hypothermia and rewarming–The role of renal H2S-producing enzymes

GJ Dugbartey, F Talaei, MC Houwertjes, M Goris… - European Journal of …, 2015 - Elsevier
Hypothermia and rewarming produces organ injury through the production of reactive
oxygen species. We previously found that dopamine prevents hypothermia and rewarming …