Role of the cystathionine β-synthase / H2S pathway in the development of cellular metabolic dysfunction and pseudohypoxia in down syndrome. [PDF]
Redox Biol, 2022 Panagaki T +4 more
europepmc +2 more sources
Overproduction of hydrogen sulfide, generated by cystathionine β-synthase, disrupts brain wave patterns and contributes to neurobehavioral dysfunction in a rat model of down syndrome. [PDF]
Redox Biol, 2022 Panagaki T +9 more
europepmc +2 more sources
Journal of Inherited Metabolic Disease, 2023 The loss of cystathionine β‐synthase (CBS), an important homocysteine (Hcy)‐metabolizing enzyme or the loss of PHF8, an important histone demethylase participating in epigenetic regulation, causes severe intellectual disability in humans.
Ł. Witucki, H. Jakubowski
semanticscholar +1 more source
Long-term functional correction of cystathionine β-synthase deficiency in mice by adeno-associated viral gene therapy. [PDF]
J Inherit Metab Dis, 2021 Cystathionine β‐synthase (CBS) deficiency is a recessive inborn error of sulfur metabolism characterized by elevated blood levels of total homocysteine (tHcy).
Lee HO +5 more
europepmc +2 more sources
Down-Regulated Expression of Cystathionine β-Synthase and Cystathionine γ-Lyase in Varicocele, and Infertile Men: A Case-Control Study. [PDF]
Cell J, 2022 Objective Cystathionine β-synthase (CBS) and cystathionine γ-lyase (CSE) are two important enzymes involved in One-Carbon metabolism. These enzymes play important roles in modulating oxidative stress and inflammation in male factor infertility through ...
Akbarian F +3 more
europepmc +2 more sources
Distribution of two kinds of cystathionine γ-synthase in various bacteria [PDF]
FEMS Microbiology Letters, 1986 Abstract The substrate specificity of cystathionine γ-synthase (EC 4.2.99.9) in various bacteria was examined. O -Succinyl- l -homoserine was used preferably as a substrate by facultative anaerobic Gram-negative bacteria belonging to such genera as Escherichia, Klebsiella, Enterobacter, Citrobacter, Erwinia, Serratia, Proteus and Salmonella .
Hiroshi Kanzaki +3 more
openaire +1 more source
Cystathionine β-Synthase Regulates the Proliferation, Migration, and Invasion of Thyroid Carcinoma Cells. [PDF]
Oxid Med Cell Longev, 2022 Thyroid cancer is considered to be one of the most common endocrine tumors worldwide. Cystathionine β‐synthase (CBS) plays a crucial role in the occurrence of several types of malignancies. And yet, the mechanism of action of CBS in the growth of thyroid
Jiang QY +11 more
europepmc +2 more sources
Redox Biology, 2020 Acute pancreatitis is an inflammatory process of the pancreatic gland that may lead to dysregulation of the trans-sulfuration pathway. The aims of this work were firstly to study the methionine cycle as well as the trans-sulfuration pathway using ...
Sergio Rius-Pérez +11 more
doaj +1 more source
Hydrogen sulfide pathway and skeletal muscle: an introductory review. [PDF]
, 2018 The presence of the H2 S pathway in skeletal muscle (SKM) has recently been established. SKM expresses the three constitutive H2 S-generating enzymes in animals and humans, and it actively produces H2 S.
Armogida Chiara +2 more
core +4 more sources
Brain phenotype of transgenic mice overexpressing cystathionine β-synthase. [PDF]
PLoS ONE, 2012 The cystathionine β-synthase (CBS) gene, located on human chromosome 21q22.3, is a good candidate for playing a role in the Down Syndrome (DS) cognitive profile: it is overexpressed in the brain of individuals with DS, and it encodes a key enzyme of ...
Vinciane Régnier +15 more
doaj +1 more source