Results 141 to 150 of about 26,277 (182)
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Hippocampal cystathionine beta synthase in young and aged mice

Neuroscience Letters, 2014
Cystathionine beta synthase (CBS) is the main contributor to the production of hydrogen sulfide (H2S) in the brain. Exogenously administered H2S has been reported to protect neurons against hypoxic injury, ischemia and LPS-induced neuro-inflammation and in the facilitating of long term potentiation (LTP).
Bruintjes, J.J.   +3 more
openaire   +3 more sources

A cystathionine beta-synthase domain containing protein, OsCBSCBS4, interacts with OsSnRK1A and OsPKG and functions in abiotic stress tolerance in rice.

Plant, Cell and Environment
The Cystathionine-β-Synthase (CBS) domain-containing proteins (CDCPs) constitute a functionally diverse protein superfamily, sharing an evolutionary conserved CBS domain either in pair or quad. Rice genome (Oryza sativa subsp.
Surabhi Tomar   +5 more
semanticscholar   +1 more source

Human Cystathionine β-Synthase Is a Target for Sumoylation

Biochemistry, 2006
Cystathionine beta-synthase (CBS) catalyzes the first irreversible step in the transsulfuration pathway and commits the toxic metabolite, homocysteine, to the synthesis of cysteine. Mutations in CBS are the most common cause of severe hereditary hyperhomocysteinemia.
Omer, Kabil, You, Zhou, Ruma, Banerjee
openaire   +2 more sources

Structural insights into mutations of cystathionine β-synthase

Biochimica et Biophysica Acta (BBA) - Proteins and Proteomics, 2003
Cystathionine beta-synthase (CBS) is a unique heme-containing enzyme that catalyses a pyridoxal 5'-phosphate (PLP)-dependent condensation of serine and homocysteine to give cystathionine. Deficiency of CBS leads to homocystinuria, an inherited disease of sulfur amino acid metabolism characterised by increased levels of homocysteine and methionine and ...
Meier, M.   +3 more
openaire   +3 more sources

Cystathionine β-synthase (human)

1987
Publisher Summary Cystathionine β-synthase (L-serine hydro-lyase [adding homocysteine]) is a pyridoxal 5’-phosphate-dependent enzyme in the transsuifuration pathway of higher eukaryotes which catalyzes the condensation of serine and homocysteine to form cystathionine.
openaire   +2 more sources

Classical homocystinuria: from cystathionine beta-synthase deficiency to novel enzyme therapies.

Biochimie, 2019
Genetic defects in cystathionine beta-synthase (CBS), a key enzyme of organic sulfur metabolism, result in deficiency of CBS activity and a rare inborn error of metabolism called classical homocystinuria (HCU).
Erez M. Bublil, T. Majtan
semanticscholar   +1 more source

Reaction mechanism and regulation of cystathionine β-synthase

Biochimica et Biophysica Acta (BBA) - Proteins and Proteomics, 2003
In mammals, cystathionine beta-synthase catalyzes the first step in the transsulfuration pathway which provides an avenue for the conversion of the essential amino acid, methionine, to cysteine. Cystathionine beta-synthase catalyzes a PLP-dependent condensation of serine and homocysteine to cystathionine and is unique in also having a heme cofactor. In
Ruma, Banerjee   +4 more
openaire   +2 more sources

The Role of Cystathionine β-Synthase in Homocysteine Metabolism

Antioxidants & Redox Signaling, 2005
Cystathionine beta-synthase (CBS) is the first enzyme in the transsulfuration pathway, catalyzing the conversion of serine and homocysteine to cystathionine and water. The enzyme contains three functional domains. The middle domain contains the catalytic core, which is responsible for the pyridoxal phosphate-catalyzed reaction.
Kwang-Hwan, Jhee, Warren D, Kruger
openaire   +2 more sources

Asymmetric dimethylarginine in adults with cystathionine β-synthase deficiency

Atherosclerosis, 2012
In hyperhomocysteinemia (HHcy), an independent risk factor for cardiovascular diseases, endothelial dysfunction due to reduced bioavailability of nitric oxide is a consistent finding. However, the underlying mechanisms remain unknown. Increased levels of the nitric oxide synthase inhibitor asymmetric dimethylarginine (ADMA) have been associated with ...
Rocha, M.S.   +10 more
openaire   +5 more sources

Cystathionine Beta-Synthase Deficiency or Classical Homocystinuria

2016
Inherited homocystinurias have in common accumulation of homocysteine and encompass two distinctive entities: classical homocystinuria due to cystathionine β‎-synthase (CBS) deficiency and the rare inborn errors of cobalamin and folate metabolism. The natural history of CBS deficiency, the subject of this chapter, is well described compared to the ...
Henk J. Blom   +2 more
openaire   +1 more source

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