Results 171 to 180 of about 12,878 (190)

Oligomeric status of human cystathionine beta‐synthase modulates AdoMet binding

FEBS Letters, 2016
Cystathionine beta‐synthase (CBS) plays a key role in the metabolism of sulfur‐containing amino acids. CBS is a multidomain tetrameric enzyme allosterically activated by S‐adenosylmethionine (AdoMet). Recent crystallographic analyses of engineered CBS lacking the loop made up of residues 516–525 revealed discrepancies in AdoMet binding compared to ...
Pey, Angel L., Martínez-Cruz, Luis Alfonso, Kraus, Jan P., Majtan, Tomas   +3 more
openaire   +2 more sources

Potential Pharmacological Chaperones for Cystathionine Beta-Synthase-Deficient Homocystinuria

2017
Classical homocystinuria (HCU) is the most common loss-of-function inbornerror of sulfur amino acid metabolism. HCU is caused by a deficiency in enzymaticdegradation of homocysteine, a toxic intermediate of methionine transformationto cysteine, chiefly due to missense mutations in the cystathionine betasynthase(CBS) gene.
Majtan, Tomas, Pey, Angel L., Gimenez-Mascarell, Paula, Martínez-Cruz, Luis Alfonso, Szabó, Csaba, Kožich, Viktor, Kraus, Jan P.   +6 more
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., Oliveriusova, J., Kraus, J. P., Burkhard, P.   +3 more
openaire   +2 more sources

Marine natural products as inhibitors of cystathionine beta-synthase activity

Bioorganic & Medicinal Chemistry Letters, 2015
A library consisting of characterized marine natural products as well as synthetic derivatives was screened for compounds capable of inhibiting the production of hydrogen sulfide (H2S) by cystathionine beta-synthase (CBS). Eight hits were validated and shown to inhibit CBS activity with IC50 values ranging from 83 to 187μM.
Megan K, Thorson, Ryan M, Van Wagoner, Mary Kay, Harper, Chris M, Ireland, Tomas, Majtan, Jan P, Kraus, Amy M, Barrios   +6 more
openaire   +2 more sources

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, Ruby, Evande, Omer, Kabil, Sunil, Ojha, Shin, Taoka   +4 more
openaire   +2 more sources

Biogenesis of Hydrogen Sulfide and Thioethers by Cystathionine Beta-Synthase

Antioxidants & Redox Signaling, 2018
Aims: The transsulfuration pathway enzymes cystathionine beta-synthase (CBS) and cystathionine gammalyaseare thought to be the major source of hydrogen sulfide (H2S). In this study, we assessed the role of CBS inH2S biogenesis.Results: We show that despite discouraging enzyme kinetics of alternative H2S-producing reactions utilizingcysteine compared ...
Majtan, Tomas, Krijt, Jakub, Sokolová, Jitka, Krizkova, Michaela, Ralat, Maria A, Kent, Jana O., Gregory, Jesse F., Kožich, Viktor, Kraus, Jan P.   +8 more
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., Teerlink, T., Janssen, M.C.H., Kluijtmans, L.A.J., Kluijtmans, L.A.J., Smulders, Y., Jakobs, C., Tavares de Almeida, I., Rivera, I., Castro, R., Blom, H.J.   +10 more
openaire   +4 more sources

Cystathionine beta-synthase and gamma-cystathionase in helminths.

Parasitology research, 1992
The activities of gamma-cystathionase and cystathionine beta-synthase were investigated in a range of gastrointestinal, free-living and entomophagous nematodes. Although nematode gamma-cystathionase used the same range of substrates as the mammalian hepatic enzyme, its activity was extremely low and there were significant interspecies variations with ...
J, Walker, J, Barrett
openaire   +1 more source

Postoperative thromboemboli in cystathionine beta-synthase deficiency

American Heart Journal, 1984
G M, Jackson, J S, Grisolia, P L, Wolf, O W, Jones, C M, Bloor   +4 more
openaire   +2 more sources

Cystathionine Beta-Synthase Deficiency

2023
openaire   +1 more source