Results 121 to 130 of about 975,983 (211)

Structural insights into mutations of cystathionine beta-synthase

, 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
Kraus, J. P., Oliveriusova, J., Burkhard, P., Meier, M.   +3 more
core  

Effect of Cysteine on Expression of Cystathionine .BETA.-Synthase in the Rat Liver.

Journal of Nutritional Science and Vitaminology, 1995
Hepatic cystathionine beta-synthase activity is decreased by the addition of cysteine to the diet. This effect of cysteine was slightly greater in diets containing 0.25% methionine than in those containing 1% methionine, and was reduced during aging.
YAMAMOTO, Nobuko, TANAKA, Takehiko, NOGUCHI, Tamio   +2 more
openaire   +3 more sources

Hydrogen Sulfide Signaling Axis as a Target for Prostate Cancer Therapeutics

Prostate Cancer, 2016
Hydrogen sulfide (H2S) was originally considered toxic at elevated levels; however just in the past decade H2S has been proposed to be an important gasotransmitter with various physiological and pathophysiological roles in the body.
Mingzhe Liu, Lingyun Wu, Sabine Montaut, Guangdong Yang   +3 more
doaj   +1 more source

Short tandem repeats and methylation in the promoter region affect expression of cystathionine beta-synthase gene in the laying hen.

Gene, 2019
Homocysteine can be converted to cysteine via the transsulfuration pathway where cystathionine beta-synthase (CBS) is a rate-limiting enzyme. Homocysteine is thought to play a role in bone remodelling and strength. Previous results indicated that some of
R. Zhou, D. de Koning, H. Mccormack, P. Wilson, I. Dunn   +4 more
semanticscholar   +1 more source

Human Cystathionine-β-Synthase Phosphorylation on Serine227 Modulates Hydrogen Sulfide Production in Human Urothelium. [PDF]

, 2015
Urothelium, the epithelial lining the inner surface of human bladder, plays a key role in bladder physiology and pathology. It responds to chemical, mechanical and thermal stimuli by releasing several factors and mediators.
SORRENTINO, RAFFAELLA, Angela Ianaro, Giuseppe Cirino, Raffaella Sorrentino, Ferdinando Fusco, RUSSO, GIULIA, Erminia Donnarumma, RUSSO, ANNAPINA, CIRINO, GIUSEPPE, Emma Mitidieri, MITIDIERI, EMMA, IANARO, ANGELA, Davide Esposito, Giulia Russo, Roberta d'Emmanuele di Villa Bianca, Donnarumm, Erminia, Vincenzo Mirone, Annapina Russo, ESPOSITO, DAVIDE, MIRONE, VINCENZO, FUSCO, FERDINANDO, D'EMMANUELE DI VILLA BIANCA, ROBERTA, Ianaro Angela, Sorrentino Raffaella, Donnarumm Erminia, Mirone Vincenzo, d’Emmanuele di Villa Bianca Roberta, Esposito Davide, Cirino Giuseppe, Russo Annapina, Fusco Ferdinando, Mitidieri Emma, Russo Giulia   +32 more
core   +2 more sources

Unraveling CBS Mutations and Their Clinical Impact in a Chinese Family With Classical Homocystinuria

Molecular Genetics & Genomic Medicine
Background Classical homocystinuria (HCU), caused by cystathionine beta‐synthase (CBS) deficiency, exhibits significant geographic variability in its mutational spectrum.
Jingfei Zhang, Xinyu Lin, Xinmei Liu, Yilu Lu, Dachang Tao, Dan Yu, Yongxin Ma   +6 more
doaj   +1 more source

Bridging the Gap in Cancer Research: Sulfur Metabolism of Leukemic Cells with a Focus on L-Cysteine Metabolism and Hydrogen Sulfide-Producing Enzymes

Biomolecules
Leukemias are cancers of the blood-forming system, representing a significant challenge in medical science. The development of leukemia cells involves substantial disturbances within the cellular machinery, offering hope in the search for effective ...
Konrad Kaleta, Klaudia Janik, Leszek Rydz, Maria Wróbel, Halina Jurkowska   +4 more
doaj   +1 more source

Hydrogen Sulfide Reduces Myeloid-Derived Suppressor Cell-Mediated Inflammatory Response in a Model of Helicobacter hepaticus-Induced Colitis

Frontiers in Immunology, 2018
Chronic inflammation contributes to tumor initiation in colitis-associated colorectal cancer (CRC). Indeed, inflammatory bowel disease (IBD) patients show an increased risk of developing CRC.
Paola De Cicco, Theodore Sanders, Giuseppe Cirino, Kevin J. Maloy, Angela Ianaro   +4 more
doaj   +1 more source

Potential Pharmacological Chaperones for Cystathionine Beta-Synthase-Deficient Homocystinuria.

Handbook of Experimental Pharmacology, 2018
Classical homocystinuria (HCU) is the most common loss-of-function inborn error of sulfur amino acid metabolism. HCU is caused by a deficiency in enzymatic degradation of homocysteine, a toxic intermediate of methionine transformation to cysteine ...
T. Majtan, A. L. Pey, Paula Giménez-Mascarell, L. Martínez-Cruz, C. Szabó, V. Kožich, J. Kraus   +6 more
semanticscholar   +1 more source

Domain Organization, Catalysis and Regulation of Eukaryotic Cystathionine Beta-Synthases

PLoS ONE, 2014
Cystathionine beta-synthase (CBS) is a key regulator of sulfur amino acid metabolism diverting homocysteine, a toxic intermediate of the methionine cycle, via the transsulfuration pathway to the biosynthesis of cysteine. Although the pathway itself is well conserved among eukaryotes, properties of eukaryotic CBS enzymes vary greatly.
Majtan, Tomas, Pey Rodríguez, Ángel Luis, Fernández, Roberto, Fernández, José A., Martínez-Cruz, Luis A., Kraus, Jan P.   +5 more
openaire   +6 more sources