Cellular turnover and degradation of the most common missense cystathionine beta-synthase variants causing homocystinuria. [PDF]
Homocystinuria (HCU) due to cystathionine beta‐synthase (CBS) deficiency is the most common inborn error of sulfur amino acid metabolism. Recent work suggests that missense pathogenic mutations—regardless of their topology—cause instability of the C ...
Mijatovic E +3 more
europepmc +2 more sources
Benserazide, a cystathionine beta-synthase (CBS) inhibitor, potentially enhances the anticancer effects of paclitaxel via inhibiting the S-sulfhydration of SIRT1 and the HIF1-α/VEGF pathway. [PDF]
Cancer targeted therapy is essential to minimize damage to normal cells and improve treatment outcomes. The elevated activity of Cystathionine beta-synthase (CBS), an enzyme responsible for producing endogenous hydrogen sulfide (H2S), plays a significant
Zhao W +7 more
europepmc +2 more sources
Huntingtin interacts with cystathionine beta-synthase [PDF]
We have screened a rat brain library to identify proteins which interact with the 5'-end of huntingtin (amino acids 1-171), including the polyglutamine tract, in the yeast two-hybrid system.
Wood, J. D. +3 more
core +3 more sources
Cystathionine Beta-Synthase [PDF]
CBS1 from Magnaporthe grisea is a structural and functional homolog of the cystathionine beta synthase (CBS) gene from Saccharomyces cerevisiae . Our studies indicated that M.
D. Schomburg, M. Salzmann
semanticscholar +3 more sources
Homocystinuria due to cystathionine beta synthase deficiency [PDF]
A two year-old male child presented with cutis marmorata congenita universalis, brittle hair, mild mental retardation, and finger spasms. Biochemical findings include increased levels of homocysteine in the blood-106.62 µmol/L (normal levels: 5.90-16µmol/
Ahmed, Kamal +4 more
core +3 more sources
Cystathionine beta synthase regulates mitochondrial dynamics and function in endothelial cells. [PDF]
Mutations in the human cystathionine beta synthase (CBS) gene are known to cause endothelial dysfunction responsible for cardiovascular and neurovascular diseases. CBS is the predominant hydrogen sulfide (H2S)‐producing enzyme in endothelial cells (ECs).
Rao G +8 more
europepmc +2 more sources
Cystathionine beta-synthase (CBS) contributes to advanced ovarian cancer progression and drug resistance. [PDF]
Epithelial ovarian cancer is the leading cause of gynecologic cancer deaths. Most patients respond initially to platinum-based chemotherapy after surgical debulking, however relapse is very common and ultimately platinum resistance emerges. Understanding
Sanjib Bhattacharyya +14 more
doaj +2 more sources
Increased expression of cystathionine beta-synthase and chemokine ligand 21 is closely associated with poor prognosis in extrahepatic cholangiocarcinoma. [PDF]
The expression of Cystathionine beta-synthase (CBS) and Chemokine ligand 21 (CCL21) is associated with the tumorigenesis and progression of a variety of tumors, but whether alterations in their expression levels correlates with the carcinogenesis and ...
Wang L +7 more
europepmc +2 more sources
Cystathionine beta-synthase deficiency causes fat loss in mice. [PDF]
Cystathionine beta synthase (CBS) is the rate-limiting enzyme responsible for the de novo synthesis of cysteine. Patients with CBS deficiency have greatly elevated plasma total homocysteine (tHcy), decreased levels of plasma total cysteine (tCys), and ...
Sapna Gupta, Warren D Kruger
doaj +4 more sources
A refined total capsular bag suspension technique for lens subluxation from cystathionine beta-synthase deficiency: A case report and literature review. [PDF]
Purpose: To report the application of a refined total capsular bag suspension technique for lens subluxation from Cystathionine beta-synthase (CBS) deficiency.
Lu Y, Jiang Y, Wang Z.
europepmc +2 more sources

