Results 71 to 80 of about 26,277 (182)

A cautionary tale of pyridoxine toxicity in cystathionine beta‐synthase deficiency detected by two‐tier newborn screening highlights the need for clear pyridoxine dosing guidelines

American Journal of Medical Genetics. Part A, 2020
Classic homocystinuria is due to deficiency of cystathionine beta‐synthase (CBS), a pyridoxine‐dependent enzyme that, depending on the molecular variants, may be co‐factor responsive. Elevated methionine is often used as the primary analyte to detect CBS
Elizabeth G. Ames, A. Scott, Kara B Pappas, Shawn Moloney, R. Conway, Ayesha Ahmad   +5 more
semanticscholar   +1 more source

Stimuli‐responsive nanostructured hydrogen sulfide donors for enhanced cancer treatments

Smart Molecules, EarlyView.
Three typical kinds of stimuli‐responsive nanostructured hydrogen sulfide (H2S) donors are reviewed. The nanostructures ensure precise delivery of H2S to the lesion site and regulate the release behavior. The H2S‐based gas therapy has been used to synergize with other cancer treatment modalities, achieving enhanced efficacy while reducing side effects.
Yuxuan Lyu, Jiaxuan Zhu, Lei Lei, Qingyang Zhong, Zhao Li, Caihong Lei, Fan Rong, Yin Wang   +7 more
wiley   +1 more source

Re‐thinking peripheral dysfunctions in obesity: The emerging role of sulphaceutics and sulphanutraceutics

British Journal of Pharmacology, EarlyView.
Obesity is a chronic, relapsing, multisystem disease in which cardiometabolic risk arises from excess adiposity and progressive dysfunction of peripheral organs, ultimately disrupting endocrine and metabolic crosstalk among tissues. Within this network, sulphur‐based biology, centred on hydrogen sulphide and related reactive sulphur species, has ...
Martina Smimmo, Veronica Casale, Roberta d'Emmanuele di Villa Bianca, Valentina Vellecco, Mariarosaria Bucci   +4 more
wiley   +1 more source

Targeting m6A Modifications Regulating Ferroptosis Offers Novel Therapy in Diseases

Cell Proliferation, EarlyView.
m6A RNA modification regulates ferroptosis by balancing iron metabolism, lipid peroxidation, and antioxidant defenses. Dysregulated m6A signaling disrupts pro‐ and anti‐ferroptotic factors, leading to excess ROS, Fe3+ accumulation, and lipid peroxidation–driven cell death. Targeting m6A‐mediated ferroptotic regulation represents a promising therapeutic
Lida Du, Joshua S. Fleishman, Shuang Wu, Yuan Zhou, Clara X. Wang, Haitong Wang, Yumin Wang, Hongquan Wang   +7 more
wiley   +1 more source

Oxidative and Antioxidant Systems in Ferroptosis of Cancer: Mechanisms, Regulations, and Therapeutic Targeting

MedComm – Oncology, Volume 5, Issue 3, September 2026.
Ferroptosis is governed by the balance between reactive oxygen species (ROS)‐driven lipid peroxidation and a multi‐tiered antioxidant network. ROS sources include mitochondrial electron transport chain, voltage‐dependent anion channels, NADPH oxidases, and endoplasmic reticulum‐resident oxidoreductases, while antioxidant defenses span the primary GSH ...
Deepak K, Pritam Kumar Roy, Maniklal Shee, Budhaditya Mukherjee, Aoife Ward Gahlawat, Mahitosh Mandal   +5 more
wiley   +1 more source

Mucosal acidification increases hydrogen sulfide release through up-regulating gene and protein expressions of cystathionine gamma-lyase in the rat gastric mucosa [PDF]

Iranian Journal of Basic Medical Sciences, 2016
Objective(s): This study was performed to investigate the effects of mucosal acidification on mRNA expression and protein synthesis of cystathionine gamma lyase (CSE), cystathionine beta synthase (CBS), and mucosal release of H2S in gastric mucosa in ...
Seyyed Ali Mard, Ali Veisi, Akram Ahangarpour, Mohammad Kazem Gharib-Naseri   +3 more
doaj  

Duchenne's muscular dystrophy involves a defective transsulfuration pathway activity

Redox Biology, 2021
Duchenne muscular dystrophy (DMD) is the most frequent X chromosome-linked disease caused by mutations in the gene encoding for dystrophin, leading to progressive and unstoppable degeneration of skeletal muscle tissues.
E. Panza, V. Vellecco, F.A. Iannotti, D. Paris, O.L. Manzo, M. Smimmo, N. Mitilini, A. Boscaino, G. de Dominicis, M. Bucci, A. Di Lorenzo, G. Cirino   +11 more
doaj   +1 more source

Polymorphisms of cystathionine beta-synthase gene are associated with susceptibility to sepsis. [PDF]

Eur J Hum Genet, 2016
Sepsis is the systemic inflammatory host response to infection. Cystathionine beta-synthase (CBS)-dependent homocysteine (Hcy) pathway was demonstrated to affect disease severity and mortality in patients with severe sepsis/septic shock. Independent studies identified a single-nucleotide polymorphism (SNP, rs6586282, hg19 chr21:g.44478497C>T) in intron
Sponholz C, Kramer M, Schöneweck F, Menzel U, Inanloo Rahatloo K, Giamarellos-Bourboulis EJ, Papavassileiou V, Lymberopoulou K, Pavlaki M, Koutelidakis I, Perdios I, Scherag A, Bauer M, Platzer M, Huse K.   +14 more
europepmc   +5 more sources

Role of cystathionine beta synthase in lipid metabolism in ovarian cancer. [PDF]

Oncotarget, 2015
Elevated lipid metabolism is implicated in poor survival in ovarian cancer (OC) and other cancers; however, current lipogenesis-targeting strategies lack cancer cell specificity. Here, we identify a novel role of cystathionine beta-synthase (CBS), a sulphur amino acid metabolizing enzyme highly expressed in several ovarian cancer cell lines, in driving
Chakraborty PK, Xiong X, Mustafi SB, Saha S, Dhanasekaran D, Mandal NA, McMeekin S, Bhattacharya R, Mukherjee P.   +8 more
europepmc   +4 more sources

Homocysteine metabolites inhibit autophagy, elevate amyloid beta, and induce neuropathy by impairing Phf8/H4K20me1‐dependent epigenetic regulation of mTOR in cystathionine β‐synthase‐deficient mice

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