Results 61 to 70 of about 6,824 (185)

Development of Pharmacological Strategies for Mitochondrial Disorders. [PDF]

, 2013
Mitochondrial diseases are an unusually genetically and phenotypically heterogeneous group of disorders, which are extremely challenging to treat. Currently, apart from supportive therapy, there are no effective treatments for the vast majority of ...
Heales, SJ, Kanabus, M, Rahman, S
core   +1 more source

Plant‐Derived Melatonin Inhibits Bacterial Virulence via CpxA/R Two‐Component System

Advanced Science, EarlyView.
Plant‐derived melatonin is sensed by CpxAE48/T51, which inhibits the phosphorylation cascade transmission from CpxAH240 to CpxRD52, resulting in the inhibition of DNA‐binding capacity of CpxR and subsequent T3SS genes expression in Pst DC3000. ABSTRACT In defending against pathogens, plants deploy diverse secondary metabolites and signaling molecules ...
Jin‐Wei Wei, Wei Liu, Bili Cao, Dan Zhao, Chengqiang Wang, Xin Hou, Tangyuan Ning, Biao Gong   +7 more
wiley   +1 more source

Inflammation mobilizes copper metabolism to promote colon tumorigenesis via an IL-17-STEAP4-XIAP axis. [PDF]

, 2020
Copper levels are known to be elevated in inflamed and malignant tissues. But the mechanism underlying this selective enrichment has been elusive. In this study, we report a axis by which inflammatory cytokines, such as IL-17, drive cellular copper ...
Bao, Shideng, Bulek, Katarzyna, Cai, Gang, Chang, Christopher J, Chen, Xing, Fox, Paul L, Huang, Emina, Jackson, Mark W, Jia, Shang, Kalady, Matthew F, Li, Xiaoxia, Liao, Yun, Pizarro, Theresa T, Sen, Ganes C, Stark, George R, Tang, Fangqiang, Zhao, Junjie   +16 more
core  

Paradoxical Impact of Two Folate Receptors, FRα and RFC, in Ovarian Cancer: Effect on Cell Proliferation, Invasion and Clinical Outcome [PDF]

, 2012
Despite being an essential vitamin, folate has been implicated to enhance tumor growth, as evidenced by reports on overexpression of folate receptor alpha (FRα) in carcinomas.
Chan, HY, Cheung, ANY, Ip, PPC, Jiang, L, Kong, DSH, Le, X-F, Ngan, HYS, Siu, MKY, Wong, ESY   +8 more
core   +1 more source

Auditory neuropathy in Brown–Vialetto–Van Laere syndrome due to riboflavin transporter RFVT2 deficiency [PDF]

Developmental Medicine & Child Neurology, 2016
AimMutations in the genes encoding the riboflavin transporters RFVT2 and RFVT3 have been identified in Brown–Vialetto–Van Laere syndrome, a neurodegenerative disorder characterized by hearing loss and pontobulbar palsy. Treatment with riboflavin has been shown to benefit individuals with the phenotype of RFVT2 deficiency.
Manoj P, Menezes, Katherine, O'Brien, Mandy, Hill, Richard, Webster, Jayne, Antony, Robert, Ouvrier, Catherine, Birman, Kirsty, Gardner-Berry   +7 more
openaire   +2 more sources

Response of an Infant With Presumed Multiple Acyl‐CoA Dehydrogenase Deficiency (MADD) to Ketone Supplementation

American Journal of Medical Genetics Part A, EarlyView.
ABSTRACT Multiple Acyl‐CoA Dehydrogenase Deficiency (MADD) is an autosomal recessive inborn error of metabolism caused by biallelic pathogenic variants in one of three known genes: ETFA, ETFB, and ETFDH. It can cause multisystem dysfunction, including cardiomyopathy in severe cases.
Yutaka Furuta, Kaitlyn N. Bloom, Jerry Vockley, Angela R. Grochowsky, Neena S. Agrawal, Ellen W. Strickler, Natalie N. Owen, Erica T. Gray, B. Lakshitha A. Perera, Eric R. Gamazon, Lynette C. Rives, Hua‐Chang Chen, Qi Liu, Rizwan Hamid, Joy D. Cogan, John A. Phillips III, Thomas A. Cassini, Bryce A. Schuler   +17 more
wiley   +1 more source

Sensitive detection systems for infectious agents in xenotransplantation*

Xenotransplantation, EarlyView., 2020
Abstract Xenotransplantation of pig cells, tissues, or organs may be associated with transmission of porcine microorganisms, first of all of viruses, to the transplant recipient, potentially inducing a disease (zoonosis). I would like to define detection systems as the complex of sample generation, sample preparation, sample origin, time of sampling ...
Joachim Denner
wiley   +1 more source

CoQ10 and Aging. [PDF]

, 2019
The aging process includes impairment in mitochondrial function, a reduction in anti-oxidant activity, and an increase in oxidative stress, marked by an increase in reactive oxygen species (ROS) production.
Barcelos, Isabella Peixoto de, Haas, Richard H   +1 more
core   +1 more source

Development of a functional outcome measure for riboflavin transporter deficiency

Journal of the Peripheral Nervous System
AbstractBackground and AimsRiboflavin transporter deficiency (RTD) is a progressive inherited neuropathy of childhood onset, characterised clinically by pontobulbar palsy, sensory ataxia, sensorineural deafness, muscle weakness, optic atrophy and respiratory failure.
Jack R. Fennessy, Gabrielle A. Donlevy, Marnee J. McKay, Joshua Burns, Kayla M. D. Cornett, Manoj P. Menezes   +5 more
openaire   +2 more sources

Mitochondrial Abnormalities in iPSC-Derived Motor Neurons From Patients With Riboflavin Transporter Deficiency [PDF]

, 2020
Riboflavin transporter deficiency (RTD) is a childhood-onset neurodegenerative disorder characterized by sensorineural deafness and motor neuron degeneration. Since riboflavin plays key functions in biological oxidation-reduction reactions, energy metabolism pathways involving flavoproteins are affected in RTD.
Fiorella Colasuonno, Enrico Bertini, Marco Tartaglia, Claudia Compagnucci, Sandra Moreno   +4 more
openaire   +1 more source