Results 51 to 60 of about 3,031 (152)

Cardioprotective potential of simvastatin in the hyperhomocysteinemic rat heart

Journal of Advanced Pharmaceutical Technology & Research, 2012
The present study investigated the probable role of simvastatin, 3-hydroxymethyl-glutaryl coenzyme A (HMG-CoA) reductase inhibitor, in abrogated cardioprotection in hyperhomocysteinemic (Hhcy) rat hearts.
Ankur Rohilla, M U Khan, Razia Khanam
doaj   +1 more source

Ratoon Season Rice Reduces Methane Emissions by Limiting Acetic Acid Transport to the Rhizosphere and Inhibiting Methanogens

Advanced Science, Volume 13, Issue 8, 9 February 2026.
This study finds that the interaction between ABA‐OsCIPK2‐OsSWEET1A reduces the allocation of methane producing bacteria carbon source (acetic acid) content to the rhizosphere soil of ratoon season rice, thereby reducing methane emissions. Abstract Rice paddies are a major, persistent source of atmospheric methane (CH4), emission rates depend on the ...
Jingnan Zou, Hailong Xu, Bin Qin, Chaojie Lan, Jinying Li, Bianhong Zhang, Huabin Zhang, Chunlin Guo, Hongfei Chen, Zhongming Fang, Quanzhi Zhao, Wenfei Wang, Changxun Fang, Zhixing Zhang, Wenxiong Lin   +14 more
wiley   +1 more source

Natural History, Outcome, and Treatment Efficacy in Children and Adults with Glutaryl-CoA Dehydrogenase Deficiency [PDF]

Pediatric Research, 2006
Glutaryl-CoA dehydrogenase (GCDH) deficiency is a rare inborn disorder of L-lysine, L-hydroxylysine, and L-tryptophan metabolism complicated by striatal damage during acute encephalopathic crises. Three decades after its description, the natural history and how to treat this disorder are still incompletely understood.
Kolker S, Garbade SF, Greenberg CR, Leonard JV, Saudubray JM, Ribes A, Kalkanoglu S, Lund AM, Merinero B, Wajner M, Troncoso M, Williams M, Walter JH, Campistol J, Marti-Herrero M, Caswill M, BURLINA A, Lagler F, Maier EM, Schwahn B, Tokatli A, Dursun A, Coskun T, Chalmers RA, Koeller DM, Zschocke J, Christensen E, Burgard P, Hoffmann GF   +28 more
openaire   +4 more sources

Balanced Expression of the Diiron Oxygenase BioE Is Essential for Biotin Homeostasis in Elizabethkingia meningoseptica

Advanced Science, Volume 13, Issue 10, 18 February 2026.
BioE is a new diiron oxygenase that catalyzes the conversion of long‐chain acyl groups into pimeloyl thioester, initiating biotin synthesis. The overexpression of EmBioE disrupts lipid metabolic homeostasis, requiring repressor BioL to maintain a balance between long‐chain fatty acids and biotin synthesis.
Meng Zhang, Ying‐ying Fu, Xiaoqiang Yang, Qiuying Qin, Xinyu Su, Jiaming Fang, Yanhua Kang, Qingwen He, Zhi Ruan, Yongchang Xu   +9 more
wiley   +1 more source

Single‐cell analysis reveals neuroprotective histone deacetylase inhibitor pathways

Alzheimer's &Dementia, Volume 22, Issue 2, February 2026.
Abstract INTRODUCTION Alzheimer's disease (AD) involves β‐amyloid (Aβ) accumulation, tau pathology, and neuroinflammation, driving cognitive decline. Despite extensive research, disease‐modifying therapies remain elusive. We integrated single‐cell RNA sequencing (scRNA‐seq), spatial transcriptomics, and in vitro validation to identify repurposable ...
Madeline Peyton, Nur Jury‐Garfe, Jiahui Liu, Caleb Beimfohr, Chitra Sunil, Steven Brooks, Pengyue Zhang, Sean D. McCabe, Timothy I. Richardson, Kun Huang, Cristian A. Lasagna‐Reeves, Jie Zhang, Travis S. Johnson   +12 more
wiley   +1 more source

Biochemical Testing Promotes Interpretation of Variants of Uncertain Significance in Prenatal Genetic Disease Testing in Four Organic Acidurias

Clinical Genetics, Volume 109, Issue 2, Page 211-217, February 2026.
This review demonstrates how amniotic fluid biochemical testing provides critical phenotypic evidence (ACMG PP4) for reclassifying variants of uncertain significance (VUS) in prenatal organic acidurias. Integrating metabolite analysis with genetic testing enhances diagnostic accuracy and enables informed clinical decisions for affected families ...
Kaili Yin, Qingwei Qi
wiley   +1 more source

Neurodevelopmental and cognitive behavior of glutaryl-CoA dehydrogenase deficient knockout mice

Life Sciences, 2013
The establishment of a genetic knockout murine model of glutaric acidemia type I (GAI) with complete loss of glutaryl-CoA dehydrogenase (GCDH) activity has been used to investigate the pathological mechanisms underlying neurological symptoms in this disorder. However, very little has been reported on the neurobehavior of GCDH deficient mice (Gcdh(-/-)).
Busanello, Estela Natacha Brandt, Pettenuzzo, Letícia, Botton, Paulo Henrique, Pandolfo, Pablo, de Souza, Diogo Onofre Gomes, Woontner, Michael, Goodman, Stephen, Koeller, David, Wajner, Moacir   +8 more
openaire   +2 more sources

Blocking Lysine Crotonylation and Aerobic Glycolysis as Targeting Strategy Against mpox Virus Replication

Advanced Science, Volume 13, Issue 4, 19 January 2026.
Wei et al. report that MPXV infection induces aerobic glycolysis, a process mediated by the viral protein I3 through lysine crotonylation at its K102 residue. The acetyltransferase MYST1 catalyzes the crotonylation of I3 to inhibit the ubiquitin‐mediated degradation of WDR26.
Pengjun Wei, Zongzheng Zhao, Ruoqi Xu, Qin Yan, Liangzi Jiang, Fuxiao Geng, Yang Gu, Tianjiao Wang, Jing Zhou, Xiao Li, Qin Yan, Chun Lu, Wan Li   +12 more
wiley   +1 more source

Characterization of novel GCDH pathogenic variants causing glutaric aciduria type 1 in the southeast of Mexico

Molecular Genetics and Metabolism Reports, 2019
Biallelic mutations of the GCDH gene result in Glutaric Aciduria type 1 (GA1; OMIM #231670), an uncommon autosomal recessive inborn error caused by the deficiency of glutaryl-CoA dehydrogenase (CCDH), a mitochondrial matrix protein involved in the ...
Felix-Julian Campos-Garcia, Oscar F. Chacon-Camacho, Silvina Contreras-Capetillo, Marisa Cruz-Aguilar, Carolina E. Medina-Escobedo, Claudia M. Moreno-Graciano, Agustín Rodas, Luz del Alba Herrera-Perez, Juan C. Zenteno   +8 more
doaj   +1 more source

Peripheral Neuropathy Expands the Neurological Phenotype in Glutaric Aciduria Type 1

Journal of Inherited Metabolic Disease, Volume 49, Issue 1, January 2026.
ABSTRACT Glutaric aciduria type 1 (GA1) is a neurometabolic disorder characterized by striatal injury in infancy and extrastriatal central nervous system abnormalities, the latter depending on the biochemical subtype. Whether the peripheral nervous system (PNS) is also affected has not been systematically studied.
Fabian Preisner, Sven F. Garbade, Inga Harting, Saskia B. Wortmann, Chris Mühlhausen, Sabine Heiland, Martin Bendszus, Stefan Kölker, Nikolas Boy   +8 more
wiley   +1 more source