Results 91 to 100 of about 4,693,382 (193)

Hypoxia‐induced vulnerability of the somatosensory nervous system

The Journal of Physiology, EarlyView.
Abstract figure legend Sensory neurons are highly energy dependent and rely on sufficient oxygen availability to maintain metabolic stability and effective neurocommunication. Within the somatosensory system, even modest reductions in tissue oxygen tension impair neuronal respiration, forcing a shift toward less efficient metabolic pathways that ...
Jack Corbett, Richard P. Hulse
wiley   +1 more source

Analysis of pristanic acid β-oxidation intermediates in plasma from healthy controls and patients affected with peroxisomal disorders by stable isotope dilution gas chromatography mass spectrometry

Journal of Lipid Research, 1999
In this paper we report the development of highly sensitive, selective, and accurate stable isotope dilution gas chromatography negative chemical ionization mass spectrometry (GC-NCI-MS) methods for quantification of peroxisomal β-oxidation intermediates
N.M. Verhoeven, D.S.M. Schor, E.A. Struys, E.E.W. Jansen, H.J. ten Brink, R.J.A. Wanders, C. Jakobs   +6 more
doaj   +1 more source

Defective lipid remodeling of GPI anchors in peroxisomal disorders, Zellweger syndrome, and rhizomelic chondrodysplasia punctata

Journal of Lipid Research, 2012
Many cell surface proteins in mammalian cells are anchored to the plasma membrane via glycosylphosphatidylinositol (GPI). The predominant form of mammalian GPI contains 1-alkyl-2-acyl phosphatidylinositol (PI), which is generated by lipid remodeling from
Noriyuki Kanzawa, N. Shimozawa, R. Wanders, K. Ikeda, Y. Murakami, H. Waterham, Satoru Mukai, Morihisa Fujita, Yusuke Maeda, R. Taguchi, Y. Fujiki, T. Kinoshita   +11 more
semanticscholar   +1 more source

Maternal protein restriction programs the ageing kidney: Proteomic signatures of early‐life origin renal and metabolic dysfunction

The Journal of Physiology, EarlyView.
Abstract figure legend Maternal protein restriction (MPR) induced persistent renal histopathological alterations accompanied by elevated serum creatinine levels. Nephron‐segment proteomic analysis revealed region‐specific molecular dysregulation affecting Bowman's capsule (PARK7, oxidative stress sensor; MSN, cytoskeletal organization), the proximal ...
Marina Pereira Pires, Matheus Naia Fioretto, Marcel Rodrigues Ferreira, Isabelle Tenori Ribeiro, Luísa Annibal Barata, Flávia Alessandra Maciel, Lucas Sobrinho Lemos, Renato Mattos, Luiz Marcos Frediane Portela, Patrick Vieira de Souza, Luisa Correia Gomes, Gustavo Monezzi Cordeiro, Wellerson Rodrigo Scarano, Patrícia Aline Boer, Jose Antonio Rocha Gontijo, Luis Antonio Justulin   +15 more
wiley   +1 more source

Organelle disease: peroxisomal disorders

, 2000
Peroxisomes are virtually ubiquitous organelles involved in numerous catabolic and anabolic pathways. Interest in peroxisomes stems from an expanding group of genetic diseases in which there is either deficiency of a specific peroxisomal function (single
Gärtner, Jutta
core   +1 more source

Molecular basis of peroxisomal biogenesis disorders caused by defects in peroxisomal matrix protein import [PDF]

, 2012
Peroxisomal biogenesis disorders (PBDs) represent a spectrum of autosomal recessive metabolic disorders that are collectively characterized by abnormal peroxisome assembly and impaired peroxisomal function. The importance of this ubiquitous organelle for
Nagotu, Shirisha, Kalel, Vishal C., Erdmann, Ralf, Platta, Harald W.   +3 more
core   +1 more source

Peroxisomal interactome mapping enables network-based modelling of function and disease

Life Science Alliance
Filling a critical gap in the human interactome, we define a comprehensive peroxisomal interaction network that enables network-based hypothesis generation for tissue-specific function, disease mechanisms, and therapeutic exploration.
Søren W Gersting, Julia V Cramer, Philipp Guder, Amelie S Lotz-Havla, Barbara Wolf, Heidi Noll-Puchta, Ralf Erdmann, Ania C Muntau, Mathias Woidy   +8 more
doaj   +1 more source

Autosomal recessive cerebellar ataxia caused by mutations in the PEX2 gene

Orphanet Journal of Rare Diseases, 2011
Objective To expand the spectrum of genetic causes of autosomal recessive cerebellar ataxia (ARCA). Case report Two brothers are described who developed progressive cerebellar ataxia at 3 1/2 and 18 years, respectively.
Wanders Ronald J, Waterham Hans R, Ferdinandusse Sacha, Sevin Caroline, Aubourg Patrick   +4 more
doaj   +1 more source

Myelin Impairment and Regeneration in the Central Nervous System: Molecular Mechanisms, Diseases, and Prospective Therapeutic Targets

MedComm, Volume 7, Issue 10, October 2026.
Proper myelin formation is crucial for normal neural circuit function, while myelin deficiency can lead to neural circuit dysfunction and cognitive decline, notably in Alzheimer's disease and other central nervous system demyelinating disorders. This review summarizes central myelin's structure and function, demyelination biomarkers and pathological ...
Lihong Huang, Peng Cheng, Guangdong Liu, Yang Xiang, Zhen Hong, Weidong Le   +5 more
wiley   +1 more source

Relaxin‐2: Shaping the Proteomic Landscape of Skeletal Muscle Physiology, Glucose Trafficking, and Mitochondrial Function in Rat

The FASEB Journal, Volume 40, Issue 18, 30 September 2026.
Cardioprotective hormone relaxin‐2 showed relevant effects on rat skeletal muscle by altering proteins linked to muscle function, regeneration, differentiation, mitochondrial function, glucose metabolism, and structural integrity and organization. Specifically, relaxin‐2 reduced the expression of 95 proteins, increased 32, and elicited unique proteins ...
Xocas Vázquez‐Abuín, Lucía Gómez‐Cibeira, Javier García‐Seara, Pablo de la Fuente‐López, Carlos Minguito‐Carazo, Susana B. Bravo, Manuel Otero‐Santiago, Oreste Gualillo, José Ramón González‐Juanatey, Francisca Lago, Sandra Feijóo‐Bandín, Alana Aragón‐Herrera   +11 more
wiley   +1 more source