Results 231 to 240 of about 89,018 (342)

A Nonketotic Hyperglycinemia Mouse Shows Wide‐Ranging Biochemical Consequences of Elevated Glycine, Reduced Folate One‐Carbon Charging, and Serine Deficiency

Journal of Inherited Metabolic Disease, Volume 49, Issue 1, January 2026.
ABSTRACT Nonketotic hyperglycinemia is a severe neonatal epileptic encephalopathy caused by deficient glycine cleavage enzyme activity, for which currently no effective treatment exists. Incomplete understanding of brain biochemistry represents a major knowledge gap to develop new treatments.
Michael A. Swanson, Hua Jiang, Lakshmi Divya Kolora, Rachel Molino, Richard Reisdorph, Cole R. Michel, Katrina A. Doenges, Kit‐Yi Leung, Xiangping Lin, Frank Wong, Samual Lancaster, Basil Michael, Michael Snyder, Daniella H. Hock, David A. Stroud, Tim Wood, Robert Binard, Laura Anderson‐Lehman, Uwe Christians, Erland Arning, Marisa W. Friederich, Roxanne A. Van Hove, Kenneth N. MacLean, Nicholas D. E. Greene, Johan L. K. Van Hove   +24 more
wiley   +1 more source

Neutralization of acyl coenzyme A binding protein for the experimental prevention and treatment of hepatocellular carcinoma. [PDF]

Cell Rep Med
Li S, Motiño O, Lambertucci F, Pol J, Chen H, Pan L, Durand S, Rossin F, Campani C, Poupel L, Klein C, Montégut L, Pérez-Lanzón M, Anagnostopoulos G, Nogueira-Recalde U, Cerone A, Aprahamian F, Dong Y, Lizarralde-Guerrero M, Xue E, Liu P, Zhao L, Pan H, Carbonnier V, Lachkar S, Saavedra Díaz EG, Sun L, Desdouets C, Colnot S, Kepp O, Martins I, Zitvogel L, Piacentini M, Nault JC, Maiuri MC, Zucman-Rossi J, Kroemer G.   +36 more
europepmc   +1 more source

Normal Biomarkers in an Acute Presentation in a Known Case of Medium-Chain Acyl-Coenzyme A Dehydrogenase Deficiency. [PDF]

Clin Chem, 2021
Alharbi H, Bennett MJ, He M, Master SR, Ganetzky RD.   +4 more
europepmc   +1 more source

Human Liver-Specific Very-Long-Chain Acyl-Coenzyme A Synthetase: cDNA Cloning and Characterization of a Second Enzymatically Active Protein

, 1999
Steven J. Steinberg, Susan J. Wang, Martina C. McGuinness, Paul A. Watkins   +3 more
openalex   +1 more source

Chemoenzymatic Synthesis of Acyl Coenzyme A Substrates Enables in Situ Labeling of Small Molecules and Proteins.

Organic Letters, 2015
V. Agarwal, S. Diethelm, L. Ray, Neha Garg, T. Awakawa, P. Dorrestein, B. Moore   +6 more
semanticscholar   +1 more source

Energy Metabolism Under Stress: Late‐Stage Leigh Syndrome Reveals Profound Cardiometabolic Perturbations in Ndufs4 KO Mice

Journal of Inherited Metabolic Disease, Volume 49, Issue 1, January 2026.
Cardiac bioenergetic and metabolic perturbations in late‐stage Leigh syndrome. Whole‐body NDUFS4 loss severely impairs cardiac complex I activity and respiration, shifting reliance to complex II and reducing glycolytic, TCA, and amino acid‐derived energy‐generating substrates in the heart.
Karin Terburgh, Nastassja Sweeney, Roan Louw   +2 more
wiley   +1 more source

Catalytic and FAD-binding Residues of Mitochondrial Very Long Chain Acyl-Coenzyme A Dehydrogenase [PDF]

, 1998
Masayoshi Souri, Toshifumi Aoyama, Gerald F. Cox, Takashi Hashimoto   +3 more
openalex   +1 more source

Characterization of Novel Acyl Coenzyme A Dehydrogenases Involved in Bacterial Steroid Degradation

Journal of Bacteriology, 2015
Amanda Ruprecht, Jay C. Maddox, A. J. Stirling, Nicole Visaggio, S. Seah   +4 more
semanticscholar   +1 more source

Structural basis of product recognition by Mycobacterium tuberculosis fatty acid synthase

Protein Science, Volume 35, Issue 1, January 2026.
Abstract Microbial iterative fatty acid synthases (FAS) are versatile multienzymes under scrutiny for their potential as anti‐infectious targets and their biotechnological applications. They produce saturated fatty acids with defined chain length and release them as coenzyme A‐conjugates.
Elnaz Khalili Samani, S M Naimul Hasan, Alexander F. A. Keszei, Mahtab Heydari, Mohammad T. Mazhab‐Jafari   +4 more
wiley   +1 more source

Pathogenic role of acyl coenzyme A binding protein (ACBP) in Cushing's syndrome. [PDF]

Nat Metab
Pan H, Tian AL, Chen H, Xia Y, Sauvat A, Moriceau S, Lambertucci F, Motiño O, Zhao L, Liu P, Mao M, Li S, Zhang S, Joseph A, Durand S, Aprahamian F, Luo Z, Ou Y, Shen Z, Xue E, Pan Y, Carbonnier V, Stoll G, Forveille S, Leduc M, Cerrato G, Cerone A, Maiuri MC, Castinetti F, Brue T, Wang H, Ma Y, Martins I, Kepp O, Kroemer G.   +34 more
europepmc   +1 more source