Results 21 to 30 of about 1,764 (178)

1H NMR-based metabolomics approach to understanding the temperature-dependent pathogenicity of Lactococcus garvieae [PDF]

International Journal of Aquatic Biology, 2019
Lactococcus garvieae is known as main agent of the bacterial diseases, Lactococcosis in trout farms. The present study was aimed to study the metabolic bases of the temperature-dependent pathogenicity of the L. garvieae using 1H NMR spectroscopy.
Nasim Safari Alighialo, Ruhollah Rahimi, Saeed Hajirezaee, Farzaneh Nikookhah   +3 more
doaj   +3 more sources

Differential Mechanism of ATP Production Occurs in Response to Succinylacetone in Colon Cancer Cells [PDF]

Molecules, 2019
Our aim was to verify the potential ability of succinylacetone (SA) to inhibit mitochondrial function, thereby suppressing cancer cell proliferation.
Phil Jun Lee, Seung Je Woo, Hee Min Yoo, Namki Cho, Hong Pyo Kim   +4 more
doaj   +3 more sources

Fumarylacetoacetate Hydrolase Regulates Seed Dormancy and Germination Through the Gibberellin Pathway in Arabidopsis [PDF]

Plants
Tyrosine (Tyr) degradation is a crucial pathway in animals. However, its role in plants remains to be examined. Fumarylacetoacetate hydrolase (FAH) is the final enzyme involved in Tyr degradation. Studies of a mutant of the SHORT-DAY SENSITIVE CELL DEATH
Chao Hu, Hua Yang, Xuewen Zhang, Chunmei Ren, Lihua Huang   +4 more
doaj   +2 more sources

Overview of European practices for management of tyrosinemia type 1 [PDF]

Journal of Inherited Metabolic Disease, Volume 48, Issue 5, September 2025.
The introduction of nitisinone (NTBC) and newborn screening for Tyrosinemia type 1 (TT1) enabled preemptive treatment ofpatients, thereby significantly improving outcomes by preventing liver, kidney, and neurological issues.
Das, Anibh M., Heiner-Fokkema, M. Rebecca, Kuypers, Allysa M., Maiorana, Arianna, Spronsen, Francjan J. van   +4 more
core   +3 more sources

Tyrosinaemia type Ia without excess of urinary succinylacetone [PDF]

Journal of Inherited Metabolic Disease, 1993
Tyrosinaemia type I (McKusick 276700) (Kvittingen 1991) is an autosomal recessively inherited metabolic disorder due to two enzymatic deficiencies: fumarylacetoacetase (FAH) (type Ia) and maleylacetoacetate isomerase (type Ib) (Berger et al 1988).
Biasucci, G, Giuffré, B, LA GRUTTA, Stefania, Riva, E.   +3 more
core   +5 more sources

Investigation of newborn blood metabolomics in varying intrauterine growth conditions [PDF]

Jornal de Pediatria
Objectives: This study aimed to investigate changes in the blood metabolic profiles of newborns with varying intrauterine growth conditions. Specifically, we analyzed the levels of amino acids, carnitine, and succinylacetone among full-term newborns ...
Shengwen Wang, Xiaofei Lin, Yu Zhou, Xin Yang, Mingming Ou, Linxin Zhang, Yumei Wang, Jing Gao   +7 more
doaj   +2 more sources

Metabolic investigations prevent liver transplantation in two young children with citrullinemia type I [PDF]

, 2010
Acute liver failure may be caused by a variety of disorders including inborn errors of metabolism. In those cases, rapid metabolic investigations and adequate treatment may avoid the need for liver transplantation.
A Dhawan, Dirk-Jan Reijngoud, DP Dimmock, Edmond H. H. M. Rings, F Endo, Francjan J. van Spronsen, Frans W. Verheijen, JM Saudubray, L Fregonese, M Summar, Maaike M. C. de Vries, Marc Eling, Marcel Cuppen, Martijn J. de Groot, MR Narkewicz, P Durand, P Keskinen, PF Whitington, RH Squires, SA Zamora, SE Olpin, SW Brusilow, T Mori, WJ Kleijer   +23 more
core   +8 more sources

Microglial SLC25A28 Knockout Mitigates Spinal Cord Injury in Mice by Inhibiting Heme Synthesis and Subsequent NOX2 Activation. [PDF]

CNS Neurosci Ther
Microglial SLC25A28 knockout reduces spinal cord injury by inhibiting mitochondrial iron accumulation and heme synthesis, leading to decreased NOX2 activation and neuroinflammation. This study reveals the SLC25A28‐heme‐NOX2 axis as a key regulator of microglial‐driven secondary damage after SCI.
Chen H, Guo S, Mi Y, Han R, Xi Y, Peng T, Fu L, Liu W, Ma R, Yu B, Zhang Y, Li L, Ye J, Gong S.   +13 more
europepmc   +2 more sources

The Importance of Succinylacetone: Tyrosinemia Type I Presenting with Hyperinsulinism and Multiorgan Failure Following Normal Newborn Screening

International Journal of Neonatal Screening, 2020
Tyrosinemia type I (TT1) is an inborn error of tyrosine metabolism with features including liver dysfunction, cirrhosis, and hepatocellular carcinoma; renal dysfunction that may lead to failure to thrive and bone disease; and porphyric crises. Once fatal
Jessica R. C. Priestley, Hana Alharbi, Katharine Press Callahan, Herodes Guzman, Irma Payan-Walters, Ligia Smith, Can Ficicioglu, Rebecca D. Ganetzky, Rebecca C. Ahrens-Nicklas   +8 more
doaj   +1 more source

Performance of succinylacetone assays and their associated proficiency testing outcomes [PDF]

Clinical Biochemistry, 2012
Succinylacetone (SUAC) is the primary metabolic marker for hepatorenal tyrosinemia.We used results reported for dried-blood-spot proficiency testing (PT) specimens and hepatorenal tyrosinemia patients' newborn screening (NBS) samples to demonstrate analytic biases in SUAC recoveries and differences in presumptive clinical classifications.SUAC ...
B W, Adam, E M, Hall, N K, Meredith, T H, Lim, C A, Haynes, V R, De Jesus, W H, Hannon   +6 more
openaire   +2 more sources