Microglial SLC25A28 Knockout Mitigates Spinal Cord Injury in Mice by Inhibiting Heme Synthesis and Subsequent NOX2 Activation [PDF]
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.
Huangtao Chen +13 more
wiley +2 more sources
Clinical, Biochemical, and Molecular Characteristics of Filipino Patients with Tyrosinemia Type 1 [PDF]
Hereditary tyrosinemia type I (HT1), or hepatorenal tyrosinemia, is an amino acid disorder which may cause hepatic failure as well as renal and neurologic comorbidities. Early detection of this disorder is possible with newborn screening (NBS).
Barbra Charina V. Cavan +4 more
doaj +2 more sources
Differential Mechanism of ATP Production Occurs in Response to Succinylacetone in Colon Cancer Cells [PDF]
Our aim was to verify the potential ability of succinylacetone (SA) to inhibit mitochondrial function, thereby suppressing cancer cell proliferation.
Phil Jun Lee +4 more
doaj +3 more sources
Nitisinone desensitization protocol, case report of hereditary Tyrosinemia type 1 with successful treatment and outcomes [PDF]
The third known case in the country of Tyrosinemia type 1 is presented, a 10-month-old male infant who was referred to the emergency room due to hepatomegaly, compromised liver function, neurological deterioration, and abnormal urinary amino acids ...
Michael Vallejo +6 more
doaj +2 more sources
1H NMR-based metabolomics approach to understanding the temperature-dependent pathogenicity of Lactococcus garvieae [PDF]
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 +3 more
doaj +3 more sources
Fumarylacetoacetate Hydrolase Regulates Seed Dormancy and Germination Through the Gibberellin Pathway in Arabidopsis [PDF]
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 +4 more
doaj +2 more sources
Differential diagnosis of (inherited) amino acid metabolism or transport disorders [PDF]
__Abstract__ Disorders of amino acid metabolism or transport are most clearly expressed in urine. Nevertheless the interpretation of abnormalities in urinary amino acid excretion remains difficult. An increase or decrease of almost every amino acid in
Blom, W.A.M. (Wim) +1 more
core +8 more sources
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 +8 more
doaj +1 more source
Performance of succinylacetone assays and their associated proficiency testing outcomes [PDF]
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 +6 more
openaire +2 more sources
Metabolic Reprogramming in SARS-CoV-2 Infection Impacts the Outcome of COVID-19 Patients
IntroductionSevere acute respiratory syndrome–coronavirus 2 (SARS-CoV-2) infection triggers inflammatory clinical stages that affect the outcome of patients with coronavirus disease 2019 (COVID-19).
Laura E. Martínez-Gómez +37 more
doaj +1 more source

