Results 101 to 110 of about 20,316 (189)

Mitochondrial Dysfunction in Propionic Acidemia: A Case‐Report and Review of the Literature

JIMD Reports, Volume 67, Issue 2, March 2026.
ABSTRACT Propionic acidemia is an inborn error of metabolism involving an enzymatic defect of propionyl‐CoA carboxylase that results in the build‐up of toxic metabolites which can induce metabolic decompensation. Secondary mitochondrial dysfunction in propionic acidemia has been commonly recognized; however, its clinical presentation and management are
Brandon K. Walther, Brittany M. Murray, Poornima Pandiyan, Randall Ray, Laura Yeoh, Amy Kritzer   +5 more
wiley   +1 more source

Long-chain acylcarnitine deficiency promotes hepatocarcinogenesis

Acta Pharmaceutica Sinica B
Despite therapy with potent antiviral agents, chronic hepatitis B (CHB) patients remain at high risk of hepatocellular carcinoma (HCC). While metabolites have been rediscovered as active drivers of biological processes including carcinogenesis, the specific metabolites modulating HCC risk in CHB patients are largely unknown.
Kaifeng Wang, Zhixian Lan, Heqi Zhou, Rong Fan, Huiyi Chen, Hongyan Liang, Qiuhong You, Xieer Liang, Ge Zeng, Rui Deng, Yu Lan, Sheng Shen, Peng Chen, Jinlin Hou, Pengcheng Bu, Jian Sun   +15 more
openaire   +3 more sources

Clinical Outcomes and Management in Late Diagnosed Siblings Affected With Attenuated GSD Ib

JIMD Reports, Volume 67, Issue 2, March 2026.
ABSTRACT Glycogen storage disease 1b (GSD1b) typically presents in early infancy with poor fasting tolerance, hepatomegaly, and neutropenia. We report two siblings who were diagnosed with GSD1b in adulthood. Both had a normal fasting tolerance throughout childhood and, as adults, were able to fast for at least 16 h without developing hypoglycaemia. The
Gregory Lynch, Alison Woodall, Charlotte Dawson, Philip Newsome, Maria Veiga‐da‐Cunha, Karolina M. Stepien   +5 more
wiley   +1 more source

Electrokinetic Manipulations Combined With Direct and Ambient Ionization Mass Spectrometry

Mass Spectrometry Reviews, Volume 45, Issue 2, Page 244-259, March/April 2026.
ABSTRACT Mass spectrometry (MS) is a powerful analytical technique that typically involves sample preparation and online analytical separation before MS detection. Traditional methods often face bottlenecks in sample preparation and analytical separation, despite the rapid detection capabilities of MS.
Nicholas E. Manicke, Lahiru Wedasingha, Magnus Rydberg   +2 more
wiley   +1 more source

Practical Applications of Secondary/Extractive Electrospray Ionization (SESI): A Versatile Tool for Real‐Time Chemical Analysis

Mass Spectrometry Reviews, Volume 45, Issue 2, Page 392-428, March/April 2026.
ABSTRACT In the 1980s, researchers discovered the remarkable ability of electrospray plumes to effectively ionize gas‐phase molecules via secondary ionization. Around 20 years later—coinciding with the ambient mass spectrometry revolution—secondary electrospray ionization (SESI) and extractive electrospray ionization (EESI) coupled to mass spectrometry
Xin Luo, Huiling Wang, Xiaolan Hu, Sasho Gligorovski, Xue Li, Pablo Sinues   +5 more
wiley   +1 more source

Trans‐Omics Integration Reveals That the Kidney Contributes to Systemic Aging via Sexually Dimorphic Accumulation of Glycosphingolipids

MedComm, Volume 7, Issue 3, March 2026.
Female‐specific renal GluCer accumulation disrupts mitochondrial quality control via a conserved purine‐mTORC1 pathway, triggering a wave of uremic toxins into the systemic circulation that constitutes a female‐biased vulnerability toward renal‐driven multiorgan senescence.
Zhen Ni, Chenyin Cao, Yanlin Tian, Jinming Mu, He Tian, Zehua Wang, Shaohua Zhang, Mingjun Cao, Yuntian Yang, Wei Ling Florence Lim, Jingkang Cui, Huan Sun, Huan Miao, Yuan Wang, Jie Du, Timothy Kwok, Huan Chen, Sin Man Lam, Guanghou Shui   +18 more
wiley   +1 more source

Chronic exercise improves hepatic acylcarnitine handling

iScience
Exercise mediates tissue metabolic function through direct and indirect adaptations to acylcarnitine (AC) metabolism, but the exact mechanisms are unclear. We found that circulating medium-chain acylcarnitines (AC) (C12-C16) are lower in active/endurance trained human subjects compared to sedentary controls, and this is correlated with elevated ...
Diego Hernández-Saavedra, J. Matthew Hinkley, Lisa A. Baer, Kelsey M. Pinckard, Pablo Vidal, Shinsuke Nirengi, Andrea M. Brennan, Emily Y. Chen, Niven R. Narain, Valerie Bussberg, Vladimir V. Tolstikov, Michael A. Kiebish, Christina Markunas, Olga Ilkayeva, Bret H. Goodpaster, Christopher B. Newgard, Laurie J. Goodyear, Paul M. Coen, Kristin I. Stanford   +18 more
openaire   +3 more sources

Tomato‐Soy Juice Reduces Inflammation and Modulates the Urinary Metabolome in Adults With Obesity

Molecular Nutrition &Food Research, Volume 70, Issue 5, 12 March 2026.
Compared to pre‐intervention, consuming a tomato‐soy juice (54 mg lycopene, 189 mg soy isoflavones/d) reduced levels of inflammatory cytokines (IL‐5, IL‐12p70, and GM‐CSF, with TNF‐alpha trending downward), increased plasma lycopene and urinary soy isoflavone metabolites, and altered the urinary metabolome. ABSTRACT Chronic, low‐grade inflammation is a
Maria J. Sholola, Jenna Miller, Emma A. Bilbrey, Janet A. Novotny, David M. Francis, Thomas A. Mace, Jessica L. Cooperstone   +6 more
wiley   +1 more source

Insights from the Newborn Screening Program for Very Long-Chain Acyl-CoA Dehydrogenase (VLCAD) Deficiency in Kuwait

International Journal of Neonatal Screening
Newborn screening for very long-chain acyl-CoA dehydrogenase (VLCAD) deficiency in Kuwait was initiated in October 2014. Over a 7-year period (January 2015 to December 2021), 43 newborns were diagnosed with VLCAD deficiency out of 356,819 screened ...
Hind Alsharhan, Amir A. Ahmed, Marwa Abdullah, Moudhi Almaie, Makia J. Marafie, Ibrahim Sulaiman, Reem M. Elshafie, Ahmad Alahmad, Asma Alshammari, Parakkal Xavier Cyril, Usama M. Elkazzaz, Samia M. Ibrahim, Mohamed Elghitany, Ayman M. Salloum, Fahmy Yassen, Rasha Alsafi, Laila Bastaki, Buthaina Albash   +17 more
doaj   +1 more source

Urine Metabolism Biomarkers Predict Preterm Infant Adiposity at Hospital Discharge

Molecular Nutrition &Food Research, Volume 70, Issue 6, 29 March 2026.
In this cohort of term and preterm infants, urinary amino acid and acylcarnitine concentrations varied by diabetes in pregnancy exposure and gestational age at birth, and certain patterns of urinary metabolites were differentially related to postnatal adiposity development in preterm infants of women with diabetes in pregnancy. ABSTRACT In infants born
Catherine O. Buck, Sarah McCollum, Weiwei Wang, TuKiet T. Lam, Sarah N. Taylor, Veronika Shabanova   +5 more
wiley   +1 more source