Review
Endocrinology & Metabolism
Cezar Gavrilovici, Jong M. Rho
Summary: Metabolic epilepsies are neurological disorders caused by rare inborn errors of metabolism, characterized by impaired bioenergetics and can be treated with ketogenic diet. Further research is needed to identify which metabolic epilepsies are suitable for ketogenic therapies and to design more effective metabolism-based treatments.
JOURNAL OF INHERITED METABOLIC DISEASE
(2021)
Article
Biochemistry & Molecular Biology
Tanakan Paladkong, Panu Pimviriyakul, Jittima Phonbuppha, Somchart Maenpuen, Pimchai Chaiyen, Ruchanok Tinikul
Summary: In the catalytic function of the succinic semialdehyde dehydrogenase (SSADH) enzyme from Acinetobacter baumannii, Cys291 and Glu255 play important roles in enhancing catalytic activity and nucleophilicity, and in regulating the enzyme. This study provides mechanistic insights into the functional importance of these residues in SSADH catalysis.
Article
Clinical Neurology
Phillip L. Pearl, Itay Tokatly Latzer, Henry H. C. Lee, Alexander Rotenberg
Summary: Treatment options for inherited metabolic epilepsies are expanding rapidly due to advancements in molecular biology and the genomic revolution. Traditional therapies like dietary modification and protein/enzyme inhibitors or enhancers are continually being improved to enhance efficacy and reduce side effects. Promising new approaches include enzyme replacement, gene replacement, and gene editing for targeted treatment and potential cures. Biomarkers such as molecular analysis, imaging, and neurophysiologic measurements are emerging as crucial indicators of disease pathophysiology, severity, and response to therapy.
Article
Nutrition & Dietetics
Orna Staretz-Chacham, Ben Pode-Shakked, Eyal Kristal, Smadar Yaala Abraham, Keren Porper, Ohad Wormser, Ilan Shelef, Yair Anikster
Summary: Partial KD treatment can improve the survival of patients with DLD-E3 deficiency, but it does not significantly improve the quality of life (QOL) of the patients.
Article
Plant Sciences
Dmitry N. Fedorin, Alexander T. Eprintsev, Orlando J. Florez Caro, Abir U. Igamberdiev
Summary: The effect of salt stress on gene expression, promoter methylation, and enzymatic activity of SDH and SSADH was investigated in maize. Salt stress rapidly increased SSADH activity and production of succinate through the GABA shunt, leading to the activation of SDH. SDH activity and succinate oxidation increased more slowly and were accompanied by increased expression of genes encoding SDH subunits. This study highlights the role of succinate metabolism in response to salt stress.
Article
Nutrition & Dietetics
Zeyu Zhou, Jocelyn Vidales, Jose A. Gonzalez-Reyes, Bradley Shibata, Keith Baar, Jennifer M. Rutkowsky, Jon J. Ramsey
Summary: The study found that a short-term ketogenic diet increased mitochondrial content in the red gastrocnemius muscle, but did not alter mitochondrial content in the liver, prefrontal cortex, or hippocampus. These results indicate tissue-specific changes in mitochondrial mass with a ketogenic diet.
Article
Biochemistry & Molecular Biology
Henry H. C. Lee, Gabrielle E. McGinty, Phillip L. Pearl, Alexander Rotenberg
Summary: Succinic semialdehyde dehydrogenase deficiency (SSADHD) is a rare genetic disorder that leads to incomplete metabolism of the neurotransmitter GABA, resulting in various clinical manifestations. This article reviews relevant molecular mechanisms and discusses the challenges in SSADHD gene and enzyme replacement therapies. It also introduces a mouse model that restores SSADH activity.
INTERNATIONAL JOURNAL OF MOLECULAR SCIENCES
(2022)
Article
Biochemistry & Molecular Biology
Guglielmina Chimienti, Antonella Orlando, Angela Maria Serena Lezza, Benedetta D'Attoma, Maria Notarnicola, Isabella Gigante, Vito Pesce, Francesco Russo
Summary: The study suggests that a ketogenic diet can reduce inflammation and oxidative stress, restore mitochondrial function and baseline autophagy by regulating the PPAR-gamma/PGC-1 alpha axis, which is effective in alleviating gut mitochondrial damage caused by stress.
INTERNATIONAL JOURNAL OF MOLECULAR SCIENCES
(2021)
Article
Biochemistry & Molecular Biology
Chunlong Mu, Thomas A. Tompkins, Jong M. Rho, Morris H. Scantlebury, Jane Shearer
Summary: A growing body of evidence suggests that the gut microbiota plays an important role in regulating physiological processes, particularly through the gut-brain axis to modulate neural function and metabolism. This study reveals that manipulating the gut microbiota through diet, antibiotics, and probiotics has the potential to enhance brain mitochondrial function, which could be a novel strategy for treating epileptic disorders.
BIOCHIMICA ET BIOPHYSICA ACTA-MOLECULAR BASIS OF DISEASE
(2022)
Article
Medicine, Research & Experimental
Jiyoung Oh, Chungmo Koo, Kyung Won Kim, Jin-Sung Lee
Summary: PDH deficiency is an inherited metabolic disorder caused by a defect in the pyruvate dehydrogenase complex, with stress-induced gluconeogenesis potentially contributing to rapid disease exacerbation.
MEDICAL HYPOTHESES
(2021)
Review
Nutrition & Dietetics
Sarah M. Gough, Alicia Casella, Kristen Jasmin Ortega, Abigail S. Hackam
Summary: The ketogenic diet is a high-fat low-carbohydrate diet that has been used for decades to treat metabolic disorders and refractory pediatric epilepsy. Recent research has shown that it has neuroprotective properties and can improve quality of life in patients with various neurological disorders such as multiple sclerosis and Alzheimer's disease. The major ketone bodies BHB and ACA are believed to play a key role in these beneficial effects through specific inflammatory proteins, transcription factors, and other mechanisms.
FRONTIERS IN NUTRITION
(2021)
Article
Clinical Neurology
Kristina A. Simeone, Julianne C. Wilke, Stephanie A. Matthews, Timothy A. Simeone, Jong M. Rho
Summary: The study provides evidence that metabolic therapies like the ketogenic diet may have a protective effect on reducing cell loss in the hippocampus of epileptic mice. This protection may involve mechanisms beyond just reducing seizure activity and could play a role in promoting or restoring hippocampal morphological integrity.
Article
Environmental Sciences
Ming Cong, Yuanmei Li, Hongchao Xu, Jiasen Lv, Huifeng Wu, Ye Zhao
Summary: The study revealed the toxic effects of ammonia nitrogen on clam mitochondria, showing that exposure to ammonia nitrogen induced mitochondrial swelling and structural disruption, providing potential biomarkers and control methods for managing ammonia nitrogen pollution in aquaculture.
ECOTOXICOLOGY AND ENVIRONMENTAL SAFETY
(2021)
Review
Nutrition & Dietetics
Valeria Calcaterra, Hellas Cena, Francesca Sottotetti, Chiara Hruby, Nagaia Madini, Noemi Zelaschi, Gianvincenzo Zuccotti
Summary: Polycystic ovary syndrome (PCOS) is the most common endocrine disorder in women of reproductive age and is mainly driven by hyperandrogenism, hyperinsulinism, and insulin resistance. Lifestyle and dietary interventions, particularly the quality and quantity of carbohydrates consumed, play a crucial role in the management of PCOS, and the ketogenic diet (KD) has shown promise in improving insulin resistance and carbohydrate metabolism. Further studies are needed to determine the specific effects of the very low-calorie ketogenic diet (LCKD) in adolescents with PCOS and excessive body weight. Rating: 8/10
Article
Biochemistry & Molecular Biology
Erin R. Saito, Cali E. Warren, Cameron M. Hanegan, John G. Larsen, Johannes D. du Randt, Mio Cannon, Jeremy Y. Saito, Rachel J. Campbell, Colin M. Kemberling, Gavin S. Miller, Jeffrey G. Edwards, Benjamin T. Bikman
Summary: This study investigated the effects of a ketone-supplemented ketogenic diet on mitochondrial function and cognitive performance in healthy adult mice. The results demonstrated that the ketogenic diet improved hippocampal mitochondrial efficiency, suggesting the neuroprotective effects of ketones and ketogenic diets in cognitive function.
Article
Biochemistry & Molecular Biology
A. Prabakaran, Amit Alexander
Summary: The molecular interactions and mucoadhesive nature of chitosan-coated liposomes with mucin are crucial for the development of an effective drug delivery system. The positively charged SA-CH-LPs showed stronger interaction and better mucoadhesive properties compared to negatively charged SALPs, thereby improving drug retention in the nasal cavity and enhancing therapeutic efficacy.
BIOCHIMICA ET BIOPHYSICA ACTA-GENERAL SUBJECTS
(2024)
Article
Biochemistry & Molecular Biology
Maryam Noei-Khesht Masjedi, Esmaeil Sadroddiny, Jafar Ai, Saeed Balalaie, Yazdan Asgari
Summary: This study discovered an effective strategy for cargo sorting within exosomes by incorporating an appropriate cleavage site, providing further insight into the potential of exosomes as nano-shuttles bearing therapeutic biomolecules.
BIOCHIMICA ET BIOPHYSICA ACTA-GENERAL SUBJECTS
(2024)
Article
Biochemistry & Molecular Biology
Pei -Gee Yap, Chee-Yuen Gan
Summary: This study collected the sequences of 128 tyrosinase inhibitory peptides and analyzed their hydrophobicity/hydrophilicity properties and amino acid profiles. Molecular docking analysis was used to investigate the binding interactions between peptides and tyrosinase. The study found that hydrophobic and/or polar neutral properties facilitate or stabilize peptide binding with tyrosinase, and short peptides with cysteine and tyrosine tend to bind to the active site of tyrosinase. These findings provide detailed explanations for the relationship between peptide/amino acid structures and tyrosinase inhibition, as well as potential anti-melanogenesis mechanisms for peptide-based treatments against skin hyperpigmentation.
BIOCHIMICA ET BIOPHYSICA ACTA-GENERAL SUBJECTS
(2024)
Article
Biochemistry & Molecular Biology
Ye Yang, Hai-Lian Chen, Su Fang Wu, Wei Bao
Summary: The study found that CHMP4B and VPS4A play an important role in reversing GSDMD-mediated pyroptosis by facilitating cell membrane remodeling in endometrial carcinoma.
BIOCHIMICA ET BIOPHYSICA ACTA-GENERAL SUBJECTS
(2024)
Article
Biochemistry & Molecular Biology
Aya Al Othman, Dmitry Bagrov, Julian M. Rozenberg, Olga Glazova, Gleb Skryabin, Elena Tchevkina, Alexandre Mezentsev, Mikhail Durymanov
Summary: Arc protein is found in glutamatergic neurons of vertebrates and can be transferred between neurons in extracellular vesicles (EVs). In glioma cells, Arc protein is also present in EVs and can facilitate the transfer of mRNA, potentially contributing to tumor progression and affecting synaptic plasticity in cancer patients.
BIOCHIMICA ET BIOPHYSICA ACTA-GENERAL SUBJECTS
(2024)
Article
Biochemistry & Molecular Biology
Nan Zhang, Meng-yu Shen, Qing-li Meng, Hao-ping Sun, Fang-yi Fan, Hai Yi, Yong-jian Yang
Summary: In this study, it was demonstrated for the first time that FAT1 inhibited AML proliferation by reducing autophagy level. FAT1 achieved this by decreasing the expression of ATG4B, which is related to autophagy. Mechanistically, FAT1 decreased the levels of phosphorylated and intranuclear smad2/3, thus decreasing the activity of the ATG4B gene promoter. It was also found that FAT1 competitively bound to TGF-beta R II, leading to reduced phosphorylation of TGF-beta R I and smad2/3. Knockdown of FAT1 promoted AML autophagy and proliferation in vivo.
BIOCHIMICA ET BIOPHYSICA ACTA-GENERAL SUBJECTS
(2024)
Article
Biochemistry & Molecular Biology
Oleh Demianchuk, Myroslava Vatashchuk, Dmytro Gospodaryov, Viktoria Hurza, Marian Ivanochko, Vitalii Derkachov, Vladyslav Berezovskyi, Oleh Lushchak, Kenneth B. Storey, Maria Bayliak, Volodymyr I. Lushchak
Summary: This study investigated the effects of a high-fat high-fructose diet (HFFD) on the behavior, energy metabolism, and oxidative stress markers in the cerebral cortex of mice. The results showed that HFFD stimulated locomotion and defecation, while an AKG-supplemented diet had a tendency to promote anxiety-like behavior. Additionally, there were significant differences in glutathione-dependent detoxification and processes related to autophagy between the two diets.
BIOCHIMICA ET BIOPHYSICA ACTA-GENERAL SUBJECTS
(2024)
Article
Biochemistry & Molecular Biology
Yusaku Chukai, Ginga Ito, Yasuo Miki, Koichi Wakabayashi, Ken Itoh, Eriko Sugano, Hiroshi Tomita, Tomokazu Fukuda, Taku Ozaki
Summary: The study found that mitochondrial calpain-5 plays an important role in the occurrence and development of ischemia-reperfusion injury and is expressed in the human and mouse brains. Targeting the expression or activity of mitochondrial calpain-5 may have significant implications for suppressing inflammation during I/R injuries such as cerebrovascular diseases.
BIOCHIMICA ET BIOPHYSICA ACTA-GENERAL SUBJECTS
(2024)
Article
Biochemistry & Molecular Biology
Aline Dias da Purificaca, Victor Debbas, Leonardo Yuji Tanaka, Gabriele Veronica de Mello Gabriel, Joao Wosniak Junior, Tiphany Coralie De Bessa, Sheila Garcia-Rosa, Francisco Rafael Martins Laurindo, Percillia Victoria Santos Oliveira
Summary: The ER transmembrane chaperones DNAJB12 and DNAJB14 play important roles in protein folding and ER stress response. They are regulated by thiol redox processes and are involved in ER protein reflux.
BIOCHIMICA ET BIOPHYSICA ACTA-GENERAL SUBJECTS
(2024)
Article
Biochemistry & Molecular Biology
Ekaterina O. Bryanskaya, Andrey Y. Vinokurov, Angelina I. Dolgikh, Andrey Dunaev, Plamena R. Angelova, Andrey Y. Abramov
Summary: FAD autofluorescence in cells can assess enzymatic activity, and its intensity variations may be related to different cell types and tissues. High levels of FAD autofluorescence can indicate cell pathology and potentially predict the occurrence of apoptosis and necrosis.
BIOCHIMICA ET BIOPHYSICA ACTA-GENERAL SUBJECTS
(2024)
Article
Biochemistry & Molecular Biology
Rumei Luan, Manyu Luo, Dongyan Ding, Xin Su, Junling Yang
Summary: Zinc deficiency can worsen obesity-related lung damage, and Nrf2 activation is one of the important mechanisms of this protective effect. Regulating zinc homeostasis can contribute to the prevention and treatment of obesity-related lung injury.
BIOCHIMICA ET BIOPHYSICA ACTA-GENERAL SUBJECTS
(2024)
