A Novel Ornithine Aminotransferase Splice Site Mutation Causes Vitamin B6-Responsive Gyrate Atrophy
CONCLUSION: Our knowledge about the diagnosis and treatment of GACR can be improved by identifying novel mutations in the OAT gene and accurate follow-up of the patients to determine how they respond to treatment.PMID:38638626 | PMC:PMC11022026 | DOI:10.18502/jovr.v19i1.15446 (Source: Journal of Ophthalmic and Vision Research)
Source: Journal of Ophthalmic and Vision Research - April 19, 2024 Category: Opthalmology Authors: Samira Molaei Ramshe Safoura Zardadi Elham Alehabib Ramin Nourinia Javad Jamshidi Mohsen Soosanabadi Hossein Darvish Source Type: research

Enantioselective Bioaccumulation and Toxicological Effects of Chiral Neonicotinoid Sulfoxaflor in Rats
In this study, toxicokinetics, bioaccumulation, tissue distribution and enantiomeric profiles of sulfoxaflor in rats were investigated through single oral exposure and 28-days continuous exposure experiment. Sulfoxaflor mainly accumulated in liver and kidney, and the (-)-2R,3R-sufloxaflor and (-)-2S,3R-sufloxaflor had higher enrichment than their enantiomers in rats. The toxicological effects were evaluated after 28-days exposure. Slight inflammation in liver and kidney were observed by histopathology. Sphingolipid, amino acid, and vitamin B6 metabolism pathways were significantly disturbed in metabonomics analysis. These ...
Source: Chemosphere - April 18, 2024 Category: Chemistry Authors: Yaofeng Fang Shengchen Lv Shouchun Xiao Haonan Hou Jianing Yao Yue Cao Bingying He Xueke Liu Peng Wang Donghui Liu Zhiqiang Zhou Source Type: research

Enantioselective Bioaccumulation and Toxicological Effects of Chiral Neonicotinoid Sulfoxaflor in Rats
In this study, toxicokinetics, bioaccumulation, tissue distribution and enantiomeric profiles of sulfoxaflor in rats were investigated through single oral exposure and 28-days continuous exposure experiment. Sulfoxaflor mainly accumulated in liver and kidney, and the (-)-2R,3R-sufloxaflor and (-)-2S,3R-sufloxaflor had higher enrichment than their enantiomers in rats. The toxicological effects were evaluated after 28-days exposure. Slight inflammation in liver and kidney were observed by histopathology. Sphingolipid, amino acid, and vitamin B6 metabolism pathways were significantly disturbed in metabonomics analysis. These ...
Source: Chemosphere - April 18, 2024 Category: Chemistry Authors: Yaofeng Fang Shengchen Lv Shouchun Xiao Haonan Hou Jianing Yao Yue Cao Bingying He Xueke Liu Peng Wang Donghui Liu Zhiqiang Zhou Source Type: research

Targeting PNPO to suppress tumor growth via inhibiting autophagic flux and to reverse paclitaxel resistance in ovarian cancer
In conclusion, PNPO has a regulatory effect on lysosomal biogenesis that in turn promotes autophagic flux, leadin g to OC cell proliferation, and tumor formation, and is a paclitaxel-resistant factor. These data imply a potential application by targeting PNPO to suppress tumor growth and reverse PTX resistance in OC. (Source: Apoptosis)
Source: Apoptosis - April 13, 2024 Category: Molecular Biology Source Type: research

Validating nutrient selection for product-group-specific nutrient indices for use as functional units in life cycle assessment of foods
Br J Nutr. 2024 Apr 12:1-23. doi: 10.1017/S0007114524000709. Online ahead of print.ABSTRACTThe ability to provide adequate nutrition is considered a key factor in evaluating the sustainability of foods and diets. Nutrient indices are used as functional units (FUs) in life cycle assessment of foods to include nutritional performance in the environmental assessment of a product. Several general and food group-specific nutrient indices exist but many lack validation, particularly when used as FU. In addition, the nutrient selection strategies and reference units for nutrient intake can vary considerably among studies. To vali...
Source: The British Journal of Nutrition - April 12, 2024 Category: Nutrition Authors: Anna K årlund Venla Kytt ä Tiina Pellinen Hanna L Tuomisto Anne-Maria Pajari Marjukka Kolehmainen Merja Saarinen Source Type: research

Validating nutrient selection for product-group-specific nutrient indices for use as functional units in life cycle assessment of foods
Br J Nutr. 2024 Apr 12:1-23. doi: 10.1017/S0007114524000709. Online ahead of print.ABSTRACTThe ability to provide adequate nutrition is considered a key factor in evaluating the sustainability of foods and diets. Nutrient indices are used as functional units (FUs) in life cycle assessment of foods to include nutritional performance in the environmental assessment of a product. Several general and food group-specific nutrient indices exist but many lack validation, particularly when used as FU. In addition, the nutrient selection strategies and reference units for nutrient intake can vary considerably among studies. To vali...
Source: The British Journal of Nutrition - April 12, 2024 Category: Nutrition Authors: Anna K årlund Venla Kytt ä Tiina Pellinen Hanna L Tuomisto Anne-Maria Pajari Marjukka Kolehmainen Merja Saarinen Source Type: research

Validating nutrient selection for product-group-specific nutrient indices for use as functional units in life cycle assessment of foods
Br J Nutr. 2024 Apr 12:1-23. doi: 10.1017/S0007114524000709. Online ahead of print.ABSTRACTThe ability to provide adequate nutrition is considered a key factor in evaluating the sustainability of foods and diets. Nutrient indices are used as functional units (FUs) in life cycle assessment of foods to include nutritional performance in the environmental assessment of a product. Several general and food group-specific nutrient indices exist but many lack validation, particularly when used as FU. In addition, the nutrient selection strategies and reference units for nutrient intake can vary considerably among studies. To vali...
Source: The British Journal of Nutrition - April 12, 2024 Category: Nutrition Authors: Anna K årlund Venla Kytt ä Tiina Pellinen Hanna L Tuomisto Anne-Maria Pajari Marjukka Kolehmainen Merja Saarinen Source Type: research

Biochemical, structural, and computational analyses of two new clinically identified missense mutations of ALDH7A1
We report a biochemical, structural, and computational study of two novel ALDH7A1 missense mutations that were identified in a child with rare recurrent seizures from the third month of life. The mutations affect two residues in the oligomer interfaces of ALDH7A1, Arg134 and Arg441 (Arg162 and Arg469 in the HGVS nomenclature). The corresponding enzyme variants R134S and R441C (p.Arg162Ser and p.Arg469Cys in the HGVS nomenclature) were expressed in Escherichia coli and purified. R134S and R441C have 10,000- and 50-fold lower catalytic efficiency than wild-type ALDH7A1, respectively. Sedimentation velocity analytical ultrace...
Source: Chemico-Biological Interactions - April 11, 2024 Category: Molecular Biology Authors: David A Korasick David P Buckley Alessandra Palpacelli Ida Cursio Elisabetta Cesaroni Jianlin Cheng John J Tanner Source Type: research

Biochemical, structural, and computational analyses of two new clinically identified missense mutations of ALDH7A1
We report a biochemical, structural, and computational study of two novel ALDH7A1 missense mutations that were identified in a child with rare recurrent seizures from the third month of life. The mutations affect two residues in the oligomer interfaces of ALDH7A1, Arg134 and Arg441 (Arg162 and Arg469 in the HGVS nomenclature). The corresponding enzyme variants R134S and R441C (p.Arg162Ser and p.Arg469Cys in the HGVS nomenclature) were expressed in Escherichia coli and purified. R134S and R441C have 10,000- and 50-fold lower catalytic efficiency than wild-type ALDH7A1, respectively. Sedimentation velocity analytical ultrace...
Source: Chemico-Biological Interactions - April 11, 2024 Category: Molecular Biology Authors: David A Korasick David P Buckley Alessandra Palpacelli Ida Cursio Elisabetta Cesaroni Jianlin Cheng John J Tanner Source Type: research

Myricanol improves metabolic profiles in dexamethasone induced lipid and protein metabolism disorders in mice
This study for the first time applied widely targeted metabolomics to investigate the beneficial effects of MY on glucose, lipids, and protein metabolism in DEX-induced metabolic abnormality in mice. The results showed that MY significantly reversed DEX-induced soleus and gastrocnemius muscle weight loss, muscle fiber damage, and muscle strength loss. MY alleviated DEX-induced metabolic disorders by increasing SIRT1 and glucose transporter type 4 (GLUT4) expressions. Additionally, myricanol prevented muscle cell apoptosis and atrophy by inhibiting caspase 3 cleavages and muscle ring-finger protein-1 (MuRF1) expression. Met...
Source: Atherosclerosis - April 7, 2024 Category: Cardiology Authors: Tiandan Li Xiaochao Hu Lingyang Fan Yong Yang Kai He Source Type: research