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ALG13-Related Epilepsy: Current Insights and Future Research Directions.
Neurochem Res
December 2024
Institute of Medical Sciences, General Hospital of Ningxia Medical University, Yinchuan, Ningxia Province, 750004, China.
The ALG13 gene encodes a subunit of the uridine diphosphate-N-acetylglucosamine (UDP-GlcNAc) transferase enzyme, which plays a key role in the N-linked glycosylation pathway. This pathway involves the attachment of carbohydrate structures to asparagine (Asn) residues in proteins within the endoplasmic reticulum, by which N-glycosylated proteins produced participate a wide range of processes such as electrical gradients formation and neurotransmission. Mutations in the ALG13 gene have been identified as a causative factor for congenital disorders of glycosylation (CDG) and have been frequently associated with epilepsy in affected individuals.
View Article and Find Full Text PDFA novel mutation, Ile344Asn, in microsomal triglyceride transfer protein abolishes binding to protein disulfide isomerase.
J Lipid Res
December 2024
Department of Foundations of Medicine, NYU Grossman Long Island School of Medicine, 101 Mineola Blvd., Mineola, NY, 11501, USA.
Microsomal triglyceride transfer protein (MTP) plays crucial roles in the assembly and secretion of apolipoprotein B-containing lipoproteins and loss of function MTP variants are associated with abetalipoproteinemia, a disease characterized by the absence of these lipoproteins. MTP is a heterodimeric protein of two subunits, MTP and protein disulfide isomerase (PDI). In this study, we report a proband with abetalipoproteinemia who was monitored annually over a period of ten years in her third decade and had very low plasma lipids and undetectable apoB-containing lipoproteins.
View Article and Find Full Text PDFHigh-level L-Gln compromises intestinal amino acid utilization efficiency and inhibits protein synthesis by GCN2/eIF2α/ATF4 signaling pathway in piglets fed low-crude protein diets.
Anim Nutr
December 2024
State Key Laboratory of Animal Nutrition and Feeding, Department of Companion Animal Science, China Agricultural University, Beijing 100193, China.
Gln, one of the most abundant amino acids (AA) in the body, performs a diverse range of fundamental physiological functions. However, information about the role of dietary Gln on AA levels, transporters, protein synthesis, and underlying mechanisms in vivo is scarce. The present study aimed to explore the effects of low-crude protein diet inclusion with differential doses of L-Gln on intestinal AA levels, transporters, protein synthesis, and potential mechanisms in weaned piglets.
View Article and Find Full Text PDFStructural elucidation and characterization of GH29A α-l-fucosidases and the effect of pH on their transglycosylation.
FEBS J
December 2024
Section for Protein Chemistry and Enzyme Technology, Department of Biotechnology and Biomedicine, DTU Bioengineering, Technical University of Denmark, Kgs. Lyngby, Denmark.
Article Synopsis
- GH29A α-l-fucosidases are enzymes that help break down specific sugars in glycoconjugates and can also be used to create human milk oligosaccharides (HMOs) through a process called transglycosylation.
- Researchers used bioinformatics tools and phylogenetic clustering to identify and analyze new microbial GH29A α-l-fucosidases from an underexplored group, as well as previously known enzymes, to determine their biochemical properties and behavior under different conditions.
- The study found that transglycosylation of certain substrates was most effective at neutral to alkaline pH levels and revealed new structural insights into how these enzymes function, particularly regarding regioselectivity in product formation.
Mechanism of Catalysis and Substrate Binding of Epoxyqueuosine Reductase in the Biosynthetic Pathway to Queuosine-Modified tRNA.
Biochemistry
December 2024
Department of Chemistry, University of Florida, Gainesville, Florida 32611, United States.
Post-transcriptional modifications at the anticodon stem-loop of tRNAs are key to the translation function. Metabolic pathways to these modifications often incorporate complex enzymology. A notable example is the hypermodified nucleoside, queuosine, found at the wobble position of Asn, Asp, His, and Tyr encoding tRNAs.
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