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http://dx.doi.org/10.1007/s00401-020-02128-8 | DOI Listing |
mBio
September 2025
Centre de Recherche du CHUM, Montreal, Québec, Canada.
HIV-1-mediated CD4 downregulation is a well-known mechanism that protects infected cells from antibody-dependent cellular cytotoxicity (ADCC). While CD4 downregulation by HIV-1 Nef and Vpu proteins has been extensively studied, the contribution of the HIV-1 envelope glycoprotein (Env) in this mechanism is less understood. While Env is known to retain CD4 in the endoplasmic reticulum (ER) through its CD4-binding site (CD4bs), little is known about the mechanisms underlying this process.
View Article and Find Full Text PDFBioresour Technol
September 2025
State Key Laboratory of Food Science and Resources, Jiangnan University, 1800 Lihu Avenue, Wuxi 214122, China; School of Biotechnology and Key Laboratory of Industrial Biotechnology Ministry of Education, Jiangnan University, 1800 Lihu Avenue, Wuxi 214122, China; International Joint Laboratory on Fo
Recombinant proteins have been widely applied in the food, biomedical, and scientific fields. Prokaryotic expression systems are preferred platforms for recombinant protein production due to their rapid growth and high protein yields. Nevertheless, disparities between recombinant expression environment and native physiological conditions frequently result in protein misfolding, leading to aggregation into non-functional inclusion bodies or proteolytic degradation.
View Article and Find Full Text PDFAnn Rheum Dis
September 2025
Department of Rheumatology and Immunology, Hannover Medical School, Hannover, Germany; Hannover Medical School, Cluster of Excellence RESIST (EXC 2155), Hannover, Germany. Electronic address:
Objectives: IκBα controls the canonical activation of NFκB. IκBα gain-of-function due to NFKBIA variants affecting the N-terminus of IκBα-especially residues 32 and 36-manifests with combined immunodeficiency. The role of NFKBIA variants affecting other IκBα domains has not been described.
View Article and Find Full Text PDFExp Eye Res
September 2025
Department of Ophthalmology, Zhengzhou University People's Hospital, Henan Provincial People's Hospital, Henan Eye Hospital, Zhengzhou, Henan, China; Henan Key Laboratory of Ophthalmology and Visual Science, Henan Eye Hospital, Henan Provincial People's Hospital, Zhengzhou, Henan, China; Eye institu
Bardet-Biedl Syndrome (BBS) is a rare autosomal recessive ciliopathy characterized by genetic heterogeneity. Despite significant progress in understanding the BBSome-coding genes associated with ciliopathies, the pathogenesis linked to mutations in chaperonin-coding genes (BBS6, BBS10, and BBS12) remains poorly defined. This study aims to confirm the genetic diagnosis of BBS and elucidate the pathological mechanisms in causative genes of BBS10 and BBS12.
View Article and Find Full Text PDFJ Biol Chem
September 2025
Department of Integrative Structural and Computational Biology, The Scripps Research Institute, La Jolla, California, USA.
Aminoacyl-tRNA synthetases (aaRSs) catalyze the aminoacylation of tRNA with their cognate amino acids, an essential step in protein biosynthesis. While biallelic mutations in aaRSs often result in severe multi-organ dysfunction accompanied by developmental delays, monoallelic mutations typically cause milder, tissue-specific symptoms. However, a de novo monoallelic nonsense mutation (R534*) in the asparaginyl-tRNA synthetase (AsnRS)-resulting in a premature stop codon and 15-residue C-terminal truncation-has been identified in multiple families and is associated with severe neurodevelopmental symptoms.
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