نتایج جستجو برای: protein misfolding

تعداد نتایج: 1235138  

Journal: :Journal of visualized experiments : JoVE 2013
Ido Karady Anna Frumkin Shiran Dror Netta Shemesh Nadav Shai Anat Ben-Zvi

The folding and assembly of proteins is essential for protein function, the long-term health of the cell, and longevity of the organism. Historically, the function and regulation of protein folding was studied in vitro, in isolated tissue culture cells and in unicellular organisms. Recent studies have uncovered links between protein homeostasis (proteostasis), metabolism, development, aging, an...

2014
Cesira de Chiara Annalisa Pastore

Understanding how proteins protect themselves from aberrant aggregation is of primary interest for understanding basic biology, protein biochemistry, and human disease. We discuss the paradigmatic example of ataxin-1 (Atx1), the protein responsible for neurodegenerative spinocerebellar ataxia type 1 (SCA1). This disease is part of the increasing family of pathologies caused by protein aggregati...

Journal: :Bioorganic & Medicinal Chemistry Letters 2013

Journal: :Journal of Cellular Biochemistry 2006

Journal: :The Journal of biological chemistry 2010
Rafal A Bartoszewski Michael Jablonsky Sylwia Bartoszewska Lauren Stevenson Qun Dai John Kappes James F Collawn Zsuzsa Bebok

Recent advances in our understanding of translational dynamics indicate that codon usage and mRNA secondary structure influence translation and protein folding. The most frequent cause of cystic fibrosis (CF) is the deletion of three nucleotides (CTT) from the cystic fibrosis transmembrane conductance regulator (CFTR) gene that includes the last cytosine (C) of isoleucine 507 (Ile507ATC) and th...

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