A5034764311- RNA Research and Splicing
- RNA modifications and cancer
- Prion Diseases and Protein Misfolding
- Protein Structure and Dynamics
- Ubiquitin and proteasome pathways
- Microbial Metabolic Engineering and Bioproduction
- Alzheimer's disease research and treatments
- Amyotrophic Lateral Sclerosis Research
- Peptidase Inhibition and Analysis
- Biofuel production and bioconversion
- Nuclear Structure and Function
- RNA regulation and disease
- Supramolecular Self-Assembly in Materials
- Enzyme Catalysis and Immobilization
- Signaling Pathways in Disease
Universitat Autònoma de Barcelona
2023-2025
Abstract hnRNPDL is a ribonucleoprotein (RNP) involved in transcription and RNA-processing that hosts missense mutations causing limb-girdle muscular dystrophy D3 (LGMD D3). Mammalian-specific alternative splicing (AS) renders three natural isoforms, hnRNPDL-2 being predominant humans. We present the cryo-electron microscopy structure of full-length amyloid fibrils, which are stable, non-toxic, bind nucleic acids. The high-resolution core consists single Gly/Tyr-rich highly hydrophilic...
The MIA40 relay system mediates the import of small cysteine-rich proteins into intermembrane mitochondrial space (IMS). substrates are synthesized in cytosol and assumed to be disordered their reduced state this compartment. As they cross outer membrane, promotes oxidation critical native disulfides facilitate folding, trapping functional species IMS. Here, we study redox-controlled folding TRIAP1, a protein with moonlighting function: regulating phospholipid trafficking between membranes...
ABSTRACT hnRNPDL is a ribonucleoprotein (RNP) involved in transcription and RNA-processing, with missense mutations causing limb-girdle muscular dystrophy-3 (LGMDD3). Mammalian-specific alternative splicing (AS) renders three natural isoforms, hnRNPDL-2 being predominant humans. We present the cryo-electron microscopy structure of full-length amyloid fibrils, which are stable, non-toxic, bind nucleic acids, RNA binding domains building solenoidal coat around them. The core consists single...