A5051266678- Prion Diseases and Protein Misfolding
- Amyotrophic Lateral Sclerosis Research
- Alzheimer's disease research and treatments
- Neurogenetic and Muscular Disorders Research
- Trace Elements in Health
- Neurological diseases and metabolism
- Amino Acid Enzymes and Metabolism
- Muscle Physiology and Disorders
- Neuroinflammation and Neurodegeneration Mechanisms
- Ion channel regulation and function
- Parkinson's Disease Mechanisms and Treatments
- Cellular transport and secretion
- Biotin and Related Studies
- RNA regulation and disease
- Ubiquitin and proteasome pathways
- Venomous Animal Envenomation and Studies
- Fungal and yeast genetics research
- Mitochondrial Function and Pathology
- Enzyme Structure and Function
- Neuroscience and Neuropharmacology Research
- RNA modifications and cancer
- Autophagy in Disease and Therapy
- Cancer-related molecular mechanisms research
- Cancer-related Molecular Pathways
- Biochemical Acid Research Studies
University of Padua
2016-2025
Istituto delle Scienze Neurologiche di Bologna
2020
Institute of Neurological Sciences
2020
Istituto Zooprofilattico Sperimentale delle Venezie
2020
Parkin, an E3 ubiquitin ligase and a Parkinson's disease (PD) related gene, translocates to impaired mitochondria drives their elimination via autophagy, process known as mitophagy. Mitochondrial pro-fusion protein Mitofusins (Mfn1 Mfn2) were found be target for Parkin mediated ubiquitination. Mfns are transmembrane GTPase embedded in the outer membrane of mitochondria, which required on adjacent mediate fusion. In mammals, Mfn2 also forms complexes that capable tethering endoplasmic...
Long non-coding RNAs (lncRNAs) are emerging as important players in the regulation of several aspects cellular biology. For a better comprehension their function, it is fundamental to determine tissue or cell specificity and identify subcellular localization. In fact, activity lncRNAs may vary according compartmentalization. Myofibers smallest complete contractile system skeletal muscle influencing its contraction velocity metabolism. How expressed different myofibers, participate metabolism...
J. Neurochem. (2011) 116 , 881–890. Abstract The cellular prion protein (PrP C ) is a cell‐surface glycoprotein mainly expressed in the CNS. structural conversion of PrP generates prion, infectious agent causing transmissible spongiform encephalopathies, which are rare and fatal diseases affecting animals humans. Despite decades intensive research, mechanism prion‐associated neurodegeneration physiologic role still obscure. Recent evidence, however, supports hypothesis that may be involved...
The prion protein (PrP(C)) is a cell surface glycoprotein mainly expressed in neurons, whose misfolded isoforms generate the responsible for incurable neurodegenerative disorders. Whereas PrP(C) involvement propagation well established, physiological function still enigmatic despite suggestions that it could act signal transduction by modulating phosphorylation cascades and Ca(2+) homeostasis. Because binds neurotoxic aggregates with high-affinity, has also been proposed acts as receptor...
TDP-43 is a nuclear protein involved in pivotal processes, extensively studied for its implication neurodegenerative disorders. cytosolic inclusions are common neuropathologic hallmark amyotrophic lateral sclerosis (ALS) and related diseases, it now established that misfolding aggregation play key role their etiopathology. neurotoxic mechanisms not yet clarified, but the identification of proteins able to modulate TDP-43-mediated damage may be promising therapeutic targets proteinopathies....
Parkinson's disease (PD) is a neurodegenerative caused by multifactorial pathogenic mechanisms. Familial PD linked with genetic mutations in genes whose products are either associated mitochondrial function or endo-lysosomal pathways. Of note, mitochondria essential to sustain high energy demanding synaptic activity of neurons and alterations Ca2+ signaling have been proposed as causal events for process, although the mechanisms responsible selective loss specific neuronal populations...
Approximately 25% of eukaryotic proteins possessing homology to at least two transmembrane domains are predicted be embedded in biological membranes. Nevertheless, this group is not usually well represented proteome-wide experiments due their refractory nature. Here we present a quantitative mass spectrometry-based comparison membrane protein expression cerebellar granule neurons grown primary culture that were isolated from wild-type mice and lacking the cellular prion protein. This...
The cellular prion protein (PrPC) whose conformational misfolding leads to the production of deadly prions, has a still-unclarified function despite decades intensive research. Following our recent finding that PrPC limits Ca2+ entry via store-operated channels in neurons, we investigated whether could also control activity ionotropic glutamate receptors (iGluRs). To this end, compared local movements primary cerebellar granule neurons and cortical transduced with genetically encoded probes...
Snake venom phospholipases A2 (PLA2s) have sequences and structures very similar to those of mammalian group I II secretory PLA2s, but they possess many toxic properties, ranging from the inhibition coagulation blockage nerve transmission, induction muscle necrosis. The biological properties these proteins are not only due their enzymatic activity, also protein–protein interactions which still unidentified. Here, we compare sequence alignments snake grouped according structure looking for...
Amyotrophic lateral sclerosis (ALS) is a fatal neurodegenerative disorder characterized by the selective death of motor neurons (MNs), probably combination cell- and non-cell-autonomous processes. The past decades have brought many important insights into role astrocytes in nervous system function disease, including implication ALS pathogenesis possibly through impairment Ca
Amyotrophic lateral sclerosis (ALS) is a progressive neurodegenerative disease whose pathophysiology largely unknown. Despite the fact that motor neuron (MN) death recognized as key event in ALS, astrocytes dysfunctionalities and neuroinflammation were demonstrated to accompany probably even drive MN loss. Nevertheless, mechanisms priming astrocyte failure hyperactivation are still obscure. In this work, altered pathways molecules ALS unveiled by investigating proteomic profile secreted...
The Saccharomyces cerevisiae atypical protein kinase Bud32p is a member of the nuclear endopeptidase‐like, kinase, chromatin‐associated/kinase, endopeptidase‐like and other small size (EKC/KEOPS) complex, known to be involved in control transcription telomere homeostasis. Complex subunits (Pcc1p, Pcc2p, Cgi121p, Kae1p) represent, however, subset proteins able interact with Bud32p, suggesting that this may endowed additional roles unrelated its participation EKC/KEOPS complex. In context, we...
Mitochondria-ER contacts (MERCs), tightly regulated by numerous tethering proteins that act as molecular and functional connections between the two organelles, are essential to maintain a variety of cellular functions. Such often compromised in early stages many neurodegenerative disorders, including amyotrophic lateral sclerosis (ALS). TDP-43, nuclear protein mainly involved RNA metabolism, has been repeatedly associated with ALS pathogenesis other diseases. Although TDP-43...