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  1. Article ; Online: Monitoring Nrf2/ARE Pathway Activity with a New Zebrafish Reporter System.

    Badenetti, Lorenzo / Manzoli, Rosa / Rubin, Michela / Cozza, Giorgio / Moro, Enrico

    International journal of molecular sciences

    2023  Volume 24, Issue 7

    Abstract: Among multiple cytoprotective mechanisms, eukaryotic cells exhibit a complex transcriptional program relying on the Nrf2 transcription factor, which is generally recruited upon biological stressors including oxidative-stress-based cellular insults. The ... ...

    Abstract Among multiple cytoprotective mechanisms, eukaryotic cells exhibit a complex transcriptional program relying on the Nrf2 transcription factor, which is generally recruited upon biological stressors including oxidative-stress-based cellular insults. The relevance of this master regulator has remarkably emerged in recent years in several research fields such as cancer, inflammatory disorders and age-related neurological diseases. Here, we document the generation and characterization of a novel Nrf2/ARE pathway biosensor fish which exhibits a dynamic spatiotemporal expression profile during the early developmental stages. The transgenic line is responsive to known Nrf2 pathway modulators but also to Edaravone, which direct activity on the Nrf2 pathway has never been documented in a live transgenic fish model. We also show that the reporter is faithfully activated during fin regeneration, and its degree of expression is slightly affected in a glucocerebrosidase (Gba1) morphant zebrafish model. Therefore, this novel transgenic fish may represent a valuable tool to be exploited for the characterization of zebrafish models of human diseases, as well as for primary high-throughput drug screening.
    MeSH term(s) Animals ; Humans ; Zebrafish/genetics ; Zebrafish/metabolism ; NF-E2-Related Factor 2/genetics ; NF-E2-Related Factor 2/metabolism ; Oxidative Stress/genetics ; Animals, Genetically Modified/genetics ; Antioxidants/metabolism ; Zebrafish Proteins/metabolism
    Chemical Substances NF-E2-Related Factor 2 ; Antioxidants ; Zebrafish Proteins
    Language English
    Publishing date 2023-04-06
    Publishing country Switzerland
    Document type Journal Article
    ZDB-ID 2019364-6
    ISSN 1422-0067 ; 1422-0067 ; 1661-6596
    ISSN (online) 1422-0067
    ISSN 1422-0067 ; 1661-6596
    DOI 10.3390/ijms24076804
    Database MEDical Literature Analysis and Retrieval System OnLINE

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  2. Article ; Online: Lysosomal Function and Axon Guidance: Is There a Meaningful Liaison?

    Manzoli, Rosa / Badenetti, Lorenzo / Rubin, Michela / Moro, Enrico

    Biomolecules

    2021  Volume 11, Issue 2

    Abstract: Axonal trajectories and neural circuit activities strongly rely on a complex system of molecular cues that finely orchestrate the patterning of neural commissures. Several of these axon guidance molecules undergo continuous recycling during brain ... ...

    Abstract Axonal trajectories and neural circuit activities strongly rely on a complex system of molecular cues that finely orchestrate the patterning of neural commissures. Several of these axon guidance molecules undergo continuous recycling during brain development, according to incompletely understood intracellular mechanisms, that in part rely on endocytic and autophagic cascades. Based on their pivotal role in both pathways, lysosomes are emerging as a key hub in the sophisticated regulation of axonal guidance cue delivery, localization, and function. In this review, we will attempt to collect some of the most relevant research on the tight connection between lysosomal function and axon guidance regulation, providing some proof of concepts that may be helpful to understanding the relation between lysosomal storage disorders and neurodegenerative diseases.
    MeSH term(s) Autophagy ; Axon Guidance ; Axons/metabolism ; Brain/metabolism ; Endosomes/metabolism ; Ephrins/metabolism ; Humans ; Lysosomal Storage Diseases/metabolism ; Lysosomes/chemistry ; Lysosomes/metabolism ; Netrins/metabolism ; Neurodegenerative Diseases/metabolism ; Neurons/metabolism ; Semaphorins/metabolism
    Chemical Substances Ephrins ; Netrins ; Semaphorins
    Language English
    Publishing date 2021-01-29
    Publishing country Switzerland
    Document type Journal Article ; Research Support, Non-U.S. Gov't ; Review
    ZDB-ID 2701262-1
    ISSN 2218-273X ; 2218-273X
    ISSN (online) 2218-273X
    ISSN 2218-273X
    DOI 10.3390/biom11020191
    Database MEDical Literature Analysis and Retrieval System OnLINE

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  3. Article ; Online: A novel CRISPR/Cas9-based iduronate-2-sulfatase (IDS) knockout human neuronal cell line reveals earliest pathological changes.

    Badenetti, Lorenzo / Manzoli, Rosa / Trevisan, Marta / D'Avanzo, Francesca / Tomanin, Rosella / Moro, Enrico

    Scientific reports

    2023  Volume 13, Issue 1, Page(s) 10289

    Abstract: Multiple complex intracellular cascades contributing to Hunter syndrome (mucopolysaccharidosis type II) pathogenesis have been recognized and documented in the past years. However, the hierarchy of early cellular abnormalities leading to irreversible ... ...

    Abstract Multiple complex intracellular cascades contributing to Hunter syndrome (mucopolysaccharidosis type II) pathogenesis have been recognized and documented in the past years. However, the hierarchy of early cellular abnormalities leading to irreversible neuronal damage is far from being completely understood. To tackle this issue, we have generated two novel iduronate-2-sulfatase (IDS) loss of function human neuronal cell lines by means of genome editing. We show that both neuronal cell lines exhibit no enzymatic activity and increased GAG storage despite a completely different genotype. At a cellular level, they display reduced differentiation, significantly decreased LAMP1 and RAB7 protein levels, impaired lysosomal acidification and increased lipid storage. Moreover, one of the two clones is characterized by a marked decrease of the autophagic marker p62, while none of the two mutants exhibit marked oxidative stress and mitochondrial morphological changes. Based on our preliminary findings, we hypothesize that neuronal differentiation might be significantly affected by IDS functional impairment.
    MeSH term(s) Humans ; Iduronic Acid ; CRISPR-Cas Systems ; Iduronate Sulfatase/genetics ; Iduronate Sulfatase/metabolism ; Mucopolysaccharidosis II/genetics ; Cell Line
    Chemical Substances Iduronic Acid (3402-98-0) ; Iduronate Sulfatase (EC 3.1.6.13)
    Language English
    Publishing date 2023-06-25
    Publishing country England
    Document type Journal Article ; Research Support, Non-U.S. Gov't
    ZDB-ID 2615211-3
    ISSN 2045-2322 ; 2045-2322
    ISSN (online) 2045-2322
    ISSN 2045-2322
    DOI 10.1038/s41598-023-37138-5
    Database MEDical Literature Analysis and Retrieval System OnLINE

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  4. Article ; Online: Mucopolysaccharidosis type II zebrafish model exhibits early impaired proteasomal-mediated degradation of the axon guidance receptor Dcc.

    Manzoli, Rosa / Badenetti, Lorenzo / Bruzzone, Matteo / Macario, Maria Carla / Rubin, Michela / Dal Maschio, Marco / Roveri, Antonella / Moro, Enrico

    Cell death & disease

    2024  Volume 15, Issue 4, Page(s) 269

    Abstract: Most of the patients affected by neuronopathic forms of Mucopolysaccharidosis type II (MPS II), a rare lysosomal storage disorder caused by defects in iduronate-2-sulfatase (IDS) activity, exhibit early neurological defects associated with white matter ... ...

    Abstract Most of the patients affected by neuronopathic forms of Mucopolysaccharidosis type II (MPS II), a rare lysosomal storage disorder caused by defects in iduronate-2-sulfatase (IDS) activity, exhibit early neurological defects associated with white matter lesions and progressive behavioural abnormalities. While neuronal degeneration has been largely described in experimental models and human patients, more subtle neuronal pathogenic defects remain still underexplored. In this work, we discovered that the axon guidance receptor Deleted in Colorectal Cancer (Dcc) is significantly dysregulated in the brain of ids mutant zebrafish since embryonic stages. In addition, thanks to the establishment of neuronal-enriched primary cell cultures, we identified defective proteasomal degradation as one of the main pathways underlying Dcc upregulation in ids mutant conditions. Furthermore, ids mutant fish-derived primary neurons displayed higher levels of polyubiquitinated proteins and P62, suggesting a wider defect in protein degradation. Finally, we show that ids mutant larvae display an atypical response to anxiety-inducing stimuli, hence mimicking one of the characteristic features of MPS II patients. Our study provides an additional relevant frame to MPS II pathogenesis, supporting the concept that multiple developmental defects concur with early childhood behavioural abnormalities.
    MeSH term(s) Animals ; Axon Guidance ; Brain/metabolism ; Iduronate Sulfatase/metabolism ; Mucopolysaccharidosis II/metabolism ; Nervous System Diseases/pathology ; Zebrafish/metabolism
    Chemical Substances Iduronate Sulfatase (EC 3.1.6.13) ; dcc protein, zebrafish
    Language English
    Publishing date 2024-04-16
    Publishing country England
    Document type Journal Article
    ZDB-ID 2541626-1
    ISSN 2041-4889 ; 2041-4889
    ISSN (online) 2041-4889
    ISSN 2041-4889
    DOI 10.1038/s41419-024-06661-2
    Database MEDical Literature Analysis and Retrieval System OnLINE

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  5. Article ; Online: Knockout of the

    Theodoridi, Antonia / Dinarello, Alberto / Badenetti, Lorenzo / Pavlidis, Michail / Dalla Valle, Luisa / Tsalafouta, Aleka

    International journal of molecular sciences

    2021  Volume 22, Issue 22

    Abstract: The Hsd11b2 enzyme converts cortisol into its inactive form, cortisone and regulates cortisol levels, in particular in response to stress. Taking advantage of CRISPR/Cas9 technology, we generated ... ...

    Abstract The Hsd11b2 enzyme converts cortisol into its inactive form, cortisone and regulates cortisol levels, in particular in response to stress. Taking advantage of CRISPR/Cas9 technology, we generated a
    MeSH term(s) 11-beta-Hydroxysteroid Dehydrogenase Type 2/genetics ; Animals ; Female ; Gene Knockout Techniques ; Homozygote ; Humans ; Hydrocortisone/adverse effects ; Hydrocortisone/pharmacology ; Larva/genetics ; Male ; Stress, Physiological/genetics ; Zebrafish/genetics ; Zebrafish/growth & development
    Chemical Substances 11-beta-Hydroxysteroid Dehydrogenase Type 2 (EC 1.1.1.146) ; Hydrocortisone (WI4X0X7BPJ)
    Language English
    Publishing date 2021-11-20
    Publishing country Switzerland
    Document type Journal Article
    ZDB-ID 2019364-6
    ISSN 1422-0067 ; 1422-0067 ; 1661-6596
    ISSN (online) 1422-0067
    ISSN 1422-0067 ; 1661-6596
    DOI 10.3390/ijms222212525
    Database MEDical Literature Analysis and Retrieval System OnLINE

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  6. Article ; Online: Zebrafish Mutant Lines Reveal the Interplay between

    Dinarello, Alberto / Tesoriere, Annachiara / Martini, Paolo / Fontana, Camilla Maria / Volpato, Davide / Badenetti, Lorenzo / Terrin, Francesca / Facchinello, Nicola / Romualdi, Chiara / Carnevali, Oliana / Dalla Valle, Luisa / Argenton, Francesco

    International journal of molecular sciences

    2022  Volume 23, Issue 5

    Abstract: Glucocorticoids mainly exert their biological functions through their cognate receptor, encoded by ... ...

    Abstract Glucocorticoids mainly exert their biological functions through their cognate receptor, encoded by the
    MeSH term(s) Animals ; Glucocorticoids/metabolism ; Receptors, Glucocorticoid/metabolism ; Receptors, Mineralocorticoid/metabolism ; Transcription, Genetic ; Zebrafish/metabolism
    Chemical Substances Glucocorticoids ; Receptors, Glucocorticoid ; Receptors, Mineralocorticoid
    Language English
    Publishing date 2022-02-28
    Publishing country Switzerland
    Document type Journal Article
    ZDB-ID 2019364-6
    ISSN 1422-0067 ; 1422-0067 ; 1661-6596
    ISSN (online) 1422-0067
    ISSN 1422-0067 ; 1661-6596
    DOI 10.3390/ijms23052678
    Database MEDical Literature Analysis and Retrieval System OnLINE

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