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Article ; Online: Basic notions about gene therapy from the nucleic acid perspective and applications in a pediatric disease: Duchenne muscular dystrophy.

Richard, Isabelle

Archives de pediatrie : organe officiel de la Societe francaise de pediatrie

2023 Volume 30, Issue 8S1, Page(s) 8S2–8S11

Abstract: Gene therapy involves the introduction of genetic material into cells as a therapeutic molecule to cure a disease. Through the transfer of specific nucleic acid to the target tissue, gene expression can be downregulated, augmented, or corrected thanks to ...

Abstract	Gene therapy involves the introduction of genetic material into cells as a therapeutic molecule to cure a disease. Through the transfer of specific nucleic acid to the target tissue, gene expression can be downregulated, augmented, or corrected thanks to the nucleic acid sequence as a support of gene expression. This is achieved through molecular interactions according to the sequence arrangement or the secondary structure of the molecules or through their catalytic properties. Over the past two decades, the rapid advances of knowledge and technologies in gene therapy have led to the development of different strategies and to the extension of its use to numerous indications, including certain cancers. Major success has been achieved in clinical trials and the field of gene therapy is booming. Several gene therapy products are now on the market in Europe, the United States, and China. In this review, we cover the basic principles of gene therapy and the characteristics of the main vectors used to transfer genetic material into the cell. As an example of applications, we address the various strategies applied to a rare pediatric muscle disease: Duchenne muscular dystrophy. © 2023 Published by Elsevier Masson SAS on behalf of French Society of Pediatrics.
MeSH term(s)	Humans ; Child ; Muscular Dystrophy, Duchenne/genetics ; Muscular Dystrophy, Duchenne/therapy ; Nucleic Acids ; Genetic Therapy ; China ; Europe
Chemical Substances	Nucleic Acids
Language	English
Publishing date	2023-10-17
Publishing country	France
Document type	Review ; Journal Article
ZDB-ID	1181947-9
ISSN	1769-664X ; 0929-693X
ISSN (online)	1769-664X
ISSN	0929-693X
DOI	10.1016/S0929-693X(23)00221-X
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Article ; Online: Context-induced renewal of passive but not active coping behaviours in the shock-probe defensive burying task.

Brown, Alexa / Martins, Melissa / Richard, Isabelle / Chaudhri, Nadia

Learning & behavior

2023 Volume 51, Issue 4, Page(s) 468–481

Abstract: Renewal is the return of extinguished responding after removal from the extinction context. Renewal has been extensively studied using classical aversive conditioning procedures that measure a passive freezing response to an aversive conditioned stimulus. ...

Abstract	Renewal is the return of extinguished responding after removal from the extinction context. Renewal has been extensively studied using classical aversive conditioning procedures that measure a passive freezing response to an aversive conditioned stimulus. However, coping responses to aversive stimuli are complex and can be reflected in passive and active behaviours. Using the shock-probe defensive burying task, we investigated whether different coping responses are susceptible to renewal. During conditioning, male, Long-Evans rats were placed into a specific context (Context A) where an electrified shock-probe delivered a 3 mA shock upon contact. During extinction, the shock-probe was unarmed in either the same (Context A) or a different context (Context B). Renewal of conditioned responses was assessed in the conditioning context (ABA) or in a novel context (ABC or AAB). Renewal of passive coping responses, indicated by an increased latency and a decreased duration of shock-probe contacts, was observed in all groups. However, renewal of passive coping, measured by increased time spent on the side of the chamber opposite the shock-probe, was only found in the ABA group. Renewal of active coping responses linked to defensive burying was not observed in any group. The present findings highlight the presence of multiple psychological processes underlying even basic forms of aversive conditioning and demonstrate the importance of assessing a broader set of behaviours to tease apart these different underlying mechanisms. The current findings suggest that passive coping responses may be more reliable indicators for assessing renewal than active coping behaviours associated with defensive burying.
MeSH term(s)	Rats ; Male ; Animals ; Rats, Long-Evans ; Conditioning, Classical/physiology ; Conditioning, Psychological ; Extinction, Psychological/physiology
Language	English
Publishing date	2023-04-24
Publishing country	United States
Document type	Journal Article ; Research Support, Non-U.S. Gov't
ZDB-ID	2048665-0
ISSN	1543-4508 ; 1543-4494
ISSN (online)	1543-4508
ISSN	1543-4494
DOI	10.3758/s13420-023-00583-3
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Article: [No title information]

Richard, Isabelle / Le Bouler, Stéphane

Recherche en soins infirmiers

2021 Volume 139, Issue 4, Page(s) 8–11

Abstract: In France, the lack of inclusion of nursing education in universities has effectively prevented it from producing knowledge through research. As the recruitment of teacher-researchers is a key issue in university integration, the public authorities have ... ...

Title translation	La création de nouvelles sections du Conseil national des universités.
Abstract	In France, the lack of inclusion of nursing education in universities has effectively prevented it from producing knowledge through research. As the recruitment of teacher-researchers is a key issue in university integration, the public authorities have decided to open new qualification paths for individuals to become university professors or lecturers in nursing, rehabilitation, and rehabilitation sciences and maieutic.Creating teams of teacher-researchers is important for research production, internal governance at universities, and the organization of the network of training institutes. Research should be considered for its contribution to the improvement of care practices but also in terms of educational investment.It is important to distinguish between the professional and disciplinary registers. At master's level and a fortiori at doctoral level, only the approach in terms of research fields can exist, on a disciplinary and, increasingly, interdisciplinary level.The creation of new disciplines has a twofold impact : it is equivalent to institutionally recognizing the research fields concerned, and it enhances universities' involvement in paramedical training. Focusing on these two areas-research and pedagogy on the one hand, and organization on the other-is one of the keys to university integration.
Language	French
Publishing date	2021-07-11
Publishing country	France
Document type	English Abstract ; Journal Article
ISSN	0297-2964
ISSN	0297-2964
DOI	10.3917/rsi.139.0008
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Article ; Online: Calpaïnopathies - État des lieux et perspectives thérapeutiques.

Malfatti, Edoardo / Richard, Isabelle

Medecine sciences : M/S

2021 Volume 36 Hors série n° 2, Page(s) 17–21

Abstract: Calpainopathies are inherited limb-girdle muscular dystrophies, most often following an autosomal recessive (AR) transmission. Autosomal dominant (AD) forms with less severe presentation are increasingly reported. Calpainopathies with autosomal recessive ...

Title translation	Calpainopathies: state of the art and therapeutic perspectives.
Abstract	Calpainopathies are inherited limb-girdle muscular dystrophies, most often following an autosomal recessive (AR) transmission. Autosomal dominant (AD) forms with less severe presentation are increasingly reported. Calpainopathies with autosomal recessive (AR) mutations of the calpain3 gene (CAPN3) are associated with limb girdle muscular dystrophy type R1 (LGMD-R1, OMIM 253600) also referred to as LGMD-2A according to the old nomenclature. LGMD-R1 is the commonest form of all LGMDs, with an estimated prevalence of 10 to 70 cases per million inhabitants, that is a cohort of between 670 and 4,200 patients in France theoritically. Patients present a symmetrical proximal axial myopathy manifesting itself between the first and second decade. The clinical course is variable. The level of Creatine- Kinase (CK) is usually high and there is no cardiac involvement. From a therapeutic perspective, the autosomal recessive form of calpainopathy is quite suitable to gene replacement strategies; the viability of recombinant AAV-mediated calpain 3 transfer has been demonstrated in animal models and clinical trials are expected in the coming years. Meanwhile, natural history studies are needed to prepare for future clinical trials.
MeSH term(s)	Calpain/deficiency ; Calpain/genetics ; Diagnosis, Differential ; France/epidemiology ; Humans ; Muscle Proteins/deficiency ; Muscle Proteins/genetics ; Muscular Dystrophies, Limb-Girdle/diagnosis ; Muscular Dystrophies, Limb-Girdle/epidemiology ; Muscular Dystrophies, Limb-Girdle/genetics ; Muscular Dystrophies, Limb-Girdle/therapy ; Mutation ; Phenotype ; Targeted Gene Repair/methods ; Targeted Gene Repair/trends ; Therapies, Investigational/methods ; Therapies, Investigational/trends
Chemical Substances	Muscle Proteins ; CAPN3 protein, human (EC 3.4.22.-) ; Calpain (EC 3.4.22.-)
Language	French
Publishing date	2021-01-11
Publishing country	France
Document type	Journal Article ; Review
ZDB-ID	632733-3
ISSN	1958-5381 ; 0767-0974
ISSN (online)	1958-5381
ISSN	0767-0974
DOI	10.1051/medsci/2020244
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Article ; Online: Service learning for health students: do it the French way.

Levaillant, Mathieu / Tran, Béatrice / Lerolle, Nicolas / Richard, Isabelle / Vaillant, Loïc

Lancet (London, England)

2023 Volume 402, Issue 10415, Page(s) 1833

MeSH term(s)	Humans ; Students ; Learning
Language	English
Publishing date	2023-08-05
Publishing country	England
Document type	Letter
ZDB-ID	3306-6
ISSN	1474-547X ; 0023-7507 ; 0140-6736
ISSN (online)	1474-547X
ISSN	0023-7507 ; 0140-6736
DOI	10.1016/S0140-6736(23)01413-7
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Article: La création de nouvelles sections du Conseil national des universités.

Richard, Isabelle / Le Bouler, Stéphane

Recherche en soins infirmiers

2020 , Issue 139, Page(s) 8–11

Title translation	The creation of new branches of the French National Council of Universities.
Abstract	In France, the lack of inclusion of nursing education in universities has effectively prevented it from producing knowledge through research. As the recruitment of teacher-researchers is a key issue in university integration, the public authorities have decided to open new qualification paths for individuals to become university professors or lecturers in nursing, rehabilitation, and rehabilitation sciences and maieutic.Creating teams of teacher-researchers is important for research production, internal governance at universities, and the organization of the network of training institutes. Research should be considered for its contribution to the improvement of care practices but also in terms of educational investment.It is important to distinguish between the professional and disciplinary registers. At master's level and a fortiori at doctoral level, only the approach in terms of research fields can exist, on a disciplinary and, increasingly, interdisciplinary level.The creation of new disciplines has a twofold impact : it is equivalent to institutionally recognizing the research fields concerned, and it enhances universities' involvement in paramedical training. Focusing on these two areas-research and pedagogy on the one hand, and organization on the other-is one of the keys to university integration.
MeSH term(s)	Education, Nursing/organization & administration ; France ; Humans ; Nursing Research/education ; Universities/organization & administration
Language	French
Publishing date	2020-05-05
Publishing country	France
Document type	Journal Article
ISSN	0297-2964
ISSN	0297-2964
DOI	10.3917/rsi.139.0008
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Article: Mitochondrial MicroRNAs Contribute to Macrophage Immune Functions Including Differentiation, Polarization, and Activation.

Duroux-Richard, Isabelle / Apparailly, Florence / Khoury, Maroun

Frontiers in physiology

2021 Volume 12, Page(s) 738140

Abstract: A subset of microRNA (miRNA) has been shown to play an important role in mitochondrial (mt) functions and are named MitomiR. They are present within or associated with mitochondria. Most of the mitochondrial miRNAs originate from the nucleus, while a ... ...

Abstract	A subset of microRNA (miRNA) has been shown to play an important role in mitochondrial (mt) functions and are named MitomiR. They are present within or associated with mitochondria. Most of the mitochondrial miRNAs originate from the nucleus, while a very limited number is encoded by mtDNA. Moreover, the miRNA machinery including the Dicer and Argonaute has also been detected within mitochondria. Recent, literature has established a close relationship between miRNAs and inflammation. Indeed, specific miRNA signatures are associated with macrophage differentiation, polarization and functions. Nevertheless, the regulation of macrophage inflammatory pathways governed specifically by MitomiR and their implication in immune-mediated inflammatory disorders remain poorly studied. Here, we propose a hypothesis in which MitomiR play a key role in triggering macrophage differentiation and modulating their downstream activation and immune functions. We sustain this proposition by bioinformatic data obtained from either the human monocytic THP1 cell line or the purified mitochondrial fraction of PMA-induced human macrophages. Interestingly, 22% of the 754 assayed miRNAs were detected in the mitochondrial fraction and are either exclusively or highly enriched cellular miRNA. Furthermore, the
Language	English
Publishing date	2021-11-03
Publishing country	Switzerland
Document type	Journal Article
ZDB-ID	2564217-0
ISSN	1664-042X
ISSN	1664-042X
DOI	10.3389/fphys.2021.738140
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Article: De L’ANRS à L’ANRS | MIE (Maladies infectieuses émergentes) : comment la France adapte sa réponse aux maladies émergentes.

Richard, Isabelle / Sicard, Jean-François / Yazdanpanah, Yazdan

Virologie (Montrouge, France)

2021 Volume 25, Issue 4, Page(s) 193–196

Title translation	From ANRS to ANRS \| MIE (Maladies infectieuses émergentes) : How France is adapting its response to emerging diseases.
MeSH term(s)	Communicable Diseases, Emerging ; France/epidemiology
Language	French
Publishing date	2021-09-01
Publishing country	France
Document type	Journal Article
ZDB-ID	2118387-9
ISSN	1950-6961 ; 1267-8694
ISSN (online)	1950-6961
ISSN	1267-8694
DOI	10.1684/vir.2021.0911
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Article ; Online: Assessment of Therapeutic Potential of a Dual AAV Approach for Duchenne Muscular Dystrophy.

Albini, Sonia / Palmieri, Laura / Dubois, Auriane / Bourg, Nathalie / Lostal, William / Richard, Isabelle

International journal of molecular sciences

2023 Volume 24, Issue 14

Abstract: Duchenne muscular dystrophy (DMD) is a yet incurable rare genetic disease that affects the skeletal and cardiac muscles, leading to progressive muscle wasting and premature death. DMD is caused by the lack of dystrophin, a muscle protein essential for ... ...

Abstract	Duchenne muscular dystrophy (DMD) is a yet incurable rare genetic disease that affects the skeletal and cardiac muscles, leading to progressive muscle wasting and premature death. DMD is caused by the lack of dystrophin, a muscle protein essential for the biochemical support and integrity of muscle fibers. Gene replacement strategies for Duchenne muscular dystrophy (DMD) employing the adeno-associated virus (AAV) face the challenge imposed by the limited packaging capacity of AAV, only allowing the accommodation of a short version of dystrophin (µDys) that is still far removed from correcting human disease. The need to develop strategies leading to the expression of a best performing dystrophin variant led to only few studies reporting on the use of dual vectors, but none reported on a method to assess in vivo transgene reconstitution efficiency, the degree of which directly affects the use of safe AAV dosing. We report here on the generation of a dual AAV vector approach for the expression of a larger dystrophin version (quasidystrophin) based on homologous recombination, and the development of a methodology employing a strategic droplet digital PCR design, to determine the recombination efficiency as well as the occurrence of unwanted concatemerization events or aberrant expression from the single vectors. We demonstrated that, upon systemic delivery in the dystrophic D2.B10-Dmd
MeSH term(s)	Mice ; Humans ; Animals ; Muscular Dystrophy, Duchenne/genetics ; Muscular Dystrophy, Duchenne/therapy ; Muscular Dystrophy, Duchenne/metabolism ; Dystrophin/genetics ; Dystrophin/metabolism ; Dependovirus/genetics ; Dependovirus/metabolism ; Muscle, Skeletal/metabolism ; Mice, Inbred mdx ; Genetic Vectors/genetics
Chemical Substances	Dystrophin
Language	English
Publishing date	2023-07-13
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/ijms241411421
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Article: Accueil de l'urgence en unité de soins palliatifs.

Richard, Isabelle

Revue de l'infirmiere

2013 , Issue 187, Page(s) 23–25

Abstract: Emergency admission to a palliative care unit. The emergency admission of a patient to a palliative care unit requires availability, trust in the professionals who request the admission and the conviction that this care is essential for the patient's ... ...

Title translation	Emergency admission to a palliative care unit.
Abstract	Emergency admission to a palliative care unit. The emergency admission of a patient to a palliative care unit requires availability, trust in the professionals who request the admission and the conviction that this care is essential for the patient's overall care. An example with the palliative care unit of Montfermeil hospital.
MeSH term(s)	Communication ; Hospital Units ; Humans ; Palliative Care ; Patient Admission ; Patient Care Team
Language	French
Publishing date	2013-01
Publishing country	France
Document type	English Abstract ; Journal Article
ZDB-ID	632538-5
ISSN	1293-8505 ; 0397-7900
ISSN	1293-8505 ; 0397-7900
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