Modeling RTT Syndrome by iPSC-Derived Neurons from Male and Female Patients with Heterogeneously Severe Hot-Spot MECP2 Variants.

Rett syndrome X inactivation mosaicism early morphological neuronal biomarkers genotype-phenotype correlation hot-spot MECP2 pathogenic variants iPSC-neurons mature neuron e-recordings

Journal

International journal of molecular sciences
ISSN: 1422-0067
Titre abrégé: Int J Mol Sci
Pays: Switzerland
ID NLM: 101092791

Informations de publication

Date de publication:
21 Nov 2022
Historique:
received: 11 10 2022
revised: 07 11 2022
accepted: 15 11 2022
entrez: 26 11 2022
pubmed: 27 11 2022
medline: 30 11 2022
Statut: epublish

Résumé

Rett syndrome caused by

Identifiants

pubmed: 36430969
pii: ijms232214491
doi: 10.3390/ijms232214491
pmc: PMC9697612
pii:
doi:

Types de publication

Journal Article

Langues

eng

Sous-ensembles de citation

IM

Subventions

Organisme : AIRETT
ID : 08A_621 and 08A_801

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Auteurs

Sara Perego (S)

Experimental Research Laboratory of Medical Cytogenetics and Molecular Genetics, IRCCS Istituto Auxologico Italiano, Via Ariosto 13, 20145 Milan, Italy.

Valentina Alari (V)

Experimental Research Laboratory of Medical Cytogenetics and Molecular Genetics, IRCCS Istituto Auxologico Italiano, Via Ariosto 13, 20145 Milan, Italy.

Gianluca Pietra (G)

Laboratory of Biology BIO@SNS, Scuola Normale Superiore, Piazza dei Cavalieri, 7, 56126 Pisa, Italy.
Institute of Neuroscience, National Research Council (CNR), Via Giuseppe Moruzzi, 1, 56124 Pisa, Italy.

Andrea Lamperti (A)

Experimental Research Laboratory of Medical Cytogenetics and Molecular Genetics, IRCCS Istituto Auxologico Italiano, Via Ariosto 13, 20145 Milan, Italy.

Alessandro Vimercati (A)

Experimental Research Laboratory of Medical Cytogenetics and Molecular Genetics, IRCCS Istituto Auxologico Italiano, Via Ariosto 13, 20145 Milan, Italy.

Nicole Camporeale (N)

Experimental Research Laboratory of Medical Cytogenetics and Molecular Genetics, IRCCS Istituto Auxologico Italiano, Via Ariosto 13, 20145 Milan, Italy.

Maria Garzo (M)

Experimental Research Laboratory of Medical Cytogenetics and Molecular Genetics, IRCCS Istituto Auxologico Italiano, Via Ariosto 13, 20145 Milan, Italy.

Francesca Cogliati (F)

Experimental Research Laboratory of Medical Cytogenetics and Molecular Genetics, IRCCS Istituto Auxologico Italiano, Via Ariosto 13, 20145 Milan, Italy.

Donatella Milani (D)

Fondazione IRCCS Ca Granda Ospedale Maggiore, Policlinico, Via Francesco Sforza, 28, 20122 Milan, Italy.

Aglaia Vignoli (A)

Department of Health Sciences, Università degli Studi di Milano, Struttura Complessa di Neuropsichiatria dell'Infazia e Adolescenza ASST GOM Niguarda, Piazza Ospedale Maggiore, 3, 20162 Milan, Italy.

Angela Peron (A)

Child Neuropsychiatry Unit-Epilepsy Center, Department of Health Sciences, ASST Santi Paolo e Carlo, San Paolo Hospital, Università Degli Studi di Milano, 20142 Milan, Italy.

Lidia Larizza (L)

Experimental Research Laboratory of Medical Cytogenetics and Molecular Genetics, IRCCS Istituto Auxologico Italiano, Via Ariosto 13, 20145 Milan, Italy.

Tommaso Pizzorusso (T)

Laboratory of Biology BIO@SNS, Scuola Normale Superiore, Piazza dei Cavalieri, 7, 56126 Pisa, Italy.
Institute of Neuroscience, National Research Council (CNR), Via Giuseppe Moruzzi, 1, 56124 Pisa, Italy.

Silvia Russo (S)

Experimental Research Laboratory of Medical Cytogenetics and Molecular Genetics, IRCCS Istituto Auxologico Italiano, Via Ariosto 13, 20145 Milan, Italy.

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Classifications MeSH