Transcriptional profiling of isogenic Friedreich ataxia neurons and effect of an HDAC inhibitor on disease signatures.

Cells, Cultured Friedreich Ataxia / genetics Gene Editing / methods Gene Expression Profiling Histone Deacetylase Inhibitors / pharmacology Humans Induced Pluripotent Stem Cells / chemistry Iron-Binding Proteins / genetics Neurons / chemistry Transcriptome Trinucleotide Repeat Expansion / genetics Frataxin
Friedreich ataxia gene editing histone deacetylase inhibitor (HDAC inhibitor) induced pluripotent stem cell (iPS cell) (iPSC) neurodegeneration neuron repeat expansion transcriptomics

Journal

The Journal of biological chemistry
ISSN: 1083-351X
Titre abrégé: J Biol Chem
Pays: United States
ID NLM: 2985121R

Informations de publication

Date de publication:
08 02 2019
Historique:
received: 02 11 2018
revised: 12 12 2018
pubmed: 16 12 2018
medline: 14 5 2019
entrez: 16 12 2018
Statut: ppublish

Résumé

Friedreich ataxia (FRDA) is a neurodegenerative disorder caused by transcriptional silencing of the frataxin (

Identifiants

DOI: 10.1074/jbc.RA118.006515 PMID: 30552117 PMC: PMC6369281
pubmed: 30552117
pii: S0021-9258(20)36833-2
doi: 10.1074/jbc.RA118.006515
pmc: PMC6369281
doi:

Substances chimiques

Histone Deacetylase Inhibitors 0
Iron-Binding Proteins 0

Types de publication

Journal Article Research Support, N.I.H., Extramural Research Support, Non-U.S. Gov't

Langues

eng

Sous-ensembles de citation

IM

Pagination

1846-1859

Subventions

Organisme : NINDS NIH HHS
ID : P30 NS062691
Pays : United States
Organisme : NIMH NIH HHS
ID : R01 MH102698
Pays : United States
Organisme : NIDCD NIH HHS
ID : R01 DC012592
Pays : United States
Organisme : NIA NIH HHS
ID : DP1 AG055944
Pays : United States
Organisme : NINDS NIH HHS
ID : R01 NS062856
Pays : United States

Informations de copyright

© 2019 Lai et al.

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Auteurs

Jiun-I Lai (JI)

From the Departments of Molecular Medicine and.

Daniel Nachun (D)

the Semel Institute for Neuroscience and Human Behavior, UCLA, Los Angeles, California 90095.

Lina Petrosyan (L)

From the Departments of Molecular Medicine and.

Benjamin Throesch (B)

Neuroscience, The Scripps Research Institute, La Jolla, California 92037 and.

Erica Campau (E)

From the Departments of Molecular Medicine and.

Fuying Gao (F)

the Semel Institute for Neuroscience and Human Behavior, UCLA, Los Angeles, California 90095.

Kristin K Baldwin (KK)

Neuroscience, The Scripps Research Institute, La Jolla, California 92037 and.

Giovanni Coppola (G)

the Semel Institute for Neuroscience and Human Behavior, UCLA, Los Angeles, California 90095.
ORCID: 0000-0003-2105-1061

Joel M Gottesfeld (JM)

From the Departments of Molecular Medicine and.
ORCID: 0000-0002-4643-5777

Elisabetta Soragni (E)

From the Departments of Molecular Medicine and soragni@scripps.edu.

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Classifications MeSH

questionsmedicales.fr › Cellules › Cellules cultivées › Transcriptional profiling of isogenic...
questionsmedicales.fr › Maladies métaboliques et nutritionnelles › Maladies métaboliques › Maladies mitochondriales › Ataxie de Friedreich › Transcriptional profiling of isogenic...
questionsmedicales.fr › Techniques d'investigation › Techniques génétiques › Génie génétique › Édition de gène › Transcriptional profiling of isogenic...
questionsmedicales.fr › Techniques d'investigation › Techniques génétiques › Analyse de profil d'expression de gènes › Transcriptional profiling of isogenic...
questionsmedicales.fr › Actions chimiques et utilisations › Actions pharmacologiques › Mécanismes moléculaires de l'action pharmacologique › Antienzymes › Inhibiteurs de désacétylase d'histone › Transcriptional profiling of isogenic...
questionsmedicales.fr › Eucaryotes › Animaux › Chordés › Vertébrés › Mammifères › Eutheria › Primates › Haplorhini › Catarrhini › Hominidae › Humains › Transcriptional profiling of isogenic...
questionsmedicales.fr › Cellules › Cellules souches › Cellules souches pluripotentes › Cellules souches pluripotentes induites › Transcriptional profiling of isogenic...
questionsmedicales.fr › Acides aminés, peptides et protéines › Protéines › Métalloprotéines › Protéines de liaison au fer › Transcriptional profiling of isogenic...
questionsmedicales.fr › Cellules › Neurones › Transcriptional profiling of isogenic...
questionsmedicales.fr › Phénomènes génétiques › Structures génétiques › Transcriptome › Transcriptional profiling of isogenic...
questionsmedicales.fr › Phénomènes génétiques › Mutagenèse › Expansion de séquence répétée de l'ADN › Expansion de trinucléotide répété › Transcriptional profiling of isogenic...