The farnesyl transferase inhibitor (FTI) lonafarnib improves nuclear morphology in ZMPSTE24-deficient fibroblasts from patients with the progeroid disorder MAD-B.

Child Humans Lamin Type A / genetics Progeria / drug therapy Enzyme Inhibitors / pharmacology Mutation Lipodystrophy / metabolism Fibroblasts / metabolism Transferases / genetics Metalloendopeptidases / genetics Membrane Proteins / metabolism
Atypical progeroid syndrome LMNA-C588R LMNA-D325N LMNA-E138K LMNA-M540T LMNA-R644C ZMPSTE24-L425P ZMPSTE24-P248L mandibuloacral dysplasia-type B prelamin A

Journal

Nucleus (Austin, Tex.)
ISSN: 1949-1042
Titre abrégé: Nucleus
Pays: United States
ID NLM: 101518322

Informations de publication

Date de publication:
Dec 2023
Historique:
medline: 7 12 2023
pubmed: 5 12 2023
entrez: 5 12 2023
Statut: ppublish

Résumé

Several related progeroid disorders are caused by defective post-translational processing of prelamin A, the precursor of the nuclear scaffold protein lamin A, encoded by

Identifiants

DOI: 10.1080/19491034.2023.2288476 PMID: 38050983
pubmed: 38050983
doi: 10.1080/19491034.2023.2288476
doi:

Substances chimiques

lonafarnib IOW153004F
Lamin Type A 0
Enzyme Inhibitors 0
Transferases EC 2.-
Metalloendopeptidases EC 3.4.24.-
ZMPSTE24 protein, human EC 3.4.24.84
Membrane Proteins 0

Types de publication

Journal Article

Langues

eng

Sous-ensembles de citation

IM

Pagination

2288476

Auteurs

Kamsi O Odinammadu (KO)

Department of Cell Biology, The Johns Hopkins School of Medicine, Baltimore, MD, USA.
ORCID: 0000-0003-0243-2395

Khurts Shilagardi (K)

Department of Cell Biology, The Johns Hopkins School of Medicine, Baltimore, MD, USA.

Kelsey Tuminelli (K)

The Progeria Research Foundation, Peabody, MA, USA.

Daniel P Judge (DP)

Department of Medicine, Medical University of South Carolina, Charleston, SC, USA.

Leslie B Gordon (LB)

The Progeria Research Foundation, Peabody, MA, USA.
Department of Anesthesiology, Critical Care and Pain Medicine, Boston Children's Hospital and Harvard Medical School, Boston, MA, USA.
Department of Pediatrics, Division of Genetics, Hasbro Children's Hospital and Warren Alpert Medical School of Brown University, Providence, RI, USA.

Susan Michaelis (S)

Department of Cell Biology, The Johns Hopkins School of Medicine, Baltimore, MD, USA.

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Classifications MeSH

questionsmedicales.fr › Personnes › Tranches d'âge › Enfant › The farnesyl transferase inhibitor (FTI)...
questionsmedicales.fr › Eucaryotes › Animaux › Chordés › Vertébrés › Mammifères › Eutheria › Primates › Haplorhini › Catarrhini › Hominidae › Humains › The farnesyl transferase inhibitor (FTI)...
questionsmedicales.fr › Acides aminés, peptides et protéines › Protéines › Protéines nucléaires › Protéines associées à la matrice nucléaire › Lamines › Lamine A › The farnesyl transferase inhibitor (FTI)...
questionsmedicales.fr › Maladies métaboliques et nutritionnelles › Maladies métaboliques › Erreurs innées du métabolisme › Progeria › The farnesyl transferase inhibitor (FTI)...
questionsmedicales.fr › Actions chimiques et utilisations › Actions pharmacologiques › Mécanismes moléculaires de l'action pharmacologique › Antienzymes › The farnesyl transferase inhibitor (FTI)...
questionsmedicales.fr › Phénomènes génétiques › Variation génétique › Mutation › The farnesyl transferase inhibitor (FTI)...
questionsmedicales.fr › Maladies métaboliques et nutritionnelles › Maladies métaboliques › Dermatoses métaboliques › Lipodystrophie › The farnesyl transferase inhibitor (FTI)...
questionsmedicales.fr › Cellules › Cellules du tissu conjonctif › Fibroblastes › The farnesyl transferase inhibitor (FTI)...
questionsmedicales.fr › Enzymes et coenzymes › Enzymes › Transferases › The farnesyl transferase inhibitor (FTI)...
questionsmedicales.fr › Enzymes et coenzymes › Enzymes › Hydrolases › Peptide hydrolases › Metalloproteases › Metalloendopeptidases › The farnesyl transferase inhibitor (FTI)...
questionsmedicales.fr › Acides aminés, peptides et protéines › Protéines › Protéines membranaires › The farnesyl transferase inhibitor (FTI)...