Farnesyltransferase inhibition in HGPS.

Enzyme Inhibitors / pharmacology Farnesyltranstransferase / antagonists & inhibitors Humans Molecular Targeted Therapy Progeria / drug therapy

Journal

Cell
ISSN: 1097-4172
Titre abrégé: Cell
Pays: United States
ID NLM: 0413066

Informations de publication

Date de publication:
21 01 2021
Historique:
entrez: 22 1 2021
pubmed: 23 1 2021
medline: 27 8 2021
Statut: ppublish

Résumé

The ultra-rare, pediatric premature aging disorder Hutchinson-Gilford progeria syndrome (HGPS) is caused by mutation of LMNA, encoding the nuclear architectural protein lamin A. Patients develop atherosclerosis and typically die of heart failure in their teens. FDA-approved Zokinvy prevents farnesylation of lamin A, reduces vascular stiffness, and extends survival in HGPS patients. To view this Bench to Bedside, open or download the PDF.

Identifiants

DOI: 10.1016/j.cell.2020.12.029 PMID: 33482093
pubmed: 33482093
pii: S0092-8674(20)31751-7
doi: 10.1016/j.cell.2020.12.029
pii:
doi:

Substances chimiques

Enzyme Inhibitors 0
Farnesyltranstransferase EC 2.5.1.29

Types de publication

Journal Article

Langues

eng

Sous-ensembles de citation

IM

Pagination

293

Informations de copyright

Copyright © 2020. Published by Elsevier Inc.

Auteurs

Tom Misteli (T)

National Cancer Institute, NIH, Bethesda, MD, USA. Electronic address: tom.misteli@nih.gov.

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Classifications MeSH

questionsmedicales.fr › Actions chimiques et utilisations › Actions pharmacologiques › Mécanismes moléculaires de l'action pharmacologique › Antienzymes › Farnesyltransferase inhibition in HGPS.
questionsmedicales.fr › Enzymes et coenzymes › Enzymes › Transferases › Alkyl et aryl transferases › Farnesyltranstransferase › Farnesyltransferase inhibition in HGPS.
questionsmedicales.fr › Eucaryotes › Animaux › Chordés › Vertébrés › Mammifères › Eutheria › Primates › Haplorhini › Catarrhini › Hominidae › Humains › Farnesyltransferase inhibition in HGPS.
questionsmedicales.fr › Thérapeutique › Traitement médicamenteux › Thérapie moléculaire ciblée › Farnesyltransferase inhibition in HGPS.
questionsmedicales.fr › Maladies métaboliques et nutritionnelles › Maladies métaboliques › Erreurs innées du métabolisme › Progeria › Farnesyltransferase inhibition in HGPS.