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

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

ABSTRACTSeveral related progeroid disorders are caused by defective post-translational processing of prelamin A, the precursor of the nuclear scaffold protein lamin A, encoded by LMNA. Prelamin A undergoes farnesylation and additional modifications at its C-terminus. Subsequently, the farnesylated C...

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Bibliographic Details
Main Authors: Kamsi O. Odinammadu, Khurts Shilagardi, Kelsey Tuminelli, Daniel P. Judge, Leslie B. Gordon, Susan Michaelis
Format: Article
Language:English
Published: Taylor & Francis Group 2023-12-01
Series:Nucleus
Subjects:
Atypical progeroid syndrome
LMNA-C588R
LMNA-D325N
LMNA-E138K
LMNA-M540T
LMNA-R644C
Online Access:https://www.tandfonline.com/doi/10.1080/19491034.2023.2288476

Internet

https://www.tandfonline.com/doi/10.1080/19491034.2023.2288476

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