Modeling Skeletal Muscle Laminopathies Using Human Induced Pluripotent Stem Cells Carrying Pathogenic LMNA Mutations

Modeling Skeletal Muscle Laminopathies Using Human Induced Pluripotent Stem Cells Carrying Pathogenic LMNA Mutations

Laminopathies are a clinically heterogeneous group of disorders caused by mutations in LMNA. The main proteins encoded by LMNA are Lamin A and C, which together with Lamin B1 and B2, form the nuclear lamina: a mesh-like structure located underneath the inner nuclear membrane. Laminopathies show stri...

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Bibliographic Details
Main Authors: Heather B. Steele-Stallard, Luca Pinton, Shilpita Sarcar, Tanel Ozdemir, Sara M. Maffioletti, Peter S. Zammit, Francesco Saverio Tedesco
Format: Article
Language:English
Published: Frontiers Media S.A. 2018-10-01
Series:Frontiers in Physiology
Subjects:
lamin A/C
LMNA
skeletal muscle
laminopathies
muscular dystrophy
iPSCs
Online Access:https://www.frontiersin.org/article/10.3389/fphys.2018.01332/full

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