Nuclear envelope and striated muscle diseases.
Maria Chatzifrangkeskou1, Gisèle Bonne2, Antoine Muchir1
1Sorbonne Universités, UPMC Univ Paris 06, Center of Research in Myology, UMRS 974, F-75013 Paris, France; INSERM U974, F-75013 Paris, France; CNRS FRE 3617, F-75013 Paris, France; Institut de Myologie, F-75013 Paris, France.
Current Opinion in Cell Biology
|October 8, 2014
Summary
Mutations in nuclear lamins, proteins forming the nuclear lamina, are linked to inherited diseases. This review highlights their role in striated muscle disorders, detailing nuclear envelope architecture.
Area of Science:
- Cell Biology
- Genetics
- Biochemistry
Background:
- The nuclear lamina is a protein network (lamins) lining the inner nuclear membrane.
- Mutations in lamins and inner nuclear membrane proteins cause inherited diseases.
Purpose of the Study:
- To summarize nuclear envelope architecture.
- To present findings on lamins' role in striated muscle disorders.
Main Methods:
- Literature review
- Summary of existing research
Main Results:
- Nuclear lamina structure and function are crucial.
- Lamins are implicated in various inherited muscle conditions.
Conclusions:
- Understanding nuclear lamina is key to comprehending muscle disease pathogenesis.
- Further research into laminopathies is warranted.
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