Does aberrant architecture of nuclear LINC complex stop muscle cell development?

作者：Fidzianska Anna; Glinka Zofia^*; Kwinta Przemko; Tomasik Tomasz; Pietrzyk Jacek J

来源：Clinical Neuropathology, 2015, 34(3): 136-140.

DOI：10.5414/NP300825

• LING complex disruption
• muscle cell immaturity
• separation of ONM from INM
• nesprin
• lamin B

The linker of nucleoskeleton and cytoskeleton (LINC) complex is a structure that spans the entire nuclear envelope (NE) and integrates the nuclear interior with the cytoskeleton. Lamin A/C together with nesprins that mainly reside along the inner nuclear membrane (INM) and outer nuclear membrane (ONM) are core components of the LINC complex. Integrity of this specific nuclear structure is critical for muscle cell maturation and function. In the present study, we analyzed the ultrastructure of the LINC complex observed in two neonates with severe hypotonia and respiratory distress. Disruption of the LINC complex manifests in a wide separation of the ONM from the NM; the loss of perinuclear space (PNS) and delayed muscle cell maturation were predominating findings. This nuclear phenomenon has never been reported and provides further support for the appearance of a neonatal fowl of laminopathy.

• 出版日期2015-6