Cryo-EM reveals the molecular basis oflaminin polymerization and LN-lamininopathies.
basic_science · Level V
Where this comes from
- Record sourced from PubMed, PMID 36658135.
- Also identified by DOI 10.1038/s41467-023-36077-z and PMC identifier 9852560.
- Licence recorded as CC BY.
- The licence permits redistribution, so the abstract is shown in full and the full text is available from the publisher.
Abstract
Laminin polymerization is the major step in basement membranes assembly. Its failures cause laminin N-terminal domain lamininopathies including Pierson syndrome. We have employed cryo-electron microscopy to determine a 3.7 Å structure of the trimeric laminin polymer node containing α1, β1 and γ1 subunits. The structure reveals the molecular basis of calcium-dependent formation of laminin lattice, and provides insights into polymerization defects manifesting in human disease.
Medical subject headings
- Nephrotic Syndrome
- Pupil Disorders