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Cryo-EM reveals the molecular basis oflaminin polymerization and LN-lamininopathies.


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.

SUBMITTER: Kulczyk AW 

PROVIDER: S-EPMC9852560 | biostudies-literature | 2023 Jan

REPOSITORIES: biostudies-literature

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Cryo-EM reveals the molecular basis oflaminin polymerization and LN-lamininopathies.

Kulczyk Arkadiusz W AW   McKee Karen K KK   Zhang Ximo X   Bizukojc Iwona I   Yu Ying Q YQ   Yurchenco Peter D PD  

Nature communications 20230119 1


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. ...[more]

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