Spatial-proteomics reveals recombinant human laminin-111 restores adhesion-signaling and metabolic function to laminin-α2 deficient muscle

Laminin-α2-related Congenital Muscular Dystrophy (LAMA2-CMD) is a severe neuromuscular disorder caused by mutations in the LAMA2 gene, leading to loss of heterotrimers laminin-211/221, key components of the skeletal muscle extracellular matrix. Their absence disrupts adhesion between the cytoskeleton and extracellular matrix, resulting in progressive muscle wasting. Laminin-211/221 interacts with adhesion complexes such as the dystrophin/utrophin glycoprotein complexes and α7β1-integrins. However, the regulatory mechanisms of these laminin-binding complexes and the broader role of laminin’s influence on the formation of the macromolecular network in skeletal muscle remain unclear. We previously demonstrated that delivering mouse laminin-111 to the dyW⁻/⁻ mouse model of LAMA2-CMD prevented disease progression, improved strength, and extended survival. We hypothesize that laminin-111, the embryonic laminin isoform, restores key adhesion-signaling networks. Using spatial proteomics on pati..., , , # Spatial-proteomics reveals recombinant human laminin-111 restores adhesion-signaling and metabolic function to laminin-α2 deficient muscle

[https://doi.org/10.5061/dryad.j0zpc86qw](https://doi.org/10.5061/dryad.j0zpc86qw)

## Description of the data and file structure

### **Supplemental tables used for data analysis**

* **Quality Control and Normalization:** Data normalization was performed using NanoString’s GeoMx DSP analysis suite, utilizing the geometric mean of negative control proteins (IgG variants) for normalization. Column A lists the experimental group used in this study (control/unaffected human versus LAMA2-CMD), and Column B contains the ROI identification associated with each skeletal muscle biopsy from the patients. Columns C to VJ then list the proteins from the GeoMx IPA panel (row 1) and the normalized GeoMx IPA panel \"counts\" found for each ROI. Sheet 1 has the values for the Area ROIs, and sheet 2 contains the data for the single-fiber ROIs. 
* These values we...,