From the Light Chain Sequence to the Tissue Microenvironment: Contribution of the Mesangial Cells to Glomerular Amyloidosis
Abstract
:1. Introduction
2. Genetic and Structural Characteristics of the Immunoglobulin LCs
3. Structural Factors and Environmental Conditions That Drive the Amyloid Aggregation of the LCs
3.1. Pro-Fibrillogenic Sequences in LCs
3.2. The Folding Stability and Role of the Somatic Mutations
3.3. Contribution of the VL Gene Segment to the Propensity of the LCs to Form Amyloid
3.4. How Does the LC Protect Itself from Aggregation?
3.5. Intact LC as the AL Precursor and the Role of Proteolysis
3.6. Environmental Factors Modulating the Propensity of LCs to Form Amyloid
4. The Structure of AL Fibrils
5. Mechanism of Misfolding and Assembly of the LC into Amyloid Fibrils
6. Role of the Mesangial Cells in Glomerular Amyloidosis: Are They an Amyloid Factory?
7. Challenges in AL Amyloidosis Research
8. Conclusions
Author Contributions
Funding
Institutional Review Board Statement
Informed Consent Statement
Acknowledgments
Conflicts of Interest
References
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Del Pozo-Yauner, L.; Turbat-Herrera, E.A.; Pérez-Carreón, J.I.; Herrera, G.A. From the Light Chain Sequence to the Tissue Microenvironment: Contribution of the Mesangial Cells to Glomerular Amyloidosis. Hemato 2022, 3, 232-267. https://doi.org/10.3390/hemato3010019
Del Pozo-Yauner L, Turbat-Herrera EA, Pérez-Carreón JI, Herrera GA. From the Light Chain Sequence to the Tissue Microenvironment: Contribution of the Mesangial Cells to Glomerular Amyloidosis. Hemato. 2022; 3(1):232-267. https://doi.org/10.3390/hemato3010019
Chicago/Turabian StyleDel Pozo-Yauner, Luis, Elba A. Turbat-Herrera, Julio I. Pérez-Carreón, and Guillermo A. Herrera. 2022. "From the Light Chain Sequence to the Tissue Microenvironment: Contribution of the Mesangial Cells to Glomerular Amyloidosis" Hemato 3, no. 1: 232-267. https://doi.org/10.3390/hemato3010019