A. Bonaud

A mouse model of cardiac AL amyloidosis unveils mechanisms of tissue accumulation and toxicity of amyloid fibrils

This paper develops a mouse model of cardiac AL amyloidosis that reproduces human disease features, including amyloid deposits and cardiac dysfunction, and identifies a role for partial LC degradation in fibril formation.

Plasma cell heterogeneity is driven by type of immune challenge

This study identifies five plasma cell subsets in humans and mice, showing their heterogeneity is driven by immune challenge type and B cell origin.

Disruption of immunoglobulin heavy and light chain assembly by antisense oligonucleotides impairs protein homeostasis and myeloma cell survival

This study shows that antisense oligonucleotides targeting IgG mRNA reduce IgG secretion in myeloma cells, leading to toxic free-light chain accumulation and cell death.

CXCR4 signaling strength regulates hematopoietic multipotent progenitor fate through extrinsic and intrinsic mechanisms

This study shows that CXCR4 signaling strength influences hematopoietic multipotent progenitor fate through both extrinsic and intrinsic mechanisms, with implications for myeloid skewing in WHIM Syndrome.

Plasma cell maintenance and antibody secretion are under the control of Sec22b-mediated regulation of organelle dynamics

This study identifies Sec22b as a critical regulator of plasma cell maintenance and function by controlling organelle dynamics, leading to reduced antibody secretion and impaired immune response.

New models to study plasma cells in mouse based on the restriction of IgJ expression to antibody secreting cells.

The paper introduces two new mouse models, IgJGFP and IgJCreERT2, for studying plasma cells by leveraging IgJ expression as a marker for antibody-secreting cells.

Glomerulosclerosis and kidney failure in a mouse model of monoclonal immunoglobulin light-chain deposition disease

This study develops a mouse model of light chain deposition disease, showing that pathogenic light chains cause glomerulosclerosis and kidney failure, and identifies potential therapeutic targets.

Cxcr4 desensitization is an essential regulatory mechanism controlling the extra-follicular B cell response.

This study shows that CXCR4 desensitization is crucial for regulating B cell activation, plasma cell differentiation, and migration in the extra-follicular response.