1. Volume 91, Issue 2, Pages 423-434 (February 2017)
Unravelling the immunopathological mechanisms of heavy chain deposition disease with implications for clinical management 
Frank Bridoux, Vincent Javaugue, Sébastien Bender, Fannie Leroy, Pierre Aucouturier, Céline Debiais-Delpech, Jean-Michel Goujon, Nathalie Quellard, Amélie Bonaud, Marie Clavel, Patrick Trouillas, Florent Di Meo, Jean-Marc Gombert, Jean-Paul Fermand, Arnaud Jaccard, Michel Cogné, Guy Touchard, Christophe Sirac 
Kidney International 
Volume 91, Issue 2, Pages (February 2017)
DOI: /j.kint
Copyright © 2016 International Society of Nephrology Terms and Conditions

2. Figure 1
Kidney biopsy sample from patient 1 with γ1-heavy chain deposition disease. (a) Light microscopy (periodic–acid Schiff staining, original magnification ×200). Section of renal cortex showing nodular glomerulosclerosis and diffuse tubular basement membrane thickening. Bar = 50 μm. (b) Immunofluorescence microscopy (fluorescein isothiocyanate–conjugate antisera to γ1-heavy chain, original magnification ×200). Linear deposits in the mesangium, along the glomerular and tubular basement membranes, and Bowman’s capsule. No significant staining was observed with κ and λ light chains or α and μ heavy chains (not shown). Bar = 50 μm. (c,d) Indirect immunofluorescence (original magnification ×400) showed positive staining with anti-γCH2 antibody (c) but not with anti-γCH1 antibody (d). Bar = 50 μm. (e,f) Electron microscopy. (e) Enlarged multilayered tubular basement membrane with electron-dense powdery deposits (arrows) (original magnification ×2500). Bar = 2 μm. (f) Linear electron-dense deposits that predominate in the inner aspect of the glomerular basement membrane (original magnification ×10,000). Bar = 1 μm. Inset: immunoelectron microscopy (original magnification ×50,000). Presence of anti-γ1 heavy- chain–conjugated gold particles along the inner aspect of glomerular basement membrane. Bar = 0.5 μm.
Kidney International  , DOI: ( /j.kint )
Copyright © 2016 International Society of Nephrology Terms and Conditions

3. Figure 2
Kidney biopsy sample from patient 15 with α heavy chain deposition disease. Light microscopy. (a) Periodic acid-Schiff staining (original magnification ×400). Section of renal cortex showing nodular glomerulosclerosis with diffuse mesangial nodular deposits and moderate mesangial hypercellularity. Bar = 50 μm. (b) Marinozzi silver staining (magnification ×200). Note the presence of double contours of the glomerular basement membranes, mesangiolysis with aneurysmal dilation of the capillary lumens, and tubular basement membrane thickening. Bar = 50 μm. Immunofluorescence microscopy with anti-α (c) and anti-κ (d) conjugates (original magnification ×400). Linear α heavy chain deposits were observed in the mesangium and along the tubular basement membranes (c), whereas no significant staining was seen with κ (c) and λ light chains or γ and μ heavy chains (not shown). Bar = 50 μm. (e) Electron microscopy (original magnification ×10,000). Linear electron-dense deposits (arrows) involving the outer aspect of the tubular basement membrane. Bar = 1 μm. (f) Immunoelectron microscopy (original magnification ×50,000). The presence of anti-α heavy chain–conjugated gold particles along the glomerular basement membrane. Gold particles were predominantly observed along the inner aspect of the glomerular basement membrane. Bar = 0.5 μm.
Kidney International  , DOI: ( /j.kint )
Copyright © 2016 International Society of Nephrology Terms and Conditions

4. Figure 3
Bone marrow biopsy. Immunofluorescence microscopy (original magnification ×600) with fluorescein isothiocyanate–conjugate antisera to γ-heavy chain (a) and phycoerythrin-conjugate antisera to λ-light chain (b). Coexpression of both heavy chain and light chain by a single plasma cell clone (c). Bar = 50 μm.
Kidney International  , DOI: ( /j.kint )
Copyright © 2016 International Society of Nephrology Terms and Conditions

5. Figure 4
Deduced amino-acid sequences of heavy chain variable regions compared with germline sequences according to IMGT numbering. Mutated amino acids are indicated by shaded squares.
Kidney International  , DOI: ( /j.kint )
Copyright © 2016 International Society of Nephrology Terms and Conditions

6. Figure 5
Calculated isoelectric point (pI) of heavy chain deposition disease (HCDD) heavy chain variable regions (HCDD HC) compared with light chain (LC) and heavy chain (HC) germlines.
Kidney International  , DOI: ( /j.kint )
Copyright © 2016 International Society of Nephrology Terms and Conditions