The Glomerular Filtration Barrier: Components and Crosstalk

Table 1

Classification of glomerular injury by pathomechanism.

(a) Inherited

Proteins involved in podocyte cytoskeletal integrity [4]
Podocin (AR), nephrin (AR), CD2-associated protein (AD), transient receptor potential cation-6 (AD), α-actinin-4 (AD), phospholipase-Cα1 (AR), tetraspanin CD-151 (AR), and Wilm’s tumor-1 (AD)
Basement membrane proteins [9, 11]
Alport’s syndrome-α-4 collagen-5 (XL~85%); Alport’s syndrome-α-4 collagen-3/4 (AR or AD~15%), thin Basement membrane disease (AD), and laminin-β2 (AR)
Proteins involved in endothelial/microvascular integrity [12, 15]
Complement regulatory proteins—complement factors H and I (AD), membrane cofactor protein (AD), and complement C3 (Gain-of-function)
Lysosomal storage diseases—metabolic injury [9]
Fabry’s disease (XL)

(b) Acquired

Immune	Nonimmune	Idiopathic
Immune complex mediated [13, 15]	Hemodynamic injury [10]	Unclear mechanisms
In situ formation or circulating complexes (postinfectious glomerulonephritis—streptococcal,	Hypertension	Minimal-change disease [22]
hepatits B/hepatitis-C-associated GN, systemic lupus erythematosus,	Adaptive hyperfiltration (nephron loss)	Membranous nephropathy [14]
and IgA nephropathy) idiopathic MP GN (types I and III)	Other	Primary FSGS [10]
Antibody-mediated [13]	Metabolic-hyperglycemia in diabetes
Anti-GBM antibody disease	Deposition disease- Amyloidosis
T-cell-mediated injury [13]	Toxic—Pamidronate, NSAIDs, D-pencillamine, and gold
Antineutrophil cytoplasm antibody vasculitis, most glomerulonephritides	Infectious—HIV [10]
Complement-mediated injury [12, 15]
MPGN type II, atypical HUS

Inheritance pattern—autosomal recessive (AR), dominant (AD), and X-linked (XL).