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2016 ; 11
(9
): 1664-1674
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The Players: Cells Involved in Glomerular Disease
#MMPMID27073196
Kitching AR
; Hutton HL
Clin J Am Soc Nephrol
2016[Sep]; 11
(9
): 1664-1674
PMID27073196
show ga
Glomerular diseases are common and important. They can arise from systemic
inflammatory or metabolic diseases that affect the kidney. Alternately, they are
caused primarily by local glomerular abnormalities, including genetic diseases.
Both intrinsic glomerular cells and leukocytes are critical to the healthy
glomerulus and to glomerular dysregulation in disease. Mesangial cells,
endothelial cells, podocytes, and parietal epithelial cells within the glomerulus
all play unique and specialized roles. Although a specific disease often
primarily affects a particular cell type, the close proximity, and interdependent
functions and interactions between cells mean that even diseases affecting one
cell type usually indirectly influence others. In addition to those cells
intrinsic to the glomerulus, leukocytes patrol the glomerulus in health and
mediate injury in disease. Distinct leukocyte types and subsets are present, with
some being involved in different ways in an individual glomerular disease. Cells
of the innate and adaptive immune systems are important, directing systemic
immune and inflammatory responses, locally mediating injury, and potentially
dampening inflammation and facilitating repair. The advent of new genetic and
molecular techniques, and new disease models means that we better understand both
the basic biology of the glomerulus and the pathogenesis of glomerular disease.
This understanding should lead to better diagnostic techniques, biomarkers, and
predictors of prognosis, disease severity, and relapse. With this knowledge comes
the promise of better therapies in the future, directed toward halting pathways
of injury and fibrosis, or interrupting the underlying pathophysiology of the
individual diseases that lead to significant and progressive glomerular disease.