title: The mesangial matrix in the normal and sclerotic glomerulus.
abstract: Mesangial sclerosis is a final common pathway to glomerular destruction in a variety of glomerular diseases. The expression of several classes of extracellular matrix (ECM) molecules has been defined in the normal and diseased mesangial matrix (MM). However, the manner in which these ECM components determine the three dimensional structure and function of the MM remains to be defined. Structural studies of the MM suggest that its constituent molecules are regionally organized into subcompartments with different three dimensional structures. The diversity of matrix molecules expressed within the MM as well as the organization of these components in nonrenal ECM's, such as the cornea, provides further support for this organizational model. The study of the cornea has also revealed that novel short chain collagenous proteins partially determine the three dimensional structure of the matrix. Recently, a novel collagen, type VIII collagen, has been described in mesangial cells and in the intact glomerulus. It is hypothesized that type VIII collagen is expressed both as a polymer and as a monomer within the glomerulus, and depending on its conformation, may serve unique functions. In the chronically diseased MM, normal MM components are overexpressed and fibrillar collagens are expressed de novo in a delayed fashion. Enhanced proteoglycan expression, observed early in disease, may determine increased volume of the mesangium. This, in turn, may stimulate the production of fibrillar collagens by mesangial cells resulting in a fibrillar noncompliant mesangial matrix.
authors.affiliation: Division of Nephrology, Children's Hospital, Harvard Medical School, Boston, Massachusetts.
authors.forename: N D
authors.initials: ND
authors.lastname: Rosenblum
mesh_terms: D000818:Animals; D002908:Chronic Disease; D003094:Collagen / Q000737:chemistry / Q000378:metabolism; D003315:Cornea / Q000378:metabolism; D005109:Extracellular Matrix / Q000378:metabolism*; D005920:Glomerular Mesangium / Q000378:metabolism*; D005923:Glomerulosclerosis, Focal Segmental / Q000378:metabolism*; D006801:Humans; D007674:Kidney Diseases / Q000378:metabolism; D007678:Kidney Glomerulus / Q000378:metabolism*; D011509:Proteoglycans / Q000378:metabolism; D012016:Reference Values
chemical_list: D011509:Proteoglycans; D003094:Collagen