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Review Article
Mechanisms of Disease
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Volume 348:2543-2556 June 19, 2003 Number 25
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Alport's Syndrome, Goodpasture's Syndrome, and Type IV Collagen
Billy G. Hudson, Ph.D., Karl Tryggvason, M.D., Ph.D., Munirathinam Sundaramoorthy, Ph.D., and Eric G. Neilson, M.D.

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Basement membranes form a complex surface on which epithelial cells reside. These membranes provide morphogenic cues that determine the fate of cells, the polarization of subcellular constituents, and the location of cell receptors and transporters.1,2,3 Basement membranes are assembled through an interweaving of type IV collagen (collagen IV) with laminins, nidogen, and sulfated proteoglycans.4,5 Collagen IV belongs to a family of collagenous proteins that has at least 25 distinct members. The COL4A1, COL4A2, COL4A3, COL4A4, COL4A5, and COL4A6 genes6,7,8,9,10,11,12,13 encoding the six chains of collagen IV — {alpha}1(IV) through {alpha}6(IV) — are selectively expressed in different membranes at various stages . . . [Full Text of this Article]

Structure and Distribution of Type IV Collagen

Alport's Syndrome

Genetics

Pathogenesis

Clinical Presentation

Findings on Kidney Biopsy

Treatment

Goodpasture's Syndrome

Genetics

Pathogenesis

Clinical Presentation

Anti–Glomerular Basement Membrane Antibodies

Findings on Kidney Biopsy

Treatment

Summary


Source Information

From the Departments of Medicine (B.H., M.S., E.G.N.), Biochemistry (B.H., M.S.), and Cell and Developmental Biology (E.G.N.), Vanderbilt University School of Medicine, Nashville; and the Department of Medical Biochemistry and Biophysics, Karolinska Institute, Stockholm, Sweden (K.T.).

Address reprint requests to Dr. Neilson at the Department of Medicine, D-3100 MCN, Vanderbilt University Medical Center, Nashville, TN 37232-2358, or at eric.neilson@vanderbilt.edu.


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