Alport's syndrome, Goodpasture's syndrome, and type IV collagen

BG Hudson, K Tryggvason… - … England Journal of …, 2003 - Mass Medical Soc
Defects in type IV collagen, a collagenous protein involved in the formation of basement
membranes, have been implicated in hereditary Alport's syndrome and acquired …

Mammalian collagen IV

J Khoshnoodi, V Pedchenko… - … research and technique, 2008 - Wiley Online Library
Four decades have passed since the first discovery of collagen IV by Kefalides in 1966.
Since then collagen IV has been investigated extensively by a large number of research …

[图书][B] Murray and Nadel's textbook of respiratory medicine E-book: 2-volume set

RJ Mason, VC Broaddus, TR Martin, TE King… - 2010 - books.google.com
Murray and Nadel's Textbook of Respiratory Medicine has long been the definitive and
comprehensive pulmonary disease reference. Robert J. Mason, MD now presents the fifth …

Alport syndrome

CE Kashtan, AF Michael - Kidney international, 1996 - Elsevier
Hereditary familial or congenital nephritis is a definite entity.... Deafness is a marked feature
in nearly all these cases... The male members of a family tend to develop nephritis and …

Anti-neutrophil cytoplasmic antibodies: current diagnostic and pathophysiological potential

CGM Kallenberg, E Brouwer, JJ Weening… - Kidney international, 1994 - Elsevier
Rapidly progressive glomerulonephritis (RPGN) is a clinical syndrome characterized by
rapid deterioration of renal function occurring within days or weeks together with signs of …

Molecular heterogeneity of basal laminae: isoforms of laminin and collagen IV at the neuromuscular junction and elsewhere.

JR Sanes, E Engvall, R Butkowski… - The Journal of cell …, 1990 - rupress.org
Laminin and collagen IV are components of most basal laminae (BLs). Recently, both have
been shown to be products of multigene families. The A, B1, and B2 subunits of the laminin …

Neph1 and nephrin interaction in the slit diaphragm is an important determinant of glomerular permeability

G Liu, B Kaw, J Kurfis, S Rahmanuddin… - The Journal of …, 2003 - Am Soc Clin Investig
Neph1-deficient mice develop nephrotic syndrome at birth, indicating the importance of this
protein in the development of a normal glomerular filtration barrier. While the precise …

The molecular basis of Goodpasture and Alport syndromes: beacons for the discovery of the collagen IV family

BG Hudson - Journal of the American Society of Nephrology, 2004 - journals.lww.com
The glomerular basement membrane (GBM), a principal component of the filtration barrier, is
abnormal in several renal diseases. Notable examples include Alport syndrome …

Structure of the human type IV collagen gene COL4A3 and mutations in autosomal Alport syndrome

L Heidet, C Arrondel, L Forestier… - Journal of the …, 2001 - journals.lww.com
Mutations in either the COL4A3 or the COL4A4 genes, encoding the α3 and α4 chains of
type IV collagen, are responsible for the autosomal-recessive form of Alport syndrome, a …

[HTML][HTML] Brenner and Rector's the kidney

PH Nachman, JC Jennette, RJ Falk - 2012 - doctorlib.info
The underlying cause of most glomerular diseases remains an enigma. Infectious agents,
autoimmunity, drugs, inherited disorders, and environmental agents have been implicated …