Published in Am J Pathol on March 01, 2003
Understanding hereditary diseases using the dog and human as companion model systems. Mamm Genome (2007) 1.09
Identification of noncollagenous sites encoding specific interactions and quaternary assembly of alpha 3 alpha 4 alpha 5(IV) collagen: implications for Alport gene therapy. J Biol Chem (2008) 0.93
Novel X-linked glomerulopathy is associated with a COL4A5 missense mutation in a non-collagenous interruption. Kidney Int (2010) 0.89
Familial hematurias: what we know and what we don't. Pediatr Nephrol (2005) 0.83
Dysregulation of renal MMP-3 and MMP-7 in canine X-linked Alport syndrome. Pediatr Nephrol (2005) 0.82
Pathological features of proteinuric nephropathy resembling Alport syndrome in a young Pyrenean Mountain dog. J Vet Med Sci (2015) 0.75
Identification of prokaryotic and eukaryotic signal peptides and prediction of their cleavage sites. Protein Eng (1997) 38.38
Collagens: molecular biology, diseases, and potentials for therapy. Annu Rev Biochem (1995) 5.52
Structure and biological activity of basement membrane proteins. Eur J Biochem (1989) 3.44
Polymerase chain reaction for detection of adenoviruses in stool samples. J Clin Microbiol (1990) 3.00
Type IV collagen: structure, gene organization, and role in human diseases. Molecular basis of Goodpasture and Alport syndromes and diffuse leiomyomatosis. J Biol Chem (1993) 2.89
X-linked Alport syndrome: natural history in 195 families and genotype- phenotype correlations in males. J Am Soc Nephrol (2000) 2.71
Identification of mutations in the alpha 3(IV) and alpha 4(IV) collagen genes in autosomal recessive Alport syndrome. Nat Genet (1994) 2.12
Macromolecular structure of basement membrane collagens. FEBS Lett (1981) 1.96
Establishment by the rat lymph node method of epitope-defined monoclonal antibodies recognizing the six different alpha chains of human type IV collagen. Histochem Cell Biol (1995) 1.88
Differential expression of two basement membrane collagen genes, COL4A6 and COL4A5, demonstrated by immunofluorescence staining using peptide-specific monoclonal antibodies. J Cell Biol (1995) 1.67
Diagnosis of fastidious enteric adenoviruses 40 and 41 in stool specimens. J Clin Microbiol (1984) 1.57
Glomerular basement membrane. Identification of a novel disulfide-cross-linked network of alpha3, alpha4, and alpha5 chains of type IV collagen and its implications for the pathogenesis of Alport syndrome. J Biol Chem (1998) 1.51
Structure of the human type IV collagen gene COL4A3 and mutations in autosomal Alport syndrome. J Am Soc Nephrol (2001) 1.46
The clinical spectrum of hereditary nephritis. Kidney Int (1985) 1.33
The NC1 domain of collagen IV encodes a novel network composed of the alpha 1, alpha 2, alpha 5, and alpha 6 chains in smooth muscle basement membranes. J Biol Chem (2001) 1.32
Quaternary organization of the goodpasture autoantigen, the alpha 3(IV) collagen chain. Sequestration of two cryptic autoepitopes by intrapromoter interactions with the alpha4 and alpha5 NC1 domains. J Biol Chem (2002) 1.32
Goodpasture antigen: expression of the full-length alpha3(IV) chain of collagen IV and localization of epitopes exclusively to the noncollagenous domain. Kidney Int (1999) 1.31
The arrangement of intra- and intermolecular disulfide bonds in the carboxyterminal, non-collagenous aggregation and cross-linking domain of basement-membrane type IV collagen. Eur J Biochem (1988) 1.27
Alport's syndrome: experience at Hôpital Necker. Kidney Int Suppl (1982) 1.25
Alport syndrome. Kidney Int (1996) 1.20
Spectrum of mutations in the COL4A5 collagen gene in X-linked Alport syndrome. Am J Hum Genet (1996) 1.20
Role of distinct type IV collagen networks in glomerular development and function. Kidney Int (1998) 1.15
Comparative distribution of the alpha 1(IV), alpha 5(IV), and alpha 6(IV) collagen chains in normal human adult and fetal tissues and in kidneys from X-linked Alport syndrome patients. J Clin Invest (1995) 1.14
Type IV collagen alpha 5 chain. Normal distribution and abnormalities in X-linked Alport syndrome revealed by monoclonal antibody. Am J Pathol (1994) 1.13
Coordinate gene expression of the alpha3, alpha4, and alpha5 chains of collagen type IV. Evidence from a canine model of X-linked nephritis with a COL4A5 gene mutation. J Biol Chem (1996) 1.11
Immunohistochemical study of alpha 1-5 chains of type IV collagen in hereditary nephritis. Kidney Int (1994) 1.11
Complete primary structure of the triple-helical region and the carboxyl-terminal domain of a new type IV collagen chain, alpha 5(IV). J Biol Chem (1990) 1.10
Canine X chromosome-linked hereditary nephritis: a genetic model for human X-linked hereditary nephritis resulting from a single base mutation in the gene encoding the alpha 5 chain of collagen type IV. Proc Natl Acad Sci U S A (1994) 1.06
Adenovirus-mediated transfer of type IV collagen alpha5 chain cDNA into swine kidney in vivo: deposition of the protein into the glomerular basement membrane. Gene Ther (2001) 1.03
Evidence for separate networks of classical and novel basement membrane collagen. Characterization of alpha 3(IV)-alport antigen heterodimer. J Biol Chem (1992) 1.02
Adenovirus-mediated gene transfer into kidney glomeruli using an ex vivo and in vivo kidney perfusion system - first steps towards gene therapy of Alport syndrome. Gene Ther (1996) 0.97
Samoyed hereditary glomerulopathy (SHG). Evolution of splitting of glomerular capillary basement membranes. Am J Pathol (1986) 0.94
Structure of the human type IV collagen COL4A5 gene. J Biol Chem (1994) 0.90
The inner ear of dogs with X-linked nephritis provides clues to the pathogenesis of hearing loss in X-linked Alport syndrome. Am J Pathol (2001) 0.89
Secretion of non-helical collagenous polypeptides of alpha1(IV) and alpha2(IV) chains upon depletion of ascorbate by cultured human cells. J Biochem (2001) 0.87
Biosynthesis of alpha2(IV) and alpha1(IV) chains of collagen IV and interactions with matrix metalloproteinase-9. J Cell Physiol (1999) 0.86
Differential splicing of COL4A5 mRNA in kidney and white blood cells: a complex mutation in the COL4A5 gene of an Alport patient deletes the NC1 domain. Kidney Int (1993) 0.86
Absence of the alpha6(IV) chain of collagen type IV in Alport syndrome is related to a failure at the protein assembly level and does not result in diffuse leiomyomatosis. Am J Pathol (1999) 0.82
Samoyed hereditary glomerulopathy: serial, clinical and laboratory (urine, serum biochemistry and hematology) studies. Can J Vet Res (1987) 0.81
Can Alport syndrome be treated by gene therapy? Kidney Int (1997) 0.81
Formation of recombinant triple-helical [alpha 1(IV)]2 alpha 2(IV) collagen molecules in CHO cells. Biochem Biophys Res Commun (1997) 0.81
Two novel alternatively spliced 9-bp exons in the COL4A5 gene. Pediatr Nephrol (2001) 0.80
Bladder smooth muscle cells in culture: I. Identification and characterization. J Urol (1993) 0.80
Gene therapy for renal diseases. Kidney Int Suppl (1997) 0.80
Production of anti-NC1 antibody by affected male dogs with X-linked hereditary nephritis: a probe for assessing the NC1 domain of collagen type IV in dogs and humans with hereditary nephritis. Virchows Arch A Pathol Anat Histopathol (1992) 0.76
Collagen IV is essential for basement membrane stability but dispensable for initiation of its assembly during early development. Development (2004) 3.12
Neuroblastoma cells isolated from bone marrow metastases contain a naturally enriched tumor-initiating cell. Cancer Res (2007) 2.56
Distinct target-derived signals organize formation, maturation, and maintenance of motor nerve terminals. Cell (2007) 2.00
Irradiation prolongs survival of Alport mice. J Am Soc Nephrol (2008) 1.84
Human podocytes adhere to the KRGDS motif of the alpha3alpha4alpha5 collagen IV network. J Am Soc Nephrol (2008) 1.81
Large-scale identification of genes implicated in kidney glomerulus development and function. EMBO J (2006) 1.69
Generation of a transplantable erythropoietin-producer derived from human mesenchymal stem cells. Transplantation (2008) 1.58
Association between DNA variant sites in the apolipoprotein A5 gene and coronary heart disease in Chinese. Metabolism (2005) 1.58
Podocytes contribute to the formation of glomerular crescents. J Am Soc Nephrol (2008) 1.56
Type IV collagen induces podocytic features in bone marrow stromal stem cells in vitro. J Am Soc Nephrol (2005) 1.53
Acute and chronic microvascular alterations in a mouse model of ischemic acute kidney injury. Am J Physiol Renal Physiol (2007) 1.46
Effectiveness of short-term and long-term zidovudine prophylaxis on detection of HIV-1 subtype E in human placenta and vertical transmission. J Acquir Immune Defic Syndr (2005) 1.41
Goodpasture autoantibodies unmask cryptic epitopes by selectively dissociating autoantigen complexes lacking structural reinforcement: novel mechanisms for immune privilege and autoimmune pathogenesis. J Biol Chem (2005) 1.35
Mouse model of X-linked Alport syndrome. J Am Soc Nephrol (2004) 1.35
Loss of alpha3/alpha4(IV) collagen from the glomerular basement membrane induces a strain-dependent isoform switch to alpha5alpha6(IV) collagen associated with longer renal survival in Col4a3-/- Alport mice. J Am Soc Nephrol (2006) 1.35
Dominant mutations of Col4a1 result in basement membrane defects which lead to anterior segment dysgenesis and glomerulopathy. Hum Mol Genet (2005) 1.33
Identification of a novel gene NCRMS on chromosome 12q21 with differential expression between rhabdomyosarcoma subtypes. Oncogene (2002) 1.32
Quaternary organization of the goodpasture autoantigen, the alpha 3(IV) collagen chain. Sequestration of two cryptic autoepitopes by intrapromoter interactions with the alpha4 and alpha5 NC1 domains. J Biol Chem (2002) 1.32
Transcriptome-based systematic identification of extracellular matrix proteins. Proc Natl Acad Sci U S A (2008) 1.31
Compositional differences between infant and adult human corneal basement membranes. Invest Ophthalmol Vis Sci (2007) 1.25
Increased activity and expression of histone deacetylase 1 in relation to tumor necrosis factor-alpha in synovial tissue of rheumatoid arthritis. Arthritis Res Ther (2010) 1.24
The murine Ten-m/Odz genes show distinct but overlapping expression patterns during development and in adult brain. Gene Expr Patterns (2003) 1.21
High nephritogenicity of monoclonal antibodies belonging to IgG2a and IgG2b subclasses in rat anti-GBM nephritis. Kidney Int (2004) 1.20
Frequent deletion and down-regulation of ING4, a candidate tumor suppressor gene at 12p13, in head and neck squamous cell carcinomas. Gene (2005) 1.18
A role for collagen IV cross-links in conferring immune privilege to the Goodpasture autoantigen: structural basis for the crypticity of B cell epitopes. J Biol Chem (2008) 1.17
Serum adipocyte fatty acid-binding protein is independently associated with coronary atherosclerotic burden measured by intravascular ultrasound. Atherosclerosis (2010) 1.16
Low-dose airborne endotoxin exposure enhances bronchial responsiveness to inhaled allergen in atopic asthmatics. J Allergy Clin Immunol (2003) 1.16
DDR1-deficient mice show localized subepithelial GBM thickening with focal loss of slit diaphragms and proteinuria. Kidney Int (2004) 1.15
A human-mouse chimera of the alpha3alpha4alpha5(IV) collagen protomer rescues the renal phenotype in Col4a3-/- Alport mice. Am J Pathol (2003) 1.14
Stromal cell-derived factor-1 from biliary epithelial cells recruits CXCR4-positive cells: implications for inflammatory liver diseases. Lab Invest (2003) 1.13
Secretion and assembly of type IV and VI collagens depend on glycosylation of hydroxylysines. J Biol Chem (2007) 1.13
Cyclosporine a slows the progressive renal disease of alport syndrome (X-linked hereditary nephritis): results from a canine model. J Am Soc Nephrol (2003) 1.12
ADAMTS-9 is synergistically induced by interleukin-1beta and tumor necrosis factor alpha in OUMS-27 chondrosarcoma cells and in human chondrocytes. Arthritis Rheum (2005) 1.10
Synovial pathology in camptodactyly-arthropathy-coxa vara-pericarditis syndrome. Pediatr Dev Pathol (2005) 1.09
In vivo antitumor and antimetastatic activity of sunitinib in preclinical neuroblastoma mouse model. Neoplasia (2009) 1.09
Bral1: its role in diffusion barrier formation and conduction velocity in the CNS. J Neurosci (2010) 1.08
The intermediate filament nestin is highly expressed in normal human podocytes and podocytes in glomerular disease. Pediatr Dev Pathol (2007) 1.08
Versican is induced in infiltrating monocytes in myocardial infarction. Mol Cell Biochem (2005) 1.07
Loss of expression of type IV collagen alpha5 and alpha6 chains in colorectal cancer associated with the hypermethylation of their promoter region. Am J Pathol (2006) 1.05
Cardio-ankle vascular index is independently associated with the severity of coronary atherosclerosis and left ventricular function in patients with ischemic heart disease. J Atheroscler Thromb (2010) 1.05
Discovery of novel orally bioavailable oxaborole 6-carboxamides that demonstrate cure in a murine model of late-stage central nervous system african trypanosomiasis. Antimicrob Agents Chemother (2010) 1.04
Bral1, a brain-specific link protein, colocalizing with the versican V2 isoform at the nodes of Ranvier in developing and adult mouse central nervous systems. Mol Cell Neurosci (2002) 1.04
Thrombospondin-1 is induced in rat myocardial infarction and its induction is accelerated by ischemia/reperfusion. Exp Biol Med (Maywood) (2005) 1.03
Vascular endothelial growth factor principally acts as the main angiogenic factor in the early stage of human osteoblastogenesis. J Biochem (2003) 1.03
All four members of the Ten-m/Odz family of transmembrane proteins form dimers. J Biol Chem (2002) 1.03
Basement membrane type IV collagen molecules in the choroid plexus, pia mater and capillaries in the mouse brain. Arch Histol Cytol (2002) 1.02
Murine membranous nephropathy: immunization with α3(IV) collagen fragment induces subepithelial immune complexes and FcγR-independent nephrotic syndrome. J Immunol (2012) 0.99
Design, synthesis, and structure-activity relationship of Trypanosoma brucei leucyl-tRNA synthetase inhibitors as antitrypanosomal agents. J Med Chem (2011) 0.99
ADAMTS1 is a unique hypoxic early response gene expressed by endothelial cells. J Biol Chem (2009) 0.99
Olmesartan reduces arterial stiffness and serum adipocyte fatty acid-binding protein in hypertensive patients. Heart Vessels (2010) 0.98
2,4-Diaminopyrimidines as potent inhibitors of Trypanosoma brucei and identification of molecular targets by a chemical proteomics approach. PLoS Negl Trop Dis (2011) 0.98
MMP-9 gene deletion mitigates microvascular loss in a model of ischemic acute kidney injury. Am J Physiol Renal Physiol (2011) 0.97
Molecular cloning of a novel transmembrane protein MOLT expressed by mature oligodendrocytes. J Biochem (2003) 0.97
Recurrent RECQL4 imbalance and increased gene expression levels are associated with structural chromosomal instability in sporadic osteosarcoma. Neoplasia (2009) 0.97
Transgene of MIF induces podocyte injury and progressive mesangial sclerosis in the mouse kidney. Kidney Int (2004) 0.97
Pediatric undifferentiated sarcoma of the soft tissues: a clinicopathologic study. Pediatr Dev Pathol (2006) 0.97
A new molecular variant of desmoplastic small round cell tumor: significance of WT1 immunostaining in this entity. Hum Pathol (2008) 0.96
Epitope-defined monoclonal antibodies against multiplexin collagens demonstrate that type XV and XVIII collagens are expressed in specialized basement membranes. Cell Struct Funct (2002) 0.96
Expression of insulin-like growth factor pathway proteins in rhabdomyosarcoma: IGF-2 expression is associated with translocation-negative tumors. Pediatr Dev Pathol (2008) 0.96
Brevican distinctively assembles extracellular components at the large diameter nodes of Ranvier in the CNS. J Neurochem (2009) 0.95
HPV genotyping in cervical cancer in Northern Thailand: adapting the linear array HPV assay for use on paraffin-embedded tissue. Gynecol Oncol (2008) 0.95
Suppression of chondrosarcoma cells by 15-deoxy-Delta 12,14-prostaglandin J2 is associated with altered expression of Bax/Bcl-xL and p21. Biochem Biophys Res Commun (2005) 0.95
Vitamin A deficiency alters the structure and collagen IV composition of rat renal basement membranes. J Nutr (2005) 0.94
Loss of alpha3(IV) collagen expression associated with corneal keratocyte activation. Invest Ophthalmol Vis Sci (2007) 0.94
The effect of dynamic femoroacetabular impingement on pubic symphysis motion: a cadaveric study. Am J Sports Med (2012) 0.93
Identification of noncollagenous sites encoding specific interactions and quaternary assembly of alpha 3 alpha 4 alpha 5(IV) collagen: implications for Alport gene therapy. J Biol Chem (2008) 0.93
Application of cryobiopsy to morphological and immunohistochemical analyses of xenografted human lung cancer tissues and functional blood vessels. Cancer (2008) 0.93
Rho-mediated regulation of TGF-beta1- and FGF-2-induced activation of corneal stromal keratocytes. Invest Ophthalmol Vis Sci (2009) 0.92
The extracellular matrix of hydra is a porous sheet and contains type IV collagen. Zoology (Jena) (2008) 0.92
HER2 amplification and overexpression is not present in pediatric osteosarcoma: a tissue microarray study. Pediatr Dev Pathol (2005) 0.91
Tumor-specific expression of the RGD-alpha3(IV)NC1 domain suppresses endothelial tube formation and tumor growth in mice. FASEB J (2006) 0.90
Comparative analysis of basal lamina type IV collagen α chains, matrix metalloproteinases-2 and -9 expressions in oral dysplasia and invasive carcinoma. Acta Histochem (2012) 0.90
Hyaluronan receptors involved in cytokine induction in monocytes. Glycobiology (2008) 0.90
Spectrum of bone tumors in Chiang Mai University Hospital, Thailand according to WHO classification 2002: A study of 1,001 cases. J Med Assoc Thai (2006) 0.89
Serum-starved adipose-derived stromal cells ameliorate crescentic GN by promoting immunoregulatory macrophages. J Am Soc Nephrol (2013) 0.89