Patients with Goodpasture's disease have two normal COL4A3 alleles encoding the NC1 domain of the type IV collagen alpha 3 chain

Ulf Persson, Jens Michael Hertz, Malin Carlsson, Thomas Hellmark, Inger juncker, Jörgen Wieslander, Mårten Segelmark

Publikation: Bidrag til tidsskriftTidsskriftartikelForskningpeer review

Resumé

Goodpasture's disease (GP) is a rare but severe disease characterized by anti-glomerular basement membrane antibodies, rapidly progressive glomerulonephritis and lung haemorrhage. The autoantibodies are restricted to a narrow epitope region on the NC1 domain of the alpha 3 chain of type IV collagen. GP is strongly associated with major histocompatibility complex (MHC) allele HLA DRB1-15. Recent research, however, has failed to identify a T-cell epitope with molecular characteristics that explain the relationship between the MHC class II molecule and the autoantibody generation. We hypothesized that an as yet unidentified sequence variant in exons 48-52 of the COL4A3 gene that encodes the NC1 domain of the type IV collagen alpha 3 chain could generate a new peptide sequence that, through interaction with specific MHC class II molecules, would increase the risk of developing GP.
OriginalsprogEngelsk
TidsskriftNephrology, Dialysis, Transplantation
Vol/bind19
Udgave nummer8
Sider (fra-til)2030-5
Antal sider6
ISSN0931-0509
DOI
StatusUdgivet - 2004

Fingeraftryk

Collagen Type IV
Alleles
HLA-DRB1 Chains
Glomerular Basement Membrane
T-Lymphocyte Epitopes
Rare Diseases
Glomerulonephritis
Lung
Peptides
Research

Citer dette

Persson, Ulf ; Hertz, Jens Michael ; Carlsson, Malin ; Hellmark, Thomas ; juncker, Inger ; Wieslander, Jörgen ; Segelmark, Mårten. / Patients with Goodpasture's disease have two normal COL4A3 alleles encoding the NC1 domain of the type IV collagen alpha 3 chain. I: Nephrology, Dialysis, Transplantation. 2004 ; Bind 19, Nr. 8. s. 2030-5.
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title = "Patients with Goodpasture's disease have two normal COL4A3 alleles encoding the NC1 domain of the type IV collagen alpha 3 chain",
abstract = "Goodpasture's disease (GP) is a rare but severe disease characterized by anti-glomerular basement membrane antibodies, rapidly progressive glomerulonephritis and lung haemorrhage. The autoantibodies are restricted to a narrow epitope region on the NC1 domain of the alpha 3 chain of type IV collagen. GP is strongly associated with major histocompatibility complex (MHC) allele HLA DRB1-15. Recent research, however, has failed to identify a T-cell epitope with molecular characteristics that explain the relationship between the MHC class II molecule and the autoantibody generation. We hypothesized that an as yet unidentified sequence variant in exons 48-52 of the COL4A3 gene that encodes the NC1 domain of the type IV collagen alpha 3 chain could generate a new peptide sequence that, through interaction with specific MHC class II molecules, would increase the risk of developing GP.",
keywords = "Adolescent, Adult, Aged, Alleles, Anti-Glomerular Basement Membrane Disease, Autoantigens, Collagen Type IV, Enzyme-Linked Immunosorbent Assay, Epitopes, European Continental Ancestry Group, Female, HLA-D Antigens, Humans, Male, Middle Aged, Protein Structure, Tertiary, Sequence Analysis, DNA, Seroepidemiologic Studies, Sweden",
author = "Ulf Persson and Hertz, {Jens Michael} and Malin Carlsson and Thomas Hellmark and Inger juncker and J{\"o}rgen Wieslander and M{\aa}rten Segelmark",
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Patients with Goodpasture's disease have two normal COL4A3 alleles encoding the NC1 domain of the type IV collagen alpha 3 chain. / Persson, Ulf; Hertz, Jens Michael; Carlsson, Malin; Hellmark, Thomas; juncker, Inger; Wieslander, Jörgen; Segelmark, Mårten.

I: Nephrology, Dialysis, Transplantation, Bind 19, Nr. 8, 2004, s. 2030-5.

Publikation: Bidrag til tidsskriftTidsskriftartikelForskningpeer review

TY - JOUR

T1 - Patients with Goodpasture's disease have two normal COL4A3 alleles encoding the NC1 domain of the type IV collagen alpha 3 chain

AU - Persson, Ulf

AU - Hertz, Jens Michael

AU - Carlsson, Malin

AU - Hellmark, Thomas

AU - juncker, Inger

AU - Wieslander, Jörgen

AU - Segelmark, Mårten

PY - 2004

Y1 - 2004

N2 - Goodpasture's disease (GP) is a rare but severe disease characterized by anti-glomerular basement membrane antibodies, rapidly progressive glomerulonephritis and lung haemorrhage. The autoantibodies are restricted to a narrow epitope region on the NC1 domain of the alpha 3 chain of type IV collagen. GP is strongly associated with major histocompatibility complex (MHC) allele HLA DRB1-15. Recent research, however, has failed to identify a T-cell epitope with molecular characteristics that explain the relationship between the MHC class II molecule and the autoantibody generation. We hypothesized that an as yet unidentified sequence variant in exons 48-52 of the COL4A3 gene that encodes the NC1 domain of the type IV collagen alpha 3 chain could generate a new peptide sequence that, through interaction with specific MHC class II molecules, would increase the risk of developing GP.

AB - Goodpasture's disease (GP) is a rare but severe disease characterized by anti-glomerular basement membrane antibodies, rapidly progressive glomerulonephritis and lung haemorrhage. The autoantibodies are restricted to a narrow epitope region on the NC1 domain of the alpha 3 chain of type IV collagen. GP is strongly associated with major histocompatibility complex (MHC) allele HLA DRB1-15. Recent research, however, has failed to identify a T-cell epitope with molecular characteristics that explain the relationship between the MHC class II molecule and the autoantibody generation. We hypothesized that an as yet unidentified sequence variant in exons 48-52 of the COL4A3 gene that encodes the NC1 domain of the type IV collagen alpha 3 chain could generate a new peptide sequence that, through interaction with specific MHC class II molecules, would increase the risk of developing GP.

KW - Adolescent

KW - Adult

KW - Aged

KW - Alleles

KW - Anti-Glomerular Basement Membrane Disease

KW - Autoantigens

KW - Collagen Type IV

KW - Enzyme-Linked Immunosorbent Assay

KW - Epitopes

KW - European Continental Ancestry Group

KW - Female

KW - HLA-D Antigens

KW - Humans

KW - Male

KW - Middle Aged

KW - Protein Structure, Tertiary

KW - Sequence Analysis, DNA

KW - Seroepidemiologic Studies

KW - Sweden

U2 - 10.1093/ndt/gfh355

DO - 10.1093/ndt/gfh355

M3 - Journal article

VL - 19

SP - 2030

EP - 2035

JO - Nephrology, Dialysis, Transplantation

JF - Nephrology, Dialysis, Transplantation

SN - 0931-0509

IS - 8

ER -