RGD Reference Report - Gelatinase B-deficient mice are resistant to experimental bullous pemphigoid. - Rat Genome Database

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Gelatinase B-deficient mice are resistant to experimental bullous pemphigoid.

Authors: Liu, Z  Shipley, JM  Vu, TH  Zhou, X  Diaz, LA  Werb, Z  Senior, RM 
Citation: Liu Z, etal., J Exp Med. 1998 Aug 3;188(3):475-82.
RGD ID: 8547843
Pubmed: PMID:9687525   (View Abstract at PubMed)
PMCID: PMC2212482   (View Article at PubMed Central)

Bullous pemphigoid (BP) is an autoimmune subepidermal blistering disease characterized by deposition of autoantibodies at the basement membrane zone. In an experimental BP model in mice, the subepidermal blistering is mediated by antibodies directed against the hemidesmosomal protein BP180 (collagen XVII, BPAG2), and depends on complement activation and neutrophil infiltration. Gelatinase B is present in BP blister fluid and can cleave BP180. In this study we investigated the role of gelatinase B in the immunopathogenesis of experimental BP using mice containing targeted disruption of the gelatinase B (MMP-9, 92 kD gelatinase) gene. Gelatinase B-deficient mice were resistant to the blistering effect of intracutaneous anti-mBP180 antibodies, although these mice showed deposition of autoantibodies at the basement membrane zone and neutrophil recruitment to the skin comparable to that observed in the control mice. Interleukin 8 given intradermally concomitantly with pathogenic anti-mBP180 elicited a significant neutrophil recruitment into the skin in gelatinase B-deficient mice, but blistering did not occur. However, gelatinase B-deficient mice reconstituted with neutrophils from normal mice developed blistering in response to anti-mBP180 antibodies. These results implicate neutrophil-derived gelatinase B in the pathogenesis of experimental BP and might lead to novel therapeutic strategies for BP.



RGD Manual Disease Annotations    Click to see Annotation Detail View

  
Object SymbolSpeciesTermQualifierEvidenceWithNotesSourceOriginal Reference(s)
MMP9Humanbullous pemphigoid  ISOMmp9 (Mus musculus) RGD 
Mmp9Ratbullous pemphigoid  ISOMmp9 (Mus musculus) RGD 
Mmp9Mousebullous pemphigoid  IMP  RGD 

Objects Annotated

Genes (Rattus norvegicus)
Mmp9  (matrix metallopeptidase 9)

Genes (Mus musculus)
Mmp9  (matrix metallopeptidase 9)

Genes (Homo sapiens)
MMP9  (matrix metallopeptidase 9)


Additional Information