Gene/Protein
Disease
Symptom
Drug
Enzyme
Compound
Pivot Concepts:
Gene/Protein
Disease
Symptom
Drug
Enzyme
Compound
Target Concepts:
Gene/Protein
Disease
Symptom
Drug
Enzyme
Compound
Query: UMLS:C0002986 (
Fabry
)
5,646
document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)
A renal disorder is one of the important manifestations of
Fabry disease
, but the details of the pathogenesis have not been clarified yet. We examined the possibility that the
vitronectin
receptor (VNR, integrin alpha V beta 3), one of the integrins, is involved in the progression of the renal injury in
Fabry disease
. We measured the urinary excretion of beta 3 originating from VNR in
Fabry
patients by immunoblotting analysis and enzyme-linked immunosorbent assay (ELISA). Immunofluorescent microscopic analyses for VNR and globotriaosylceramide were performed on urinary sediments from
Fabry
patients. Furthermore, beta 3 and
vitronectin
in kidney tissues were analyzed immunohistochemically. Immunoblotting analysis and ELISA showed that the urinary excretion of beta 3 originating from VNR was significantly increased in the
Fabry
group compared with both the pathological and healthy control groups. Immunofluorescent microscopy revealed the expression of VNR and accumulation of globotriaosylceramide in urinary sediments from the
Fabry
patients. Increased expression of beta 3 was observed in glomerular epithelial cells, and in Bowman's capsular epithelial layer and tubular cells, and the amount of
vitronectin
was moderately increased in the kidney tissues from the
Fabry
patients. The urinary excretion of VNR was increased, and the expression of VNR was observed in
Fabry
kidney tissues. The expression of VNR may be involved in the progression of the renal injury in this disease.
...
PMID:Urinary excretion of the vitronectin receptor (integrin alpha V beta 3) in patients with Fabry disease. 1006 18
