title: Increased urinary CD80 excretion and podocyturia in Fabry disease.
abstract: Certain glomerulopathies are associated with increased levels of CD80 (B7-1). We measured the urinary excretion of CD80, podocyturia and proteinuria in controls and in subjects with Fabry disease either untreated or on enzyme replacement therapy (ERT).
  
  
  
  Cross-sectional study including 65 individuals: controls (n = 20) and Fabry patients (n = 45, 23 of them not on ERT and 22 on ERT). Variables included age, gender, urinary protein/creatinine ratio (UPCR), estimated glomerular filtration rate (eGFR), urinary uCD80/creatinine ratio (uCD80) and podocyturia. CD80 mRNA expression in response to lyso-Gb3, a bioactive glycolipid accumulated in Fabry disease, was studied in cultured human podocytes.
  
  
  
  Controls and Fabry patients did not differ in age, eGFR and gender. However, UPCR, uCD80 and podocyturia were significantly higher in Fabry patients than in controls. As expected, Fabry patients not on ERT were younger and a higher percentage were females. Non-ERT Fabry patients had less advanced kidney disease than ERT Fabry patients: UPCR was lower and eGFR higher, but uCD80 and podocyturia did not differ between non-ERT or ERT Fabry patients. There was a significant correlation between uCD80 and UPCR in the whole population (r 0.44, p 0.0005) and in Fabry patients (r 0.42, p 0.0046). Lyso-Gb3 at concentrations found in the circulation of Fabry patients increased uCD80 expression in cultured podocytes.
  
  
  
  Fabry disease is characterized by early occurrence of increased uCD80 excretion that appears to be a consequence of glycolipid accumulation. The potential for uCD80 excretion to reflect early, subclinical renal Fabry involvement should be further studied.
authors.affiliation: Nephrology Service, Hospital Británico de Buenos Aires, Perdriel 74, 1280, Buenos Aires, Argentina. htrimarchi@hotmail.com.
authors.forename: H
authors.initials: H
authors.lastname: Trimarchi
authors.affiliation: Central Laboratory, Hospital Británico de Buenos Aires, Buenos Aires, Argentina.
authors.forename: R
authors.initials: R
authors.lastname: Canzonieri
authors.affiliation: Central Laboratory, Hospital Británico de Buenos Aires, Buenos Aires, Argentina.
authors.forename: A
authors.initials: A
authors.lastname: Schiel
authors.affiliation: IFIBIO Houssay, CONICET, Physiopathology, Pharmacy and Biochemistry Faculty, Universidad de Buenos Aires, Buenos Aires, Argentina.
authors.forename: C
authors.initials: C
authors.lastname: Costales-Collaguazo
authors.affiliation: Neurology Department, Laboratorio Neuroquímica Dr. Néstor Chamoles, Buenos Aires, Argentina.
authors.forename: J
authors.initials: J
authors.lastname: Politei
authors.affiliation: Central Laboratory, Hospital Británico de Buenos Aires, Buenos Aires, Argentina.
authors.forename: A
authors.initials: A
authors.lastname: Stern
authors.affiliation: Nephrology Service, Hospital Británico de Buenos Aires, Perdriel 74, 1280, Buenos Aires, Argentina.
authors.forename: M
authors.initials: M
authors.lastname: Paulero
authors.affiliation: Nephrology Service, Hospital Británico de Buenos Aires, Perdriel 74, 1280, Buenos Aires, Argentina.
authors.forename: T
authors.initials: T
authors.lastname: Rengel
authors.affiliation: Nephrology Service, Hospital Británico de Buenos Aires, Perdriel 74, 1280, Buenos Aires, Argentina.
authors.forename: J
authors.initials: J
authors.lastname: Andrews
authors.affiliation: Nephrology Service, Hospital Británico de Buenos Aires, Perdriel 74, 1280, Buenos Aires, Argentina.
authors.forename: M
authors.initials: M
authors.lastname: Forrester
authors.affiliation: Nephrology Service, Hospital Británico de Buenos Aires, Perdriel 74, 1280, Buenos Aires, Argentina.
authors.forename: M
authors.initials: M
authors.lastname: Lombi
authors.affiliation: Nephrology Service, Hospital Británico de Buenos Aires, Perdriel 74, 1280, Buenos Aires, Argentina.
authors.forename: V
authors.initials: V
authors.lastname: Pomeranz
authors.affiliation: Nephrology Service, Hospital Británico de Buenos Aires, Perdriel 74, 1280, Buenos Aires, Argentina.
authors.forename: R
authors.initials: R
authors.lastname: Iriarte
authors.affiliation: Central Laboratory, Hospital Británico de Buenos Aires, Buenos Aires, Argentina.
authors.forename: A
authors.initials: A
authors.lastname: Muryan
authors.affiliation: IFIBIO Houssay, CONICET, Physiopathology, Pharmacy and Biochemistry Faculty, Universidad de Buenos Aires, Buenos Aires, Argentina.
authors.forename: E
authors.initials: E
authors.lastname: Zotta
authors.affiliation: IIS-Fundacion Jimenez Diaz, School of Medicine, UAM, Avda Reyes Catolicos 2, 28040, Madrid, Spain. mdsanchez@fjd.es.|REDINREN, Madrid, Spain. mdsanchez@fjd.es.
authors.forename: M D
authors.initials: MD
authors.lastname: Sanchez-Niño
authors.affiliation: IIS-Fundacion Jimenez Diaz, School of Medicine, UAM, Avda Reyes Catolicos 2, 28040, Madrid, Spain.|REDINREN, Madrid, Spain.
authors.forename: A
authors.initials: A
authors.lastname: Ortiz
mesh_terms: D000293:Adolescent; D000328:Adult; D000368:Aged; D018122:B7-1 Antigen / Q000235:genetics / Q000378:metabolism / Q000652:urine*; D016022:Case-Control Studies; D002478:Cells, Cultured; D002648:Child; D000795:Fabry Disease / Q000378:metabolism / Q000473:pathology* / Q000652:urine*; D005260:Female; D006017:Glycolipids / Q000378:metabolism; D006801:Humans; D008297:Male; D008875:Middle Aged; D050199:Podocytes / Q000378:metabolism* / Q000473:pathology*; D012333:RNA, Messenger / Q000235:genetics / Q000378:metabolism; D013107:Sphingolipids / Q000378:metabolism; D055815:Young Adult
chemical_list: D018122:B7-1 Antigen; D006017:Glycolipids; D012333:RNA, Messenger; D013107:Sphingolipids; C063288:globotriaosyl lysosphingolipid
keywords: CD80; Enzyme replacement therapy; Fabry disease; Lyso-Gb3; Podocyte; Podocyturia; Proteinuria