M-type phospholipase A2 receptor (PLA2R) and thrombospondin type-1 domain-containing 7A (THSD7A) in membranous nephropathy

Research output: Chapter in Book/Report/Conference proceedingChapter

Abstract

Membranous nephropathy (MN) is the most common cause of nephrotic syndrome in Caucasians adults. It is defined by the presence of subepithelial immune deposits localized between the podocyte and the glomerular basement membrane (GBM) on electron microscopy examination (EM). Clinical course is variable: although up to 30 % of patients may go into spontaneous remission, approximately 40 % of patients eventually develop ESRD. The discovery of two major podocytes antigens in adults: first, the M-type phospholipase A2 receptor 1 (PLA2R1) and more recently, the thrombospondin type-1 domain-containing 7A (THSD7A) protein has revolutionized our understanding of the pathogenesis of human MN. Approximately 75 % of patients with active disease have circulating anti-PLA2R autoantibodies, and up to 10 % of the patients with MN that are anti- PLA2R negative have antibodies against THSD7A. Quantification and follow-up of anti-PLA2R levels have major implications regarding prognosis and treatment response. The presence of anti-PLA2R antibodies can also predict disease recurrence following kidney transplantation. Genetic studies are elucidating predisposing factors for development of the disease. Further research into the antigen-autoantibody systems is likely to elucidate a clearer understanding of the pathophysiology and treatment of patients with MN.

Original languageEnglish (US)
Title of host publicationMolecular Mechanisms in the Pathogenesis of Idiopathic Nephrotic Syndrome
PublisherSpringer Japan
Pages181-206
Number of pages26
ISBN (Print)9784431552703, 9784431552697
DOIs
StatePublished - Jan 1 2016

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Phospholipase A2 Receptors
Thrombospondin 1
Membranous Glomerulonephritis
Podocytes
Autoantibodies
Spontaneous Remission
Antigens
Glomerular Basement Membrane
Antibodies
Nephrotic Syndrome
Causality
Kidney Transplantation
Electron microscopy
Chronic Kidney Failure
Electron Microscopy
Deposits
Recurrence
Therapeutics
Research

Keywords

  • M-type phospholipase a2 receptor 1
  • Membranous nephropathy
  • Pla2r1
  • Thrombospondin type-1 domain-containing 7a protein
  • Thsd7a
  • Tissue staining
  • Transplantation

ASJC Scopus subject areas

  • Medicine(all)
  • Biochemistry, Genetics and Molecular Biology(all)

Cite this

M-type phospholipase A2 receptor (PLA2R) and thrombospondin type-1 domain-containing 7A (THSD7A) in membranous nephropathy. / Beck, Laurence H.; Sethi, Sanjeev M; Fervenza, Fernando Custodio.

Molecular Mechanisms in the Pathogenesis of Idiopathic Nephrotic Syndrome. Springer Japan, 2016. p. 181-206.

Research output: Chapter in Book/Report/Conference proceedingChapter

Beck, Laurence H. ; Sethi, Sanjeev M ; Fervenza, Fernando Custodio. / M-type phospholipase A2 receptor (PLA2R) and thrombospondin type-1 domain-containing 7A (THSD7A) in membranous nephropathy. Molecular Mechanisms in the Pathogenesis of Idiopathic Nephrotic Syndrome. Springer Japan, 2016. pp. 181-206
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abstract = "Membranous nephropathy (MN) is the most common cause of nephrotic syndrome in Caucasians adults. It is defined by the presence of subepithelial immune deposits localized between the podocyte and the glomerular basement membrane (GBM) on electron microscopy examination (EM). Clinical course is variable: although up to 30 {\%} of patients may go into spontaneous remission, approximately 40 {\%} of patients eventually develop ESRD. The discovery of two major podocytes antigens in adults: first, the M-type phospholipase A2 receptor 1 (PLA2R1) and more recently, the thrombospondin type-1 domain-containing 7A (THSD7A) protein has revolutionized our understanding of the pathogenesis of human MN. Approximately 75 {\%} of patients with active disease have circulating anti-PLA2R autoantibodies, and up to 10 {\%} of the patients with MN that are anti- PLA2R negative have antibodies against THSD7A. Quantification and follow-up of anti-PLA2R levels have major implications regarding prognosis and treatment response. The presence of anti-PLA2R antibodies can also predict disease recurrence following kidney transplantation. Genetic studies are elucidating predisposing factors for development of the disease. Further research into the antigen-autoantibody systems is likely to elucidate a clearer understanding of the pathophysiology and treatment of patients with MN.",
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