Primary Membranous Nephropathy With Enhanced Staining of Exostosin 1/Exostosin 2 in the Glomeruli: A Report of 2 Cases [PDF]
Kidney Medicine, 2021 Technological advances have allowed the discovery of 6 subtypes of membranous nephropathy based on target antigens: M-type phospholipase A2 receptor (PLA2R), thrombospondin type 1 domain-containing 7A (THSD7A), neural epidermal growth factor-like 1 ...
Takamasa Iwakura +7 more
doaj +5 more sources
Prevalence of neural epidermal growth factor-like 1- and exostosin 1/exostosin 2-associated membranous nephropathy: a single-center retrospective study in Japan [PDF]
Scientific Reports, 2022 Membranous nephropathy (MN) is the leading cause of nephrotic syndrome in adults. We previously reported that the prevalence of phospholipase A2 receptor (PLA2R)- and thrombospondin type 1 domain containing 7A (THSD7A)-associated MN patients in Japan is ...
Takamasa Iwakura +6 more
doaj +5 more sources
Clinical significance of exostosin 1 in confirmed and suspected lupus membranous nephropathy [PDF]
Lupus Science and Medicine, 2023 Objective This study aimed to investigate the clinical significance of exostosin 1 (EXT1) in confirmed and suspected lupus membranous nephropathy (LMN).Methods EXT1 was detected in 67 renal tissues of M-type phospholipase A2 receptor (PLA2R)-negative and
Xiaohong Li +7 more
doaj +4 more sources
Exostosin 1/exostosin 2-associated membranous nephropathy in undifferentiated connective tissue disease: a case report and literature review [PDF]
BMC NephrologyBackground Renal involvement in undifferentiated connective tissue disease (UCTD) is rare and not well characterized, with membranous nephropathy (MN) representing an uncommon but clinically significant manifestation. While exostosin 1/exostosin 2 (EXT 1/
Huan Ye +4 more
doaj +3 more sources
Rare case of exostosin 1/exostosin 2-related membranous lupus nephritis concomitant with dual ANCA- and anti-GBM antibody-associated crescentic glomerulonephritis effectively diagnosed by mass spectrometry: a case report [PDF]
BMC Nephrology, 2023 Background Recent developments in mass spectrometry (MS) have revealed target antigens for membranous nephropathy (MN), including phospholipase A2 receptor and exostosin 1/exostosin 2 (EXT1/2).
Takuya Yamazaki +11 more
doaj +4 more sources
Case Report: Exostosin 1-associated membranous nephropathy and Guillain-Barré syndrome: a common autoimmune etiology? [PDF]
Frontiers in NephrologyMembranous nephropathy is one of the most common causes of nephrotic syndrome in adults and is caused by the deposition of immune complexes in the subepithelial space of the glomerular basement membranes.
Mariana León-Póo +7 more
doaj +4 more sources
Exostosin 1/Exostosin 2-Associated Membranous Nephropathy. [PDF]
J Am Soc Nephrol, 2019 Significance Statement In approximately 70%–80% of cases of primary membranous nephropathy (MN), phospholipase A2 receptor (PLA2R)/Thrombospondin Type-1 Domain–Containing 7A (THSD7A) and anti-PLA2R/THSD7A antibodies form immune complexes along the glomerular basement membrane (GBM) that characterize the condition.
Sethi S +9 more
europepmc +4 more sources
Identification of Two Novel Frameshift Mutations in Exostosin 1 in Two Families with Multiple Osteochondromas. [PDF]
Mol Syndromol, 2021 Multiple osteochondromas (MO) is an autosomal dominant hereditary disorder, which typically manifests as skeletal dysplasia, mainly involving long bones and knees, ankles, elbows, wrists, shoulders, and pelvis. Previous studies have demonstrated that mutations in exostosin glycosyl transferase-1 (<i>EXT1</i>) and exostosin glycosyl ...
Wang CY +6 more
europepmc +4 more sources
A Case of Pla2r and Exostosin 1 Positive Membranous Nephropathy - The Diagnostic and Therapeutic Dilemma. [PDF]
Cureus, 2023 Membranous nephropathy is an immune disease that commonly presents as nephrotic syndrome. The understanding of the pathogenesis of membranous nephropathy has rapidly evolved over the past few years due to the discovery of newer antigens. Exostosin 1 and exostosin 2 are antigens discovered in 2019 and found to be specific for membranous nephropathy ...
Sivagnanam H +4 more
europepmc +3 more sources
A Diagnostic and Therapeutic Dilemma Concerning Exostosin 1/Exostosin 2-associated Lupus-like Membranous Nephropathy with Positive Antinuclear Antibody in an Elderly Man with Various Immune Abnormalities. [PDF]
Intern Med, 2023 Exostosin 1 (EXT1) and exostosin 2 (EXT2)-associated membranous nephropathy (MN) may be associated with active autoimmune disease. We encountered an elderly man who presented with EXT1/EXT2-associated lupus-like MN with full house immune deposits, monoclonal gammopathy of uncertain significance and Sjögren's syndrome.
Kawagoe M +11 more
europepmc +3 more sources