ArticleClinical journal of the American Society of Nephrology : CJASN2013
Prognostic value of glomerular collagen IV immunofluorescence studies in male patients with X-linked Alport syndrome.
Article in Clinical journal of the American Society of Nephrology : CJASN, 2013. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 10 papers.
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10 citing papers in PubMed, 18 citations in OpenAlex.
- Effects of a Novel COL4A3 Homozygous/Heterozygous Splicing Mutation on the Mild Phenotype in a Family With Autosomal Recessive Alport Syndrome and a Literature Review.Molecular genetics & genomic medicine · 2025Review
- Alport Syndrome With a Rare Collagen Type IV Alpha-4 (COL4A4) Gene Mutation: A Case Report.Cureus · 2024Article
- A novel missense mutation ofTranslational pediatrics · 2020Article
- An overview of the multi-pronged approach in the diagnosis of Alport syndrome for 22 children in Northeast China.BMC nephrology · 2020Article
- A NovelKidney diseases (Basel, Switzerland) · 2020Article
- Somatic Mosaicism in a Male Patient With X-linked Alport Syndrome.Kidney international reports · 2019Article
- Novel deletion mutation in a Chinese family with X-linked alport syndrome.International journal of clinical and experimental pathology · 2018Article
- Negative Staining for COL4A5 Correlates With Worse Prognosis and More Severe Ultrastructural Alterations in Males With Alport Syndrome.Kidney international reports · 2017Article
- Alport syndrome: facts and opinions.F1000Research · 2017Review
- Identification of 47 novel mutations in patients with Alport syndrome and thin basement membrane nephropathy.Pediatric nephrology (Berlin, Germany) · 2016Article
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9 authors at 5 institutions in 2 countries.
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Abstract
BACKGROUND AND
objectivesX-linked Alport syndrome (X-AS) is caused by mutations of the COL4A5 gene, which encodes for the collagen IV α5 chain (α5[COLIV]), resulting in structural and functional abnormalities of the glomerular basement membrane (GBM) and leading to CKD. The aim of the present study was to evaluate the prognostic value of residual collagen IV chain expression in the GBM of patients with X-AS. DESIGN, SETTING, PARTICIPANTS, & MEASUREMENTS: The medical records of 22 patients with X-AS from 21 unrelated families collected between 1987 and 2009 were reviewed (median age at last follow-up, 19.9 years; range, 5.4-35.1 years); GBM expression of α1, α3, and α5(COLIV) chains was assessed by immunofluorescence microscopy.
resultsGBM distribution of the α5(COLIV) chain was diffuse in 1 and segmental or absent in 21 of the 22 patients; the expression of the α3(COLIV) chain was diffuse in 5 of 22 patients and segmental or absent in 17 of 22 patients. Patients with diffuse staining for the α3(COLIV) chain presented with proteinuria significantly later (median age, 16.9 versus 6.1 years; P=0.02) and reached an estimated GFR < 90 ml/min per 1.73 m(2) at an older age (median age, 27.0 versus 14.9 years; P=0.01) compared with patients with segmental or absent staining. Two thirds of patients with abnormal α3(COLIV) expression by immunofluorescence studies had null or truncating COL4A5 mutations, as opposed to none of the 4 tested patients with diffuse α3(COLIV) chain glomerular distribution.
conclusionsThese results indicate that maintained expression of the α3(COLIV) chain is an early positive prognostic marker in patients with X-linked Alport symdrome.
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