Erythropoietin production by distal nephron in normal and familial amyloidotic adult human kidneys

• Type: Article in International Scientific Journal
• Date: 2010

Authors

beirao, i (Author) ICBAS; moreira, l (Author) Other; barandela, t (Author) Other; lobato, l (Author) ICBAS; silva, p (Author) Other; gouveia, cm (Author) Other; carneiro, f (Author) FMUP; fonseca, i (Author) Other; porto, g (Author) ICBAS; costa, ppe (Author) ICBAS

Journal

Title: Clinical Nephrology

Vol. 74

Pages: 327-335

ISSN: 0301-0430

Publisher: Dustri-Verlag Dr. Karl Feistle

Indexing

ISI Web of Knowledge - 9 Citations

Scopus - 8 Citations

Scientific classification

FOS: Medical and Health sciences > Clinical medicine

Other information

Authenticus ID: P-003-19B

Abstract (EN): Background/Aim The kidney is the major site of erythropoietin production Many efforts have been made to identify renal erythropoietin-producing cells Previous studies showed conflicting results, but the predominant localization reported was the peritubular interstitial and tubular epithelial cells This study was conducted to identify the erythropoietin-producing cells in renal biopsies from 10 cadaveric donors and 45 patients with familial amyloidosis ATTR V30M, thirteen of them with anemia Familial amyloidosis Type I (FAP-I) is a genetic disorder caused by a transthyretin (TTR) protein variant presenting a single amino acid substitution of methionine for valine at position 30 of the polypeptide chain (TTR V30M) Anemia in FAP-I is associated with inappropriately low serum erythropoietin levels Methods Erythropoietin expression was detected by in situ hybridization (ISH) and confirmed by laser capture microdissection followed by PCR Renal segments were identified by immuno-histochemistry Results Erythropoietin was mainly expressed by epithelial distal tubular cells and collecting tubules and additionally, in a few biopsies, by glomerular cells A similar expression pattern was observed in donors and FAP-I patients No increased mRNA erythropoietin expression was found in anemic patients, all of them presenting only a slight expression in medulla and cortex Conclusions These results suggest the distal nephron as the major site of erythropoietin production, and support the notion that an inappropriate erythropoietin production is the cause of anemia in familial amyloidosis ATTR V30M

Language: English

Type (Professor's evaluation): Scientific

No. of pages: 9