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TRPML-1 Dysfunction and Renal Tubulopathy in Mucolipidosis Type IV.
J Am Soc Nephrol
December 2024
Telethon Institute of Genetics and Medicine (TIGEM), Pozzuoli, Naples, Italy.
Article Synopsis
- * A study reviewed 21 MLIV patients and conducted experiments on MLIV mouse models to understand kidney function, revealing that adult patients often have chronic kidney disease along with altered kidney functions and structure in the mice due to issues with endolysosomal function and autophagy.
- * Results showed decreased kidney function, presence of fibrosis and inflammation in MLIV models, and impaired uptake of essential proteins, which highlights cellular and systemic dysfunction in the
Attenuation of diabetic nephropathy by dietary fenugreek (Trigonella foenum-graecum) seeds and onion (Allium cepa) via suppression of glucose transporters and renin-angiotensin system.
Nutrition
September 2020
Department of Biochemistry, Central Food Technological Research Institute, Mysore, India. Electronic address:
Objectives: The aim of this study was to determine the effects of dietary fenugreek (Trigonella foenum-graecum) seeds and onion on the hyperglycemia-stimulated glucose transporters and activation of renin-angiotensin system-mediated cascade of events leading to renal lesions in diabetic animals.
Methods: The mechanistic aspects of nephroprotective influence of dietary fenugreek seeds (10%) and onion (3%) on diabetic renal lesions was investigated in streptozotocin diabetic rats. Renal damage was assessed by measuring proteinuria, enzymuria, expression of glucose transporters, renin-angiotensin system, and activities of polyol pathway enzymes.
Biomarkers and renal arterial resistive index in dogs naturally infected with Leishmania infantum.
Parasitol Res
November 2018
Faculty of Veterinary Medicine and Zootechnics, Federal University of Mato Grosso do Sul (UFMS), Campo Grande, Mato Grosso do Sul, 79070-900, Brazil.
Canine visceral leishmaniasis frequently causes glomerulonephritis and tubulointerstitial nephritis, nephropathies for which diagnosis has been limited by the low sensitivity of traditional tests. The aim of this study was to evaluate serum cystatin C and urinary gamma-glutamyltransferase (uGGT) levels and the urinary GGT/urinary creatinine ratio (uGGT/uCR) and to measure the renal arterial resistive index (RARI) in dogs with leishmaniasis with varying degrees of renal injury based on the urine protein: creatinine ratio (UP/C) and serum creatinine (SCr) level. We tested 59 untreated adult dogs of both sexes and undefined breeds naturally infected with Leishmania infantum.
View Article and Find Full Text PDFTubular Dysfunction Mimicking Dent's Disease in 2 Infants Born with Extremely Low Birth Weight.
Case Rep Nephrol Dial
January 2017
Department of Pediatrics, Keio University School of Medicine, Tokyo, Japan.
Two preterm infants, with extremely low birth weight born at gestational weeks 24 and 25, showed generalized proximal tubular dysfunction during their stay in the neonatal intensive care unit, including glucosuria, low molecular weight proteinuria, phosphaturia, uricosuria, enzymuria (elevated urine N-acetyl-β-D-glucosaminidase), panaminoaciduria, and hypercalciuria, associated with renal calcification. Renal tubular acidosis was not present in either patient. DNA mutation analysis for Dent's disease, performed in patient 1, was negative.
View Article and Find Full Text PDFProtective effect of nitric oxide in aristolochic acid-induced toxic acute kidney injury: an old friend with new assets.
Exp Physiol
January 2016
Molecular Physiology Research Unit-URPHYM, University of Namur (UNamur), B-5000, Namur, Belgium.
Aristolochic acid (AA) nephropathy (AAN), a progressive tubulointerstitial injury of toxic origin, is characterized by early and transient acute tubular necrosis. This process has been demonstrated to be associated with reduced nitric oxide (NO) production, which can disrupt the regulation of renal function. In this study, we tested the hypothesis that L-arginine (L-Arg) supplementation could restore renal function and reduce renal injury after AA intoxication.
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