PMID- 38286722
OWN - NLM
STAT- MEDLINE
DCOM- 20240216
LR  - 20240223
IS  - 2013-2514 (Electronic)
IS  - 2013-2514 (Linking)
VI  - 43 Suppl 2
DP  - 2023 Dec
TI  - Isolation and characterization of exosome-enriched urinary extracellular vesicles 
      from Dent's disease type 1 Spanish patients.
PG  - 77-84
LID - S2013-2514(24)00026-9 [pii]
LID - 10.1016/j.nefroe.2024.01.016 [doi]
AB  - BACKGROUND AND OBJECTIVES: Dent's disease type 1 (DD1) is a rare X-linked 
      hereditary pathology caused by CLCN5 mutations that is characterized mainly by 
      proximal tubule dysfunction, hypercalciuria, nephrolithiasis/nephrocalcinosis, 
      progressive chronic kidney disease, and low-weight proteinuria, the molecular 
      hallmark of the disease. Currently, there is no specific curative treatment, only 
      symptomatic and does not prevent the progression of the disease. In this study we 
      have isolated and characterized urinary extracellular vesicles (uEVs) enriched in 
      exosomes that will allow us to identify biomarkers associated with DD1 
      progression and a better understanding of the pathophysiological bases of the 
      disease. MATERIALS AND METHODS: Through a national call from the Spanish Society 
      of Nephrology (SEN) and the Spanish Society of Pediatric Nephrology (AENP), urine 
      samples were obtained from patients and controls from different Spanish 
      hospitals, which were processed to obtain the uEVS. The data of these patients 
      were provided by the respective nephrologists and/or extracted from the RENALTUBE 
      registry. The uEVs were isolated by ultracentrifugation, morphologically 
      characterized and their protein and microRNA content extracted. RESULTS: 25 
      patients and 10 controls were recruited, from which the urine was processed to 
      isolate the uEVs. Our results showed that the relative concentration of uEVs/mL 
      is lower in patients compared to controls (0.26 x 10(6) uEVs/mL vs 1.19 x 10(6) 
      uEVs/mL, p < 0.01). In addition, the uEVs of the patients were found to be 
      significantly larger than those of the control subjects (mean diameter: 187.8 nm 
      vs 143.6 nm, p < 0.01). Finally, our data demonstrated that RNA had been 
      correctly extracted from both patient and control exosomes. CONCLUSIONS: In this 
      work we describe the isolation and characterization of uEVs from patients with 
      Dent 1 disease and healthy controls, that shall be useful for the subsequent 
      study of differentially expressed cargo molecules in this pathology.
CI  - Copyright (c) 2023 Sociedad Espanola de Nefrologia. Published by Elsevier Espana, 
      S.L.U. All rights reserved.
FAU - Burballa, Carla
AU  - Burballa C
AD  - Departamento de Nefrologia, Hospital del Mar, Barcelona, Spain.
FAU - Duran, Monica
AU  - Duran M
AD  - Grupo de Fisiopatologia Renal, Institut de Recerca Vall d'Hebron (VHIR), 
      Barcelona, Spain.
FAU - Martinez, Cristina
AU  - Martinez C
AD  - Grupo de Fisiopatologia Renal, Institut de Recerca Vall d'Hebron (VHIR), 
      Barcelona, Spain; Grupo de Investigacion Traslacional Vascular y Renal, 
      IRB-Lleida, Lleida, Spain.
FAU - Ariceta, Gema
AU  - Ariceta G
AD  - Servicio de Nefrologia Pediatrica, Hospital Universitario Vall d'Hebron (HUVH), 
      Barcelona, Spain; Departament de Bioquimica i Biologia Molecular, Unitat de 
      Bioquimica de Medicina, Universitat Autonoma de Barcelona (UAB), Barcelona, 
      Spain.
FAU - Cantero-Recasens, Gerard
AU  - Cantero-Recasens G
AD  - Grupo de Fisiopatologia Renal, Institut de Recerca Vall d'Hebron (VHIR), 
      Barcelona, Spain.
FAU - Meseguer, Anna
AU  - Meseguer A
AD  - Grupo de Fisiopatologia Renal, Institut de Recerca Vall d'Hebron (VHIR), 
      Barcelona, Spain; Departament de Bioquimica i Biologia Molecular, Unitat de 
      Bioquimica de Medicina, Universitat Autonoma de Barcelona (UAB), Barcelona, 
      Spain. Electronic address: ana.meseguer@vhir.org.
LA  - eng
PT  - Journal Article
PT  - Research Support, Non-U.S. Gov't
DEP - 20240128
PL  - Spain
TA  - Nefrologia (Engl Ed)
JT  - Nefrologia
JID - 101778581
RN  - 0 (MicroRNAs)
SB  - IM
MH  - Child
MH  - Humans
MH  - *Dent Disease/genetics/metabolism
MH  - *Exosomes/metabolism
MH  - *Nephrolithiasis
MH  - *Nephrocalcinosis/genetics
MH  - *MicroRNAs
OTO - NOTNLM
OT  - CLCN5
OT  - DD1
OT  - Dent's disease
OT  - Enfermedad de Dent
OT  - Exosomas
OT  - Exosomes
OT  - Extracelular vesicles
OT  - Vesiculas extracelulares
EDAT- 2024/01/30 00:42
MHDA- 2024/02/16 06:43
CRDT- 2024/01/29 21:53
PHST- 2023/02/22 00:00 [received]
PHST- 2023/06/13 00:00 [accepted]
PHST- 2024/02/16 06:43 [medline]
PHST- 2024/01/30 00:42 [pubmed]
PHST- 2024/01/29 21:53 [entrez]
AID - S2013-2514(24)00026-9 [pii]
AID - 10.1016/j.nefroe.2024.01.016 [doi]
PST - ppublish
SO  - Nefrologia (Engl Ed). 2023 Dec;43 Suppl 2:77-84. doi: 
      10.1016/j.nefroe.2024.01.016. Epub 2024 Jan 28.