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Hereditary renal hypouricemia type 1 and autosomal dominant polycystic kidney disease

The result's identifiers

  • Result code in IS VaVaI

    <a href="https://www.isvavai.cz/riv?ss=detail&h=RIV%2F00023728%3A_____%2F15%3A%230005035" target="_blank" >RIV/00023728:_____/15:#0005035 - isvavai.cz</a>

  • Alternative codes found

    RIV/00216208:11110/15:10306882 RIV/00064165:_____/15:10306882

  • Result on the web

    <a href="http://apps.webofknowledge.com/full_record.do?product=UA&search_mode=GeneralSearch&qid=1&SID=Y2RsBNomTQf3ElNG91B&page=1&doc=1" target="_blank" >http://apps.webofknowledge.com/full_record.do?product=UA&search_mode=GeneralSearch&qid=1&SID=Y2RsBNomTQf3ElNG91B&page=1&doc=1</a>

  • DOI - Digital Object Identifier

    <a href="http://dx.doi.org/10.1097/MAJ.0000000000000550" target="_blank" >10.1097/MAJ.0000000000000550</a>

Alternative languages

  • Result language

    angličtina

  • Original language name

    Hereditary renal hypouricemia type 1 and autosomal dominant polycystic kidney disease

  • Original language description

    Renal hypouricemia is a heterogeneous inherited disorder characterized by impaired tubular uric acid transport with severe complications, such as acute kidney injury. In this article, the authors present a coexpression functional characterization of variants responsible for renal hypouricemia type 1 in a Czech family with polycystic kidney disease. The findings suggest that not only loss-of-function mutation of URAT1 but also the dominant-negative effect cause renal hypouricemia through loss of uric acid absorption, partly due to protein misfolding caused by accumulation of URAT1 protein in the endoplasmic reticulum.

  • Czech name

  • Czech description

Classification

  • Type

    J<sub>x</sub> - Unclassified - Peer-reviewed scientific article (Jimp, Jsc and Jost)

  • CEP classification

    FE - Other fields of internal medicine

  • OECD FORD branch

Result continuities

  • Project

    <a href="/en/project/LH13245" target="_blank" >LH13245: Molecular pathology and genetic diagnostics of renal hypouricemia</a><br>

  • Continuities

    V - Vyzkumna aktivita podporovana z jinych verejnych zdroju

Others

  • Publication year

    2015

  • Confidentiality

    S - Úplné a pravdivé údaje o projektu nepodléhají ochraně podle zvláštních právních předpisů

Data specific for result type

  • Name of the periodical

    AMERICAN JOURNAL OF THE MEDICAL SCIENCES

  • ISSN

    0002-9629

  • e-ISSN

  • Volume of the periodical

    350

  • Issue of the periodical within the volume

    4

  • Country of publishing house

    US - UNITED STATES

  • Number of pages

    4

  • Pages from-to

    268-271

  • UT code for WoS article

    000362218400006

  • EID of the result in the Scopus database