Reduction of cellular expression levels is a common feature of functionally affected pendrin (SLC26A4) protein variants

VCS de Moraes, E Bernardinelli, N Zocal… - Molecular …, 2016 - Springer
Sequence alterations in the pendrin gene (SLC26A4) leading to functionally affected protein
variants are frequently involved in the pathogenesis of syndromic and nonsyndromic …

Integration of human and mouse genetics reveals pendrin function in hearing and deafness

AA Dror, Z Brownstein, KB Avraham - Cellular Physiology and …, 2011 - karger.com
Genomic technology has completely changed the way in which we are able to diagnose
human genetic mutations. Genomic techniques such as the polymerase chain reaction …

Systematic quantification of the anion transport function of pendrin (SLC26A4) and its disease‐associated variants

K Wasano, S Takahashi, SK Rosenberg… - Human …, 2020 - Wiley Online Library
Thanks to the advent of rapid DNA sequencing technology and its prevalence, many
disease‐associated genetic variants are rapidly identified in many genes from patient …

Functional characterization of pendrin mutations found in the Israeli and Palestinian populations

S Dossena, C Nofziger, Z Brownstein… - Cellular Physiology and …, 2011 - karger.com
Background: Pendrin is a transport protein exchanging chloride for other anions, such as
iodide in the thyroid gland or bicarbonate in the inner ear. Mutations in the SLC26A4 gene …

Identification of allelic variants of pendrin (SLC26A4) with loss and gain of function

S Dossena, A Bizhanova, C Nofziger… - Cellular physiology and …, 2011 - karger.com
Background: Pendrin is a multifunctional anion transporter that exchanges chloride and
iodide in the thyroid, as well as chloride and bicarbonate in the inner ear, kidney and …

The ESF Meeting on „The Proteomics, Epigenetics and Pharmacogenetics of Pendrin “

S Dossena, C Nofziger, F Lang, G Valenti… - Cellular Physiology and …, 2011 - karger.com
Abstract Human pendrin (SCL26A4, PDS) is a 780 amino acid integral membrane protein
with transport function. It acts as an electroneutral, sodium-independent anion exchanger for …

The pendrin polypeptide

S Dossena, E Bernardinelli, AK Sharma… - The Role of Pendrin in …, 2017 - Springer
Abstract Pendrin (SLC26A4) is an electroneutral anion exchanger in which function-altering
mutations cause inherited forms of non-syndromic (DFNB4) and syndromic deafness …

Heterogeneity in the processing defect of SLC26A4 mutants

JS Yoon, HJ Park, SY Yoo, W Namkung… - Journal of medical …, 2008 - jmg.bmj.com
Background: Mutations in the SLC26A4 gene are responsible for Pendred syndrome and
non-syndromic hearing loss (DFNB4). This study analysed non-synonymous SLC26A4 …

Mapping pathogenic mutations suggests an innovative structural model for the pendrin (SLC26A4) transmembrane domain

C Bassot, G Minervini, E Leonardi, SCE Tosatto - Biochimie, 2017 - Elsevier
Abstract Human pendrin (SLC26A4) is an anion transporter mostly expressed in the inner
ear, thyroid and kidney. SLC26A4 gene mutations are associated with a broad phenotypic …

Functional characterization of wild-type and mutated pendrin (SLC26A4), the anion transporter involved in Pendred syndrome.

S Dossena, S Rodighiero, V Vezzoli… - Journal of molecular …, 2009 - europepmc.org
Pendred syndrome (PS) is the most frequent form of genetically related syndromic hearing
loss, and is associated with mutations of pendrin, encoded by the SLC26A4 gene. This …