TY - JOUR
T1 - Recent advances in understanding ion transport mechanisms in polycystic kidney disease
AU - Sudarikova, Anastasia V.
AU - Vasileva, Valeriia Y.
AU - Sultanova, Regina F.
AU - Ilatovskaya, Daria V.
N1 - Funding Information:
This work was supported by the APS Lazaro J. Mandel Young Investigator Award, NIH grants NIDDK R00 DK105160, NHLBI R01HL148114; and the PKD Foundation Award [grant number 221G18a (all to D.V.I.)].
Publisher Copyright:
© 2021 The Author(s).
PY - 2021/11
Y1 - 2021/11
N2 - This review focuses on the most recent advances in the understanding of the electrolyte transport-relatedmechanisms important for the development of severe inherited renal disorders, autosomal dominant (AD) and recessive (AR) forms of polycystic kidney disease (PKD). We provide here a basic overview of the origins and clinical aspects of ARPKD and ADPKD and discuss the implications of electrolyte transport in cystogenesis. Special attention is devoted to intracellular calcium handling by the cystic cells, with a focus on polycystins and fibrocystin, as well as other calcium level regulators, such as transient receptor potential vanilloid type 4 (TRPV4) channels, ciliary machinery, and purinergic receptor remodeling. Sodium transport is reviewed with a focus on the epithelial sodium channel (ENaC), and the role of chloride-dependent fluid secretion in cystic fluid accumulation is discussed. In addition, we highlight the emerging promising concepts in the field, such as potassium transport, and suggest some new avenues for research related to electrolyte handling.
AB - This review focuses on the most recent advances in the understanding of the electrolyte transport-relatedmechanisms important for the development of severe inherited renal disorders, autosomal dominant (AD) and recessive (AR) forms of polycystic kidney disease (PKD). We provide here a basic overview of the origins and clinical aspects of ARPKD and ADPKD and discuss the implications of electrolyte transport in cystogenesis. Special attention is devoted to intracellular calcium handling by the cystic cells, with a focus on polycystins and fibrocystin, as well as other calcium level regulators, such as transient receptor potential vanilloid type 4 (TRPV4) channels, ciliary machinery, and purinergic receptor remodeling. Sodium transport is reviewed with a focus on the epithelial sodium channel (ENaC), and the role of chloride-dependent fluid secretion in cystic fluid accumulation is discussed. In addition, we highlight the emerging promising concepts in the field, such as potassium transport, and suggest some new avenues for research related to electrolyte handling.
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U2 - 10.1042/CS20210370
DO - 10.1042/CS20210370
M3 - Review article
C2 - 34751394
AN - SCOPUS:85119592206
SN - 0143-5221
VL - 135
SP - 2521
EP - 2540
JO - Clinical Science
JF - Clinical Science
IS - 21
ER -