Deprecated: Implicit conversion from float 211.6 to int loses precision in C:\Inetpub\vhosts\kidney.de\httpdocs\pget.php on line 534
Deprecated: Implicit conversion from float 211.6 to int loses precision in C:\Inetpub\vhosts\kidney.de\httpdocs\pget.php on line 534
Deprecated: Implicit conversion from float 211.6 to int loses precision in C:\Inetpub\vhosts\kidney.de\httpdocs\pget.php on line 534
Deprecated: Implicit conversion from float 211.6 to int loses precision in C:\Inetpub\vhosts\kidney.de\httpdocs\pget.php on line 534
Deprecated: Implicit conversion from float 211.6 to int loses precision in C:\Inetpub\vhosts\kidney.de\httpdocs\pget.php on line 534
Deprecated: Implicit conversion from float 211.6 to int loses precision in C:\Inetpub\vhosts\kidney.de\httpdocs\pget.php on line 534
Deprecated: Implicit conversion from float 211.6 to int loses precision in C:\Inetpub\vhosts\kidney.de\httpdocs\pget.php on line 534
Deprecated: Implicit conversion from float 211.6 to int loses precision in C:\Inetpub\vhosts\kidney.de\httpdocs\pget.php on line 534
Deprecated: Implicit conversion from float 245.2 to int loses precision in C:\Inetpub\vhosts\kidney.de\httpdocs\pget.php on line 534
Deprecated: Implicit conversion from float 245.2 to int loses precision in C:\Inetpub\vhosts\kidney.de\httpdocs\pget.php on line 534
Warning: imagejpeg(C:\Inetpub\vhosts\kidney.de\httpdocs\phplern\29325224
.jpg): Failed to open stream: No such file or directory in C:\Inetpub\vhosts\kidney.de\httpdocs\pget.php on line 117 Cancer+Sci
2018 ; 109
(3
): 581-586
Nephropedia Template TP
gab.com Text
Twit Text FOAVip
Twit Text #
English Wikipedia
Hereditary kidney cancer syndromes: Genetic disorders driven by alterations in
metabolism and epigenome regulation
#MMPMID29325224
Hasumi H
; Yao M
Cancer Sci
2018[Mar]; 109
(3
): 581-586
PMID29325224
show ga
Although hereditary kidney cancer syndrome accounts for approximately five
percent of all kidney cancers, the mechanistic insight into tumor development in
these rare conditions has provided the foundation for the development of
molecular targeting agents currently used for sporadic kidney cancer. In the late
1980s, the comprehensive study for hereditary kidney cancer syndrome was launched
in the National Cancer Institute, USA and the first kidney cancer-associated
gene, VHL, was identified through kindred analysis of von Hippel-Lindau (VHL)
syndrome in 1993. Subsequent molecular studies on VHL function have elucidated
that the VHL protein is a component of E3 ubiquitin ligase complex for
hypoxia-inducible factor (HIF), which provided the basis for the development of
tyrosine kinase inhibitors targeting the HIF-VEGF/PDGF pathway. Recent
whole-exome sequencing analysis of sporadic kidney cancer exhibited the recurrent
mutations in chromatin remodeling genes and the later study has revealed that
several chromatin remodeling genes are altered in kidney cancer kindred at the
germline level. To date, more than 10 hereditary kidney cancer syndromes together
with each responsible gene have been characterized and most of the causative
genes for these genetic disorders are associated with either metabolism or
epigenome regulation. In this review article, we describe the molecular
mechanisms of how an alteration of each kidney cancer-associated gene leads to
renal tumorigenesis as well as denote therapeutic targets elicited by studies on
hereditary kidney cancer.