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.jpg): Failed to open stream: No such file or directory in C:\Inetpub\vhosts\kidney.de\httpdocs\pget.php on line 117 J+Biol+Chem
2014 ; 289
(52
): 36070-88
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English Wikipedia
Whirlin and PDZ domain-containing 7 (PDZD7) proteins are both required to form
the quaternary protein complex associated with Usher syndrome type 2
#MMPMID25406310
Chen Q
; Zou J
; Shen Z
; Zhang W
; Yang J
J Biol Chem
2014[Dec]; 289
(52
): 36070-88
PMID25406310
show ga
Usher syndrome (USH) is the leading genetic cause of combined hearing and vision
loss. Among the three USH clinical types, type 2 (USH2) occurs most commonly.
USH2A, GPR98, and WHRN are three known causative genes of USH2, whereas PDZD7 is
a modifier gene found in USH2 patients. The proteins encoded by these four USH
genes have been proposed to form a multiprotein complex, the USH2 complex, due to
interactions found among some of these proteins in vitro, their colocalization in
vivo, and mutual dependence of some of these proteins for their normal in vivo
localizations. However, evidence showing the formation of the USH2 complex is
missing, and details on how this complex is formed remain elusive. Here, we
systematically investigated interactions among the intracellular regions of the
four USH proteins using colocalization, yeast two-hybrid, and pull-down assays.
We show that multiple domains of the four USH proteins interact among one
another. Importantly, both WHRN and PDZD7 are required for the complex formation
with USH2A and GPR98. In this USH2 quaternary complex, WHRN prefers to bind to
USH2A, whereas PDZD7 prefers to bind to GPR98. Interaction between WHRN and PDZD7
is the bridge between USH2A and GPR98. Additionally, the USH2 quaternary complex
has a variable stoichiometry. These findings suggest that a non-obligate, short
term, and dynamic USH2 quaternary protein complex may exist in vivo. Our work
provides valuable insight into the physiological role of the USH2 complex in vivo
and informs possible reconstruction of the USH2 complex for future therapy.