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2017 ; 7
(1
): 9765
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gab.com Text
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English Wikipedia
BBS4 regulates the expression and secretion of FSTL1, a protein that participates
in ciliogenesis and the differentiation of 3T3-L1
#MMPMID28852127
Prieto-Echagüe V
; Lodh S
; Colman L
; Bobba N
; Santos L
; Katsanis N
; Escande C
; Zaghloul NA
; Badano JL
Sci Rep
2017[Aug]; 7
(1
): 9765
PMID28852127
show ga
Bardet-Biedl syndrome is a model ciliopathy. Although the characterization of BBS
proteins has evidenced their involvement in cilia, extraciliary functions for
some of these proteins are also being recognized. Importantly, understanding both
cilia and cilia-independent functions of the BBS proteins is key to fully dissect
the cellular basis of the syndrome. Here we characterize a functional interaction
between BBS4 and the secreted protein FSTL1, a protein linked to adipogenesis and
inflammation among other functions. We show that BBS4 and cilia regulate FSTL1
mRNA levels, but BBS4 also modulates FSTL1 secretion. Moreover, we show that
FSTL1 is a novel regulator of ciliogenesis thus underscoring a regulatory loop
between FSTL1 and cilia. Finally, our data indicate that BBS4, cilia and FSTL1
are coordinated during the differentiation of 3T3-L1 cells and that FSTL1 plays a
role in this process, at least in part, by modulating ciliogenesis. Therefore,
our findings are relevant to fully understand the development of BBS-associated
phenotypes such as obesity.