Article
Bardet-Biedl Syndrome ciliopathy is linked to altered hematopoiesis and dysregulated self-tolerance.
EMBO reports - 3 Feb 2021
Tsyklauri Oksana, Niederlova Veronika, Forsythe Elizabeth, Prasai Avishek, Drobek Ales, Kasparek Petr, Sparks Kathryn, Trachtulec Zdenek, Prochazka Jan, Sedlacek Radislav, Beales Philip, Huranova Martina, Stepanek Ondrej
Abstract excerpt
Bardet-Biedl Syndrome (BBS) is a pleiotropic genetic disease caused by the dysfunction of primary cilia. The immune system of patients with ciliopathies has not been investigated. However, there are multiple indications that the impairment of the processes typically associated with cilia may have influence on the hematopoietic compartment and immunity. In this study, we analyze clinical data of BBS patients and...
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