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FBXW11A gene on chromosome 5q35.1 that encodes a so-called F-box protein, which contains a 40 amino acid protein-interacting motif (the F box). F-box proteins form a complex with SKP1 and cullin, resulting in phosphorylation-dependent ubiquitination and ubiquitin-mediated degradation of targeted proteins. F-box proteins are divided into three classes based on the presence of:
(1) WD-40 repeats—as is the case with this F-box protein (Fbws class);
(2) leucine-rich repeats (Fbls class); or
(3) the presence or absence of other protein-protein-interacting domains (Fbxs class).
FBXW11 has been linked to pseudotrisomy 13 syndrome.