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|3v42, resolution 2.00Å ()|
|Gene:||BHD, FLCN (Homo sapiens)|
Crystal structure of renal tumor suppressor protein, folliculin
Mutations in the renal tumour suppressor protein, folliculin, lead to proliferative skin lesions, lung complications and renal cell carcinoma. Folliculin has been reported to interact with AMP-activated kinase, a key component of the mammalian target of rapamycin pathway. Most cancer-causing mutations lead to a carboxy-terminal truncation of folliculin, pointing to a functional importance of this domain in tumour suppression. We present here the crystal structure of folliculin carboxy-terminal domain and demonstrate that it is distantly related to differentially expressed in normal cells and neoplasia (DENN) domain proteins, a family of Rab guanine nucleotide exchange factors (GEFs). Using biochemical analysis, we show that folliculin has GEF activity, indicating that folliculin is probably a distantly related member of this class of Rab GEFs.
Crystal structure of folliculin reveals a hidDENN function in genetically inherited renal cancer., Nookala RK, Langemeyer L, Pacitto A, Ochoa-Montano B, Donaldson JC, Blaszczyk BK, Chirgadze DY, Barr FA, Bazan JF, Blundell TL, Open Biol. 2012 Aug;2(8):120071. PMID:22977732
From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine.