1a12
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REGULATOR OF CHROMOSOME CONDENSATION (RCC1) OF HUMAN
OverviewOverview
The gene encoding the regulator of chromosome condensation (RCC1) was, cloned by virtue of its ability to complement the temperature-sensitive, phenotype of the hamster cell line tsBN2, which undergoes premature, chromosome condensation or arrest in the G1 phase of the cell cycle at, non-permissive temperatures. RCC1 homologues have been identified in many, eukaryotes, including budding and fission yeast. Mutations in the gene, affect pre-messenger RNA processing and transport, mating, initiation of, mitosis and chromatin decondensation, suggesting that RCC1 is important in, the control of nucleo-cytoplasmic transport and the cell cycle., Biochemically, RCC1 is a guanine-nucleotide-exchange factor for the, nuclear Ras homologue Ran; it increases the dissociation of Ran-bound GDP, by 10(5)-fold. It may also bind to DNAvia a protein-protein complex. Here, we show that the structure of human RCC1, solved to 1.7-A resolution by, X-ray crystallography, consists of a seven-bladed propeller formed from, internal repeats of 51-68 residues per blade. The sequence and structure, of the repeats differ from those of WD40-domain proteins, which also form, seven-bladed propellers and include the beta-subunits of G proteins. The, nature of the structure explains the consequences of a wide range of known, mutations. The region of the protein that is involved in, guanine-nucleotide exchange is located opposite the region that is thought, to be involved in chromosome binding.
DiseaseDisease
Known disease associated with this structure: Alveolar soft-part sarcoma OMIM:[606236]
About this StructureAbout this Structure
1A12 is a Single protein structure of sequence from Homo sapiens. Full crystallographic information is available from OCA.
ReferenceReference
The 1.7 A crystal structure of the regulator of chromosome condensation (RCC1) reveals a seven-bladed propeller., Renault L, Nassar N, Vetter I, Becker J, Klebe C, Roth M, Wittinghofer A, Nature. 1998 Mar 5;392(6671):97-101. PMID:9510255
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