DGCR8

DGCR8 microprocessor complex subunit
NMR structure of the double-stranded RNA binding motif (DSRM) domain of human DGCR8 (PDB: 1X47​).
Available structures PDB Ortholog search: PDBe, RCSB List of PDB id codes 1X47​, 2YT4​, 3LE4​
Identifiers
Symbols	DGCR8 ; C22orf12; DGCRK6; Gy1; pasha
External IDs	OMIM: 609030 MGI: 2151114 HomoloGene: 11223 GeneCards: DGCR8 Gene
Gene ontology Molecular function • double-stranded RNA binding • ribonuclease III activity • protein binding • heme binding • protein homodimerization activity • metal ion binding • primary miRNA binding Cellular component • nucleus • nucleoplasm • nucleolus • cytoplasm • microtubule cytoskeleton • microprocessor complex Biological process • gene expression • primary miRNA processing • regulation of stem cell proliferation • RNA phosphodiester bond hydrolysis • RNA phosphodiester bond hydrolysis, endonucleolytic Sources: Amigo / QuickGO
RNA expression pattern
More reference expression data
Orthologs
Species	Human	Mouse
Entrez	54487	94223
Ensembl	ENSG00000128191	ENSMUSG00000022718
UniProt	Q8WYQ5	Q9EQM6
RefSeq (mRNA)	NM_001190326	NM_033324
RefSeq (protein)	NP_001177255	NP_201581
Location (UCSC)	Chr 22: 20.08 – 20.11 Mb	Chr 16: 18.25 – 18.29 Mb
PubMed search

The DGCR8 microprocessor complex subunit (DiGeorge syndrome chromosomal [or critical] region 8) is a protein that in humans is encoded by the DGCR8 gene.^[1] In other animals, particularly the common model organisms Drosophila melanogaster and Caenorhabditis elegans, the protein is known as Pasha (partner of Drosha).^[2] It is a required component of the RNA interference pathway.

Function

DGCR8 is localized to the cell nucleus and is required for microRNA (miRNA) processing. It binds to Drosha, an RNase III enzyme, to form the Microprocessor complex that cleaves a primary transcript known as pri-miRNA to a characteristic stem-loop structure known as a pre-miRNA, which is then further processed to miRNA fragments by the enzyme Dicer. DGCR8 contains an RNA-binding domain and is thought to bind pri-miRNA to stabilize it for processing by Drosha.^[3]

References

1. "Entrez Gene: DGCR8 DiGeorge syndrome critical region gene 8".
2. Denli AM, Tops BB, Plasterk RH, Ketting RF, Hannon GJ (Nov 2004). "Processing of primary microRNAs by the Microprocessor complex". Nature 432 (7014): 231–5. doi:10.1038/nature03049. PMID 15531879.
3. Yeom KH, Lee Y, Han J, Suh MR, Kim VN (2006). "Characterization of DGCR8/Pasha, the essential cofactor for Drosha in primary miRNA processing". Nucleic Acids Research 34 (16): 4622–9. doi:10.1093/nar/gkl458. PMC 1636349. PMID 16963499.

Further reading

• Simpson JC, Wellenreuther R, Poustka A, Pepperkok R, Wiemann S (Sep 2000). "Systematic subcellular localization of novel proteins identified by large-scale cDNA sequencing". EMBO Reports 1 (3): 287–92. doi:10.1093/embo-reports/kvd058. PMC 1083732. PMID 11256614.
• Shiohama A, Sasaki T, Noda S, Minoshima S, Shimizu N (Apr 2003). "Molecular cloning and expression analysis of a novel gene DGCR8 located in the DiGeorge syndrome chromosomal region". Biochemical and Biophysical Research Communications 304 (1): 184–90. doi:10.1016/S0006-291X(03)00554-0. PMID 12705904.
• Gregory RI, Yan KP, Amuthan G, Chendrimada T, Doratotaj B, Cooch N, Shiekhattar R (Nov 2004). "The Microprocessor complex mediates the genesis of microRNAs". Nature 432 (7014): 235–40. doi:10.1038/nature03120. PMID 15531877.
• Han J, Lee Y, Yeom KH, Kim YK, Jin H, Kim VN (Dec 2004). "The Drosha-DGCR8 complex in primary microRNA processing". Genes & Development 18 (24): 3016–27. doi:10.1101/gad.1262504. PMC 535913. PMID 15574589.
• Landthaler M, Yalcin A, Tuschl T (Dec 2004). "The human DiGeorge syndrome critical region gene 8 and Its D. melanogaster homolog are required for miRNA biogenesis". Current Biology 14 (23): 2162–7. doi:10.1016/j.cub.2004.11.001. PMID 15589161.
• Han J, Lee Y, Yeom KH, Nam JW, Heo I, Rhee JK, Sohn SY, Cho Y, Zhang BT, Kim VN (Jun 2006). "Molecular basis for the recognition of primary microRNAs by the Drosha-DGCR8 complex". Cell 125 (5): 887–901. doi:10.1016/j.cell.2006.03.043. PMID 16751099.
• Faller M, Matsunaga M, Yin S, Loo JA, Guo F (Jan 2007). "Heme is involved in microRNA processing". Nature Structural & Molecular Biology 14 (1): 23–9. doi:10.1038/nsmb1182. PMID 17159994.
• Sohn SY, Bae WJ, Kim JJ, Yeom KH, Kim VN, Cho Y (Sep 2007). "Crystal structure of human DGCR8 core". Nature Structural & Molecular Biology 14 (9): 847–53. doi:10.1038/nsmb1294. PMID 17704815.