| Post Translational Modifications | Phosphorylated at Ser-677 by ATM upon radiation, which is crucial for its stability. Ubiquitinated, leading to degradation in a proteasome-dependent manner. Deubiquitinated by USP51, leading to stabilization. |
| Function | Component of the microprocessor complex that acts as a RNA- and heme-binding protein that is involved in the initial step of microRNA (miRNA) biogenesis. Component of the microprocessor complex that is required to process primary miRNA transcripts (pri-miRNAs) to release precursor miRNA (pre-miRNA) in the nucleus. Within the microprocessor complex, DGCR8 function as a molecular anchor necessary for the recognition of pri-miRNA at dsRNA-ssRNA junction and directs DROSHA to cleave 11 bp away form the junction to release hairpin-shaped pre-miRNAs that are subsequently cut by the cytoplasmic DICER to generate mature miRNAs. The heme-bound DGCR8 dimer binds pri-miRNAs as a cooperative trimer (of dimers) and is active in triggering pri-miRNA cleavage, whereas the heme-free DGCR8 monomer binds pri-miRNAs as a dimer and is much less active. Both double-stranded and single-stranded regions of a pri-miRNA are required for its binding. Specifically recognizes and binds N6-methyladenosine (m6A)-containing pri-miRNAs, a modification required for pri-miRNAs processing. Involved in the silencing of embryonic stem cell self-renewal. Plays also a role in DNA repair by promoting the recruitment of RNF168 to RNF8 and MDC1 at DNA double-strand breaks and subsequently the clearance of DNA breaks. |
| Protein Name | Microprocessor Complex Subunit Dgcr8Digeorge Syndrome Critical Region 8 |
| Database Links | Reactome: R-HSA-203927Reactome: R-HSA-8986944 |
| Cellular Localisation | NucleusNucleolusColocalizes With Nucleolin And Drosha In The NucleolusMostly Detected In The Nucleolus As Electron-Dense Granular Patches Around The Fibrillar Center (Fc) And Granular Component (Gc)Also Detected In The Nucleoplasm As Small Foci Adjacent To Splicing Speckles Near The Chromatin StructureLocalized With Drosha In Gw Bodies (Gwbs)Also Known As P-Bodies |
| Alternative Protein Names | Microprocessor Complex Subunit Dgcr8 proteinDigeorge Syndrome Critical Region 8 proteinDGCR8 proteinC22orf12 proteinDGCRK6 proteinLP4941 protein |
Information sourced from Uniprot.org
