AAI50288.1
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UniProt Primary Accession #
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UniProt Secondary Accession #
UniProt Related Accession #
NCBI Official Full Name
Dicer 1, ribonuclease type III
NCBI Official Synonym Full Names
dicer 1, ribonuclease type III
NCBI Protein Information
endoribonuclease Dicer; K12H4.8-LIKE; helicase MOI; helicase-moi; Dicer1, Dcr-1 homolog; helicase with RNAse motif; dicer 1, double-stranded RNA-specific endoribonuclease
UniProt Protein Name
Endoribonuclease Dicer
UniProt Synonym Protein Names
Helicase with RNase motif
UniProt Synonym Gene Names
UniProt Entry Name
DICER_HUMAN
NCBI Summary for DICER1
This gene encodes a protein possessing an RNA helicase motif containing a DEXH box in its amino terminus and an RNA motif in the carboxy terminus. The encoded protein functions as a ribonuclease and is required by the RNA interference and small temporal RNA (stRNA) pathways to produce the active small RNA component that represses gene expression. Alternative splicing results in multiple transcript variants. [provided by RefSeq, Sep 2010]
UniProt Comments for DICER1
Function: Required for formation of the RNA induced silencing complex (RISC). Component of the RISC loading complex (RLC), also known as the micro-RNA (miRNA) loading complex (miRLC), which is composed of DICER1, AGO2 and TARBP2. Within the RLC/miRLC, DICER1 and TARBP2 are required to process precursor miRNAs (pre-miRNAs) to mature miRNAs and then load them onto AGO2. AGO2 bound to the mature miRNA constitutes the minimal RISC and may subsequently dissociate from DICER1 and TARBP2. Also cleaves double-stranded RNA to produce short interfering RNAs (siRNAs) which target the selective destruction of complementary RNAs. Ref.10 Ref.12 Ref.13 Ref.14 Ref.15 Ref.16 Ref.17 Ref.18 Ref.23 Ref.25
Cofactor: Binds 2 magnesium or manganese ions per subunit
Probable. Ref.31
Subunit structure: Component of the RISC loading complex (RLC), or micro-RNA (miRNA) loading complex (miRLC), which is composed of DICER1, AGO2 and TARBP2. Note that the trimeric RLC/miRLC is also referred to as RISC. Interacts with DHX9, AGO1, PIWIL1 and PRKRA. Associates with the 60S ribosome. Interacts with BCDIN3D. Ref.11 Ref.12 Ref.13 Ref.14 Ref.15 Ref.16 Ref.17 Ref.18 Ref.19 Ref.20 Ref.21 Ref.23 Ref.29 Ref.31
Subcellular location: Cytoplasm Ref.17.
Involvement in disease: Pleuropulmonary blastoma (PPB) [MIM:601200]: A rare pediatric intrathoracic neoplasm. The tumor arises from the lung, pleura, or both, and appears to be purely mesenchymal in phenotype. It lacks malignant epithelial elements, a feature that distinguishes it from the classic adult-type pulmonary blastoma. It arises during fetal lung development and is often part of an inherited cancer syndrome. The tumor contain both epithelial and mesenchymal cells. Early in tumorigenesis, cysts form in lung airspaces, and these cysts are lined with benign-appearing epithelium. Mesenchymal cells susceptible to malignant transformation reside within the cyst walls and form a dense layer beneath the epithelial lining. In a subset of patients, overgrowth of the mesenchymal cells produces a sarcoma, a transition that is associated with a poorer prognosis. Some patients have multilocular cystic nephroma, a benign kidney tumor.Note: The disease is caused by mutations affecting the gene represented in this entry. Ref.27 Ref.32Goiter multinodular 1, with or without Sertoli-Leydig cell tumors (MNG1) [MIM:138800]: A common disorder characterized by nodular overgrowth of the thyroid gland. Some individuals may also develop Sertoli-Leydig cell tumors, usually of the ovary.Note: The disease is caused by mutations affecting the gene represented in this entry. Ref.27 Ref.33DICER1 mutations have been found in uterine cervix embryonal rhabdomyosarcoma, primitive neuroectodermal tumor, Wilms tumor, pulmonary sequestration and juvenile intestinal polyp (Ref.27). Somatic missense mutations affecting the RNase IIIb domain of DICER1 are common in non-epithelial ovarian tumors. These mutations do not abolish DICER1 function but alter it in specific cell types, a novel mechanism through which perturbation of microRNA processing may be oncogenic (Ref.30). Ref.27
Sequence similarities: Belongs to the helicase family. Dicer subfamily.Contains 1 Dicer dsRNA-binding fold domain.Contains 1 DRBM (double-stranded RNA-binding) domain.Contains 1 helicase ATP-binding domain.Contains 1 helicase C-terminal domain.Contains 1 PAZ domain.Contains 2 RNase III domains.
Caution: It is uncertain whether Met-1 or Met-11 is the initiator.
Sequence caution: The sequence CAB38857.2 differs from that shown. Reason: Erroneous initiation. Translation N-terminally extended.
Research Articles on DICER1
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Products associated with DICER1 enzyme
Pathways associated with DICER1 enzyme
Diseases associated with DICER1 enzyme
Organs/Tissues associated with DICER1 enzyme
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