DICER1 Chromosome 14

Dicer 1, ribonuclease III

929 variants 929 Health Risk

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What This Gene Does

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. This protein also acts as a strong antiviral agent with activity against RNA viruses, including the Zika and SARS-CoV-2 viruses. Alternative splicing results in multiple transcript variants. [provided by RefSeq, Jul 2021]

Gene Info

Gene Group

"Endoribonucleases|MicroRNA protein coding host genes|RISC loading complex subunits"

Locus Type

gene with protein product

Location

14q32.13

Ensembl

ENSG00000100697

Associated Conditions (37)

DICER1-related tumor predisposition

Hereditary cancer-predisposing syndrome

Pleuropulmonary blastoma

Euthyroid goiter

Global developmental delay - lung cysts - overgrowth - Wilms tumor syndrome

Rhabdomyosarcoma

embryonal

Ovarian cancer

DICER1-related disorder

Breast neoplasm

Primary intracranial sarcoma

DICER1-mutant

Sertoli-Leydig cell tumor

Follicular thyroid carcinoma

Papillary thyroid carcinoma

Cancer or benign tumor

Sarcoma

Pediatric high-grade glioma

Ovarian serous cystadenocarcinoma

+17 more conditions

Key Variants

RS1024226278

Conflicting classifications of pathogenicity

Health Risk

RS1032236740

Conflicting classifications of pathogenicity

Health Risk

RS1045097916

Conflicting classifications of pathogenicity

Hereditary cancer-predisposing syndrome, DICER1-related tumor predisposition, Hereditary cancer-predisposing syndrome

Health Risk

RS1047347414

Conflicting classifications of pathogenicity

Health Risk

RS1060503601

Conflicting classifications of pathogenicity

Health Risk

RS1060503614

Conflicting classifications of pathogenicity

Health Risk

RS1060503616

Conflicting classifications of pathogenicity

Health Risk

RS1060503631

Conflicting classifications of pathogenicity

Health Risk

RS1060503632

Conflicting classifications of pathogenicity

Health Risk

RS1060503647

Conflicting classifications of pathogenicity

Health Risk

RS1060503649

Conflicting classifications of pathogenicity

Health Risk

RS1060503652

Conflicting classifications of pathogenicity

Health Risk

All Variants (929)

RSID	Category	Clinical Significance	Conditions
RS766598800	Health Risk	Conflicting classifications of pathogenicity	DICER1-related tumor predisposition, Hereditary cancer-predisposing syndrome, DICER1-related tumor predisposition
RS767605412	Health Risk	Conflicting classifications of pathogenicity	DICER1-related tumor predisposition, Hereditary cancer-predisposing syndrome, DICER1-related tumor predisposition
RS767907056	Health Risk	Conflicting classifications of pathogenicity	DICER1-related tumor predisposition, Hereditary cancer-predisposing syndrome, DICER1-related tumor predisposition
RS768311617	Health Risk	Conflicting classifications of pathogenicity	DICER1-related tumor predisposition, Hereditary cancer-predisposing syndrome, DICER1-related tumor predisposition
RS768445477	Health Risk	Conflicting classifications of pathogenicity	DICER1-related tumor predisposition, Hereditary cancer-predisposing syndrome, Global developmental delay - lung cysts - overgrowth - Wilms tumor syndrome
RS768820364	Health Risk	Conflicting classifications of pathogenicity	DICER1-related tumor predisposition, Global developmental delay - lung cysts - overgrowth - Wilms tumor syndrome, Hereditary cancer-predisposing syndrome
RS769292296	Health Risk	Conflicting classifications of pathogenicity	DICER1-related tumor predisposition, Hereditary cancer-predisposing syndrome, DICER1-related tumor predisposition
RS769549511	Health Risk	Conflicting classifications of pathogenicity	DICER1-related tumor predisposition, Hereditary cancer-predisposing syndrome, DICER1-related tumor predisposition
RS771049445	Health Risk	Conflicting classifications of pathogenicity	Hereditary cancer-predisposing syndrome, DICER1-related tumor predisposition, Hereditary cancer-predisposing syndrome
RS772431718	Health Risk	Conflicting classifications of pathogenicity	DICER1-related tumor predisposition, Hereditary cancer-predisposing syndrome, DICER1-related tumor predisposition
RS773410751	Health Risk	Conflicting classifications of pathogenicity	DICER1-related tumor predisposition, Hereditary cancer-predisposing syndrome, DICER1-related tumor predisposition
RS773875981	Health Risk	Conflicting classifications of pathogenicity	DICER1-related tumor predisposition, Hereditary cancer-predisposing syndrome, DICER1-related tumor predisposition
RS774024734	Health Risk	Conflicting classifications of pathogenicity	DICER1-related tumor predisposition, DICER1-related tumor predisposition
RS774583162	Health Risk	Conflicting classifications of pathogenicity	DICER1-related tumor predisposition, Hereditary cancer-predisposing syndrome, DICER1-related tumor predisposition
RS774703353	Health Risk	Conflicting classifications of pathogenicity	DICER1-related tumor predisposition, Hereditary cancer-predisposing syndrome, DICER1-related tumor predisposition
RS774727760	Health Risk	Conflicting classifications of pathogenicity	DICER1-related tumor predisposition, Hereditary cancer-predisposing syndrome, DICER1-related tumor predisposition
RS774765473	Health Risk	Conflicting classifications of pathogenicity	DICER1-related tumor predisposition, Hereditary cancer-predisposing syndrome, DICER1-related tumor predisposition
RS774875481	Health Risk	Conflicting classifications of pathogenicity	DICER1-related tumor predisposition, Hereditary cancer-predisposing syndrome, DICER1-related tumor predisposition
RS775828379	Health Risk	Conflicting classifications of pathogenicity	DICER1-related tumor predisposition, Hereditary cancer-predisposing syndrome, Global developmental delay - lung cysts - overgrowth - Wilms tumor syndrome
RS776023608	Health Risk	Conflicting classifications of pathogenicity	DICER1-related tumor predisposition, Hereditary cancer-predisposing syndrome, DICER1-related tumor predisposition
RS776219930	Health Risk	Conflicting classifications of pathogenicity	DICER1-related tumor predisposition, Hereditary cancer-predisposing syndrome, Global developmental delay - lung cysts - overgrowth - Wilms tumor syndrome
RS777737447	Health Risk	Conflicting classifications of pathogenicity	DICER1-related tumor predisposition, Hereditary cancer-predisposing syndrome, DICER1-related tumor predisposition
RS777823741	Health Risk	Conflicting classifications of pathogenicity	DICER1-related tumor predisposition, Hereditary cancer-predisposing syndrome, DICER1-related tumor predisposition
RS777894117	Health Risk	Conflicting classifications of pathogenicity	DICER1-related tumor predisposition, Hereditary cancer-predisposing syndrome, Global developmental delay - lung cysts - overgrowth - Wilms tumor syndrome
RS778494781	Health Risk	Conflicting classifications of pathogenicity	DICER1-related tumor predisposition, Hereditary cancer-predisposing syndrome, DICER1-related tumor predisposition
RS778669119	Health Risk	Conflicting classifications of pathogenicity	DICER1-related tumor predisposition, DICER1-related tumor predisposition
RS779062746	Health Risk	Conflicting classifications of pathogenicity	Hereditary cancer-predisposing syndrome, DICER1-related tumor predisposition, Global developmental delay - lung cysts - overgrowth - Wilms tumor syndrome
RS779443466	Health Risk	Conflicting classifications of pathogenicity	Hereditary cancer-predisposing syndrome, DICER1-related tumor predisposition, Hereditary cancer-predisposing syndrome
RS780084290	Health Risk	Conflicting classifications of pathogenicity	DICER1-related tumor predisposition, Hereditary cancer-predisposing syndrome, DICER1-related tumor predisposition
RS780545199	Health Risk	Conflicting classifications of pathogenicity	DICER1-related tumor predisposition, Hereditary cancer-predisposing syndrome, Global developmental delay - lung cysts - overgrowth - Wilms tumor syndrome
RS780815020	Health Risk	Conflicting classifications of pathogenicity	DICER1-related tumor predisposition, Hereditary cancer-predisposing syndrome, Pleuropulmonary blastoma
RS866993296	Health Risk	Conflicting classifications of pathogenicity	DICER1-related tumor predisposition, Hereditary cancer-predisposing syndrome, Hereditary cancer-predisposing syndrome
RS878855248	Health Risk	Conflicting classifications of pathogenicity	DICER1-related tumor predisposition, Hereditary cancer-predisposing syndrome, DICER1-related disorder
RS878855250	Health Risk	Conflicting classifications of pathogenicity	DICER1-related tumor predisposition, Hereditary cancer-predisposing syndrome, DICER1-related tumor predisposition
RS878855267	Health Risk	Conflicting classifications of pathogenicity	DICER1-related tumor predisposition, Hereditary cancer-predisposing syndrome, DICER1-related tumor predisposition
RS878855269	Health Risk	Conflicting classifications of pathogenicity	DICER1-related tumor predisposition, Hereditary cancer-predisposing syndrome, DICER1-related tumor predisposition
RS886050940	Health Risk	Conflicting classifications of pathogenicity	DICER1-related tumor predisposition, Hereditary cancer-predisposing syndrome, DICER1-related tumor predisposition
RS886050943	Health Risk	Conflicting classifications of pathogenicity	DICER1-related tumor predisposition, Hereditary cancer-predisposing syndrome, DICER1-related tumor predisposition
RS886050944	Health Risk	Conflicting classifications of pathogenicity	DICER1-related tumor predisposition, Hereditary cancer-predisposing syndrome, DICER1-related tumor predisposition
RS890402869	Health Risk	Conflicting classifications of pathogenicity	DICER1-related tumor predisposition, Hereditary cancer-predisposing syndrome, DICER1-related tumor predisposition
RS894664317	Health Risk	Conflicting classifications of pathogenicity	DICER1-related tumor predisposition, Hereditary cancer-predisposing syndrome, DICER1-related disorder
RS921899291	Health Risk	Conflicting classifications of pathogenicity	DICER1-related tumor predisposition, Hereditary cancer-predisposing syndrome, DICER1-related tumor predisposition
RS953855540	Health Risk	Conflicting classifications of pathogenicity	DICER1-related tumor predisposition, Hereditary cancer-predisposing syndrome, DICER1-related tumor predisposition
RS981303167	Health Risk	Conflicting classifications of pathogenicity	DICER1-related tumor predisposition, DICER1-related tumor predisposition
RS982548109	Health Risk	Conflicting classifications of pathogenicity	DICER1-related tumor predisposition, Hereditary cancer-predisposing syndrome, DICER1-related tumor predisposition
RS984076701	Health Risk	Conflicting classifications of pathogenicity	DICER1-related tumor predisposition, Hereditary cancer-predisposing syndrome, DICER1-related tumor predisposition
RS1131691204	Health Risk	Likely pathogenic	Hereditary cancer-predisposing syndrome, Hereditary cancer-predisposing syndrome
RS1131691227	Health Risk	Likely pathogenic	Hereditary cancer-predisposing syndrome, DICER1-related tumor predisposition, DICER1-related tumor predisposition
RS1232103009	Health Risk	Likely pathogenic	Hereditary cancer-predisposing syndrome, Hereditary cancer-predisposing syndrome
RS1357513890	Health Risk	Likely pathogenic	—

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