DICER1 Chromosome 14

Dicer 1, ribonuclease III

929 variants 929 Health Risk

Upload your DNA to see your personal genotypes for variants in DICER1.

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
RS2139815270	Health Risk	Likely pathogenic	Ovarian cancer, Ovarian cancer
RS2139815576	Health Risk	Likely pathogenic	Euthyroid goiter, DICER1-related tumor predisposition, Euthyroid goiter
RS2139815940	Health Risk	Likely pathogenic	DICER1-related tumor predisposition, DICER1-related tumor predisposition
RS2139855793	Health Risk	Likely pathogenic	DICER1-related tumor predisposition, DICER1-related tumor predisposition
RS2139900517	Health Risk	Likely pathogenic	DICER1-related tumor predisposition, DICER1-related tumor predisposition
RS2139994024	Health Risk	Likely pathogenic	DICER1-related tumor predisposition, DICER1-related tumor predisposition
RS2139994161	Health Risk	Likely pathogenic	DICER1-related tumor predisposition, Euthyroid goiter, Rhabdomyosarcoma
RS2140001131	Health Risk	Likely pathogenic	DICER1-related tumor predisposition, DICER1-related tumor predisposition
RS2140066644	Health Risk	Likely pathogenic	DICER1-related tumor predisposition, DICER1-related tumor predisposition
RS2140122536	Health Risk	Likely pathogenic	DICER1-related tumor predisposition, DICER1-related tumor predisposition
RS2140138974	Health Risk	Likely pathogenic	DICER1-related tumor predisposition, DICER1-related tumor predisposition
RS2140259561	Health Risk	Likely pathogenic	DICER1-related tumor predisposition, DICER1-related tumor predisposition
RS2140261649	Health Risk	Likely pathogenic	DICER1-related tumor predisposition, DICER1-related tumor predisposition
RS2140295068	Health Risk	Likely pathogenic	DICER1-related tumor predisposition, DICER1-related tumor predisposition
RS2542403356	Health Risk	Likely pathogenic	DICER1-related tumor predisposition, DICER1-related tumor predisposition
RS2542404363	Health Risk	Likely pathogenic	Pleuropulmonary blastoma, Pleuropulmonary blastoma
RS2542478659	Health Risk	Likely pathogenic	Global developmental delay - lung cysts - overgrowth - Wilms tumor syndrome, Global developmental delay - lung cysts - overgrowth - Wilms tumor syndrome
RS2542480965	Health Risk	Likely pathogenic	DICER1-related tumor predisposition, DICER1-related tumor predisposition
RS2542483570	Health Risk	Likely pathogenic	Pleuropulmonary blastoma, Euthyroid goiter, Pleuropulmonary blastoma
RS2542484985	Health Risk	Likely pathogenic	Global developmental delay - lung cysts - overgrowth - Wilms tumor syndrome, Global developmental delay - lung cysts - overgrowth - Wilms tumor syndrome
RS2542600568	Health Risk	Likely pathogenic	DICER1-related tumor predisposition, DICER1-related tumor predisposition
RS2542721174	Health Risk	Likely pathogenic	Global developmental delay - lung cysts - overgrowth - Wilms tumor syndrome, Global developmental delay - lung cysts - overgrowth - Wilms tumor syndrome
RS2542724105	Health Risk	Likely pathogenic	DICER1-related tumor predisposition, DICER1-related tumor predisposition
RS2542772141	Health Risk	Likely pathogenic	Global developmental delay - lung cysts - overgrowth - Wilms tumor syndrome, Global developmental delay - lung cysts - overgrowth - Wilms tumor syndrome
RS2542772237	Health Risk	Likely pathogenic	Hereditary cancer-predisposing syndrome, Hereditary cancer-predisposing syndrome
RS2542851457	Health Risk	Likely pathogenic	Hereditary cancer-predisposing syndrome, Pleuropulmonary blastoma, Rhabdomyosarcoma
RS2542932578	Health Risk	Likely pathogenic	Hereditary cancer-predisposing syndrome, Hereditary cancer-predisposing syndrome
RS2542952666	Health Risk	Likely pathogenic	Global developmental delay - lung cysts - overgrowth - Wilms tumor syndrome, Global developmental delay - lung cysts - overgrowth - Wilms tumor syndrome
RS2543019173	Health Risk	Likely pathogenic	DICER1-related tumor predisposition, DICER1-related tumor predisposition
RS2543031258	Health Risk	Likely pathogenic	Global developmental delay - lung cysts - overgrowth - Wilms tumor syndrome, Global developmental delay - lung cysts - overgrowth - Wilms tumor syndrome
RS2543038200	Health Risk	Likely pathogenic	DICER1-related tumor predisposition, DICER1-related tumor predisposition
RS2543064634	Health Risk	Likely pathogenic	Global developmental delay - lung cysts - overgrowth - Wilms tumor syndrome, Hereditary cancer-predisposing syndrome, Global developmental delay - lung cysts - overgrowth - Wilms tumor syndrome
RS2543178455	Health Risk	Likely pathogenic	Global developmental delay - lung cysts - overgrowth - Wilms tumor syndrome, Global developmental delay - lung cysts - overgrowth - Wilms tumor syndrome
RS2543180436	Health Risk	Likely pathogenic	Global developmental delay - lung cysts - overgrowth - Wilms tumor syndrome, Global developmental delay - lung cysts - overgrowth - Wilms tumor syndrome
RS2543188305	Health Risk	Likely pathogenic	Pleuropulmonary blastoma, Pleuropulmonary blastoma
RS2543280292	Health Risk	Likely pathogenic	DICER1-related tumor predisposition, DICER1-related tumor predisposition
RS2543344129	Health Risk	Likely pathogenic	DICER1-related tumor predisposition, DICER1-related tumor predisposition
RS2543373409	Health Risk	Likely pathogenic	DICER1-related tumor predisposition, DICER1-related tumor predisposition
RS387906934	Health Risk	Likely pathogenic	Euthyroid goiter, DICER1-related tumor predisposition, Hereditary cancer-predisposing syndrome
RS751053813	Health Risk	Likely pathogenic	—
RS754838493	Health Risk	Likely pathogenic	Pleuropulmonary blastoma, DICER1-related tumor predisposition, Pleuropulmonary blastoma
RS765059994	Health Risk	Likely pathogenic	DICER1-related tumor predisposition, Hereditary cancer-predisposing syndrome, DICER1-related tumor predisposition
RS875989783	Health Risk	Likely pathogenic	Pineoblastoma, Hereditary cancer-predisposing syndrome, DICER1-related tumor predisposition
RS886037722	Health Risk	Likely pathogenic	DICER1-related tumor predisposition, DICER1-related tumor predisposition, DICER1-related tumor predisposition
RS886037727	Health Risk	Likely pathogenic	DICER1-related tumor predisposition, Hereditary cancer-predisposing syndrome, DICER1-related tumor predisposition
RS886037729	Health Risk	Likely pathogenic	DICER1-related tumor predisposition, Hereditary cancer-predisposing syndrome, DICER1-related tumor predisposition
RS1060503583	Health Risk	Pathogenic	DICER1-related tumor predisposition, DICER1-related tumor predisposition
RS1060503596	Health Risk	Pathogenic	DICER1-related tumor predisposition, Hereditary cancer-predisposing syndrome, DICER1-related tumor predisposition
RS1060503605	Health Risk	Pathogenic	DICER1-related tumor predisposition, Pleuropulmonary blastoma, Hereditary cancer-predisposing syndrome
RS1060503617	Health Risk	Pathogenic	DICER1-related tumor predisposition, DICER1-related tumor predisposition

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