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
RS2140295151	Health Risk	Conflicting classifications of pathogenicity	DICER1-related tumor predisposition, Hereditary cancer-predisposing syndrome, DICER1-related tumor predisposition
RS2275182	Health Risk	Conflicting classifications of pathogenicity	DICER1-related tumor predisposition, DICER1-related tumor predisposition
RS2542494913	Health Risk	Conflicting classifications of pathogenicity	Hereditary cancer-predisposing syndrome, DICER1-related tumor predisposition, Hereditary cancer-predisposing syndrome
RS2542597073	Health Risk	Conflicting classifications of pathogenicity	Hereditary cancer-predisposing syndrome, DICER1-related tumor predisposition, Hereditary cancer-predisposing syndrome
RS2542830280	Health Risk	Conflicting classifications of pathogenicity	Hereditary cancer-predisposing syndrome, Hereditary cancer-predisposing syndrome
RS2543001376	Health Risk	Conflicting classifications of pathogenicity	DICER1-related tumor predisposition, Hereditary cancer-predisposing syndrome, DICER1-related tumor predisposition
RS2543005176	Health Risk	Conflicting classifications of pathogenicity	Hereditary cancer-predisposing syndrome, DICER1-related tumor predisposition, Hereditary cancer-predisposing syndrome
RS2543281741	Health Risk	Conflicting classifications of pathogenicity	Hereditary cancer-predisposing syndrome, Hereditary cancer-predisposing syndrome
RS2543285003	Health Risk	Conflicting classifications of pathogenicity	Hereditary cancer-predisposing syndrome, DICER1-related tumor predisposition, Hereditary cancer-predisposing syndrome
RS2543365910	Health Risk	Conflicting classifications of pathogenicity	Hereditary cancer-predisposing syndrome, DICER1-related tumor predisposition, Hereditary cancer-predisposing syndrome
RS368343829	Health Risk	Conflicting classifications of pathogenicity	DICER1-related tumor predisposition, Hereditary cancer-predisposing syndrome, Global developmental delay - lung cysts - overgrowth - Wilms tumor syndrome
RS368588781	Health Risk	Conflicting classifications of pathogenicity	DICER1-related tumor predisposition, Hereditary cancer-predisposing syndrome, DICER1-related disorder
RS368610812	Health Risk	Conflicting classifications of pathogenicity	DICER1-related tumor predisposition, Hereditary cancer-predisposing syndrome, DICER1-related tumor predisposition
RS368963384	Health Risk	Conflicting classifications of pathogenicity	DICER1-related tumor predisposition, Hereditary cancer-predisposing syndrome, Global developmental delay - lung cysts - overgrowth - Wilms tumor syndrome
RS368984998	Health Risk	Conflicting classifications of pathogenicity	Hereditary cancer-predisposing syndrome, DICER1-related tumor predisposition, DICER1-related disorder
RS369145814	Health Risk	Conflicting classifications of pathogenicity	DICER1-related tumor predisposition, Hereditary cancer-predisposing syndrome, Global developmental delay - lung cysts - overgrowth - Wilms tumor syndrome
RS370474705	Health Risk	Conflicting classifications of pathogenicity	DICER1-related tumor predisposition, Hereditary cancer-predisposing syndrome, DICER1-related tumor predisposition
RS372581591	Health Risk	Conflicting classifications of pathogenicity	Hereditary cancer-predisposing syndrome, DICER1-related tumor predisposition, DICER1-related tumor predisposition
RS372967646	Health Risk	Conflicting classifications of pathogenicity	Hereditary cancer-predisposing syndrome, DICER1-related tumor predisposition, Global developmental delay - lung cysts - overgrowth - Wilms tumor syndrome
RS373412959	Health Risk	Conflicting classifications of pathogenicity	DICER1-related tumor predisposition, Hereditary cancer-predisposing syndrome, Ovarian cancer
RS373532001	Health Risk	Conflicting classifications of pathogenicity	DICER1-related tumor predisposition, Hereditary cancer-predisposing syndrome, DICER1-related tumor predisposition
RS373646414	Health Risk	Conflicting classifications of pathogenicity	DICER1-related tumor predisposition, Hereditary cancer-predisposing syndrome, Pleuropulmonary blastoma
RS373715574	Health Risk	Conflicting classifications of pathogenicity	DICER1-related tumor predisposition, Hereditary cancer-predisposing syndrome, Pleuropulmonary blastoma
RS373734886	Health Risk	Conflicting classifications of pathogenicity	Hereditary cancer-predisposing syndrome, DICER1-related tumor predisposition, Hereditary cancer-predisposing syndrome
RS373810197	Health Risk	Conflicting classifications of pathogenicity	DICER1-related tumor predisposition, Hereditary cancer-predisposing syndrome, DICER1-related disorder
RS375239471	Health Risk	Conflicting classifications of pathogenicity	DICER1-related tumor predisposition, DICER1-related disorder, Pleuropulmonary blastoma
RS375772460	Health Risk	Conflicting classifications of pathogenicity	DICER1-related tumor predisposition, Hereditary cancer-predisposing syndrome, DICER1-related disorder
RS376038413	Health Risk	Conflicting classifications of pathogenicity	DICER1-related tumor predisposition, Hereditary cancer-predisposing syndrome, Global developmental delay - lung cysts - overgrowth - Wilms tumor syndrome
RS376041812	Health Risk	Conflicting classifications of pathogenicity	DICER1-related tumor predisposition, Hereditary cancer-predisposing syndrome, DICER1-related disorder
RS376110719	Health Risk	Conflicting classifications of pathogenicity	DICER1-related tumor predisposition, Hereditary cancer-predisposing syndrome, DICER1-related tumor predisposition
RS376762569	Health Risk	Conflicting classifications of pathogenicity	DICER1-related tumor predisposition, Hereditary cancer-predisposing syndrome, DICER1-related disorder
RS377208116	Health Risk	Conflicting classifications of pathogenicity	DICER1-related tumor predisposition, Hereditary cancer-predisposing syndrome, Global developmental delay - lung cysts - overgrowth - Wilms tumor syndrome
RS377326167	Health Risk	Conflicting classifications of pathogenicity	DICER1-related tumor predisposition, Hereditary cancer-predisposing syndrome, Pleuropulmonary blastoma
RS377685186	Health Risk	Conflicting classifications of pathogenicity	DICER1-related tumor predisposition, Hereditary cancer-predisposing syndrome, Global developmental delay - lung cysts - overgrowth - Wilms tumor syndrome
RS527568726	Health Risk	Conflicting classifications of pathogenicity	DICER1-related tumor predisposition, Hereditary cancer-predisposing syndrome, Pleuropulmonary blastoma
RS529131866	Health Risk	Conflicting classifications of pathogenicity	DICER1-related tumor predisposition, Hereditary cancer-predisposing syndrome, Ovarian cancer
RS529235567	Health Risk	Conflicting classifications of pathogenicity	DICER1-related tumor predisposition, DICER1-related tumor predisposition
RS543843695	Health Risk	Conflicting classifications of pathogenicity	DICER1-related tumor predisposition, Hereditary cancer-predisposing syndrome, DICER1-related tumor predisposition
RS544623004	Health Risk	Conflicting classifications of pathogenicity	DICER1-related tumor predisposition, Hereditary cancer-predisposing syndrome, Global developmental delay - lung cysts - overgrowth - Wilms tumor syndrome
RS544960260	Health Risk	Conflicting classifications of pathogenicity	DICER1-related tumor predisposition, Hereditary cancer-predisposing syndrome, DICER1-related tumor predisposition
RS545392797	Health Risk	Conflicting classifications of pathogenicity	DICER1-related tumor predisposition, Hereditary cancer-predisposing syndrome, Global developmental delay - lung cysts - overgrowth - Wilms tumor syndrome
RS549532374	Health Risk	Conflicting classifications of pathogenicity	DICER1-related tumor predisposition, Hereditary cancer-predisposing syndrome, Global developmental delay - lung cysts - overgrowth - Wilms tumor syndrome
RS553116282	Health Risk	Conflicting classifications of pathogenicity	DICER1-related tumor predisposition, Hereditary cancer-predisposing syndrome, DICER1-related tumor predisposition
RS557182925	Health Risk	Conflicting classifications of pathogenicity	Hereditary cancer-predisposing syndrome, DICER1-related tumor predisposition, Hereditary cancer-predisposing syndrome
RS559078811	Health Risk	Conflicting classifications of pathogenicity	DICER1-related tumor predisposition, Hereditary cancer-predisposing syndrome, DICER1-related tumor predisposition
RS560739585	Health Risk	Conflicting classifications of pathogenicity	DICER1-related tumor predisposition, Hereditary cancer-predisposing syndrome, DICER1-related tumor predisposition
RS561584807	Health Risk	Conflicting classifications of pathogenicity	DICER1-related tumor predisposition, Hereditary cancer-predisposing syndrome, DICER1-related tumor predisposition
RS564224919	Health Risk	Conflicting classifications of pathogenicity	Hereditary cancer-predisposing syndrome, DICER1-related tumor predisposition, Pleuropulmonary blastoma
RS566464225	Health Risk	Conflicting classifications of pathogenicity	DICER1-related tumor predisposition, Hereditary cancer-predisposing syndrome, Global developmental delay - lung cysts - overgrowth - Wilms tumor syndrome
RS567895583	Health Risk	Conflicting classifications of pathogenicity	DICER1-related tumor predisposition, Hereditary cancer-predisposing syndrome, Euthyroid goiter

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