Previous symbols: MNG1. Aliases: Dicer, KIAA0928, K12H4.8-LIKE, HERNA.
Relationship is the entry's genetic[].relationship_type. untyped means the entry has a genetic record for the gene but does not type it; its free-text association is shown instead.
| Disorder | Relationship | Where the gene appears |
|---|---|---|
| DICER1 Tumor Predisposition Syndrome | causative germline loss of function |
mechanism: Germline DICER1 First-Hit Inactivation
|
| Embryonal Rhabdomyosarcoma | risk factor germline and somatic | |
| Familial Nonmedullary Thyroid Carcinoma | causative germline | |
| Wilms Tumor | risk factor |
mechanism: MicroRNA Processing Defect
|
| Congenital Pulmonary Airway Malformation |
untyped
Pathogenic Variants
germline
|
mechanism: DICER1-Driven Pleuropulmonary Blastoma Overlap
|
| Malignant Sertoli-Leydig Cell Tumor of Ovary |
untyped
Somatic missense mutations at the four metal-binding residues of the RNase IIIb catalytic centre are the defining recurrent lesion, present in about 44-60% of Sertoli-Leydig cell tumors and in all tumors with retiform or heterologous elements. A substantial fraction of patients also carry a germline loss-of-function DICER1 variant (DICER1-related tumor predisposition).
somatic
|
mechanism: DICER1 RNase IIIb Hotspot Mutation; Germline DICER1 Loss-of-Function Predisposition
|
| Pineoblastoma |
untyped
Germline and Somatic Alterations
|
mechanism: MicroRNA-Processing Pathway Inactivation
subtype: PB-miRNA1; PB-miRNA2
|
| Pleuropulmonary Blastoma |
untyped
Germline Loss-of-Function with Somatic RNase IIIb Second Hit
|
mechanism: Compound DICER1 Disruption; Germline DICER1 Predisposition
|
| Sarcoma Of Cervix Uteri |
untyped
Somatic and germline loss-of-function mutation; Somatic mutation in a subset
|
An entry matches when ClinGen's MONDO disease is the entry's own disease term, a subtype term, or an exact-match mapping. An amber status marks an entry ClinGen supports the gene for (Definitive, Strong or Moderate) whose own record does not type it. A Limited, Disputed or Refuted tier on such an entry is not a gap.
| ClinGen disease | MOI | Classification | dismech entry |
|---|---|---|---|
| DICER1-related tumor predisposition MONDO:0100216 | AD | Definitive 2023-07-05 |
DICER1 Tumor Predisposition Syndrome
causative
|
Identity from HGNC (snapshot 2026-10-01). Page layers are described in the gene pages documentation.