1. Gene
  2. DROSHA - drosha ribonuclease III Gene

DROSHA - drosha ribonuclease III Gene

Homo sapiens

Also known as RN3; ETOHI2; RNASEN; RANSE3L; RNASE3L; HSA242976

Gene ID: 29102 | Gene type: protein coding

About DROSHA

Cytogenetic location: 5p13.3 Genomic coordinates (GRCh38): 5:31,400,494-31,532,093 (from NCBI)

This gene has 20 transcripts (splice variants), 199 orthologues and is associated with 90 phenotypes. Ubiquitous expression in brain (RPKM 9.5), ovary (RPKM 7.6) and 25 other tissues.

Summary

This gene encodes a ribonuclease (RNase) III double-stranded RNA-specific ribonuclease and subunit of the microprocessor protein complex, which catalyzes the initial processing step of MicroRNA (miRNA) synthesis. The encoded protein cleaves the stem loop structure from the primary MicroRNA (pri-miRNA) in the nucleus, yielding the precursor miRNA (pre-miRNA), which is then exported to the cytoplasm for further processing. In a human cell line lacking a functional copy of this gene, canonical miRNA synthesis is reduced. Somatic mutations in this gene have been observed in human patients with kidney Cancer. [provided by RefSeq, Sep 2016]

DROSHA Products(3)

mRNA Protein Name
NM_001100412.2 NP_001093882.1 ribonuclease 3 isoform 2
NM_001382508.1 NP_001369437.1 ribonuclease 3 isoform 1
NM_013235.5 NP_037367.3 ribonuclease 3 isoform 1

DROSHA Protein Structure

Ribonucleas_3_3

Ribonucleas_3_3: Ribonuclease-III-like (962 - 1070)

Ribonuclease_3

Ribonuclease_3: Ribonuclease III domain (1144 - 1232)

dsrm

dsrm: Double-stranded RNA binding motif (1261 - 1331)

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  • 1374 a.a.
Protein Preferred Names Protein Names

ribonuclease 3

RNase III

Related Diseases

Diseases Alias
Hereditary Breast Ovarian Cancer Syndrome

Hereditary Breast And Ovarian Cancer Syndrome

Brca1- And Brca2-Associated Hereditary Breast And Ovarian Cancer

Breast And/Or Ovarian Cancer

Breast And Ovarian Cancer Syndrome

Hboc Syndrome

Hereditary Breast And Ovarian Cancer

Brca1- Brca2-Associated Hboc

Digeorge Syndrome

Chromosome 22q11.2 Deletion Syndrome

DGS

Hypoplasia Of Thymus And Parathyroids

Third And Fourth Pharyngeal Pouch Syndrome

22q11.2 Deletion Syndrome

Digeorge Sequence

Digeorge'S Syndrome

Pharyngeal Pouch Syndrome

Di-George Syndrome

Shprintzen Syndrome

Pineal Region Choriocarcinoma

Pineal Choriocarcinoma

Dicer1 Syndrome

Pleuro-Pulmonary Blastoma Familial Tumor Susceptibility Syndrome

Pleuropulmonary Blastoma Familial Tumor Susceptibility Syndrome

Ppb Familial Tumor Susceptibility Syndrome

Dicer1-Related Pleuropulmonary Blastoma Cancer Predisposition Syndrome

Pleuro-Pulmonary Blastoma Familial Tumor Susceptibility

Dicer1-Related Pleuropulmonary Blastoma

Pleuropulmonary Blastoma Familial Tumor And Dysplasia Syndrome

Pleuropulmonary Blastoma Family Tumor Susceptibility Syndrome

Ppbftds

Doid:0081063

Acrocephalopolysyndactyly Type Iii

Sakati Syndrome

Sakati-Nyhan Syndrome

Acps With Leg Hypoplasia

Acps Iii

Acrocephalopolysyndactyly Type 3

Sakati-Nyhan-Tisdale Syndrome

Pineoblastoma

Pineal Pnet

Pinealoma

Pineal Gland Neoplasm

Pineocytoma

Tumor Of The Pineal Region

Pineal Gland Cancer

Tumor Of The Pineal Region

Malignant Pineal Region Tumor

Malignant Tumor Of Pineal Gland

Neoplasm Of Pineal Gland

Neoplasm Of The Pineal Region

Pineal Body Neoplasm

Pineocytic Tumor

Malignant Neoplasm Of Pineal Gland

Pineal Gland Neoplasm

Pineocytoma

Pineal Parenchymal Tumor Of Intermediate Differentiation

Pineal Parenchymal Tumour Of Intermediate Differentiation

Pineal Parenchymal Tumors Of Intermediate Differentiation

Pptid

Kidney Cancer

Renal Cancer

Renal Carcinoma

Kidney Neoplasm

Malignant Neoplasm Of Kidney Except Pelvis

Malignant Tumour Of Kidney

Kidney Neoplasms

Cancer, Kidney

Cancer, Renal

Malignant Neoplasm Of Kidney

Renal Cell Carcinoma

Pleuropulmonary Blastoma

PPB

Ppb Familial Tumor And Dysplasia Syndrome

Ppbftds

Pulmonary Blastoma

Blastoma, Pleuropulmonary

Dicer1 Syndrome

Respiratory Tract Neoplasms

Lung Cancer

Lung Carcinoma

Non-Small Cell Lung Carcinoma

Lung Cancer, Susceptibility To

Lung Cancer, Protection Against

Adenocarcinoma Of Lung, Somatic

Adenocarcinoma Of Lung, Response To Tyrosine Kinase Inhibitor In

Nonsmall Cell Lung Cancer

Lung Neoplasm

Carcinoma Of Lung

Lung Non-Small Cell Carcinoma

Non-Small Cell Lung Cancer

Nsclc

Lung Neoplasms

Malignant Neoplasm Of Lung

Alveolar Cell Carcinoma

Nonsmall Cell Lung Cancer, Somatic

Nonsmall Cell Lung Cancer, Response To Tyrosine Kinase Inhibitor In

Nonsmall Cell Lung Cancer, Susceptibility To

Lung Cancer, Somatic

Lung Cancer, Resistance To

Cancer Of Lung

Cancer Of Bronchus

Cancer Of The Lung

Lung Malignancies

Lung Malignant Tumors

Malignant Lung Tumor

Malignant Tumor Of Lung

Pulmonary Cancer

Pulmonary Carcinoma

Pulmonary Neoplasms

Respiratory Carcinoma

LNCR

Adenocarcinoma Of Lung

Neoplasm Of Lung

Cancer Lung

Carcinoma Non-Small Cell Lung

Carcinoma, Non-Small-Cell Lung

Lung Cancers

Lung Carcinomas

Cancer, Lung

Cancer, Lung, Non-Small Cell

Primary Malignant Neoplasm Of Lung

Bronchioloalveolar Adenocarcinoma

Maxillonasal Dysplasia, Binder Type

Binder Syndrome

Binder Type Maxillonasal Dysplasia

Maxillonasal Dysplasia

Maxillonasal Dysostosis

Intrahepatic Gall Duct Cancer

Malignant Neoplasm Of Intrahepatic Bile Ducts

Malignant Neoplasm Of Intrahepatic Gall Duct

Ca Intrahepatic Bile Ducts

Malignant Neoplasm Of Intrahepatic Biliary Passages

Intrahepatic Bile Duct Carcinoma

X-Linked Hereditary Ataxia
Fragile X-Associated Tremor/Ataxia Syndrome

Fxtas Syndrome

Fragile X Tremor/Ataxia Syndrome

Fxtas

Chromosomal Deletion Syndrome
Premature Ovarian Failure 1

Ovarian Failure, Premature

Fmr1-Related Primary Ovarian Insufficiency

Fragile X-Associated Primary Ovarian Insufficiency

POF1

Pofx

Hypergonadotropic Ovarian Failure, X-Linked

Pof

Primary Ovarian Insufficiency, Fragile X-Associated

Primary Ovarian Insufficiency 1

Ovarian Failure Premature

Premature Ovarian Failure, X-Linked

Fragile X Premature Ovarian Failure

Fmr1-Related Premature Ovarian Failure

Familial Premature Ovarian Failure

Idiopathic Familial Premature Ovarian Failure

Fxpoi

X-Linked Hypergonadotropic Ovarian Failure

Hypergonadotropic Ovarian Failure X-Linked

Poi

Premature Ovarian Failure X-Linked

Primary Ovarian Insufficiency

Premature Ovarian Failure-1

Ovarian Failure, Premature, Type 1

Premature Ovarian Failure, Familial

Premature Menopause

Primary Hypogonadism

Turner Syndrome

Colorectal Cancer

Colon Cancer

Colorectal Carcinoma

Colon Carcinoma

Colorectal Cancer, Susceptibility To

Carcinoma Of Colon

CRC

Colorectal Cancer With Chromosomal Instability, Somatic

Colon Cancer, Somatic

Colon Cancer, Susceptibility To

Colonic Neoplasms

Colorectal Neoplasms

Colorectal Cancer, Somatic

Colon Cancer, Advanced, Somatic

Colonic Carcinoma

Colorectal Carcinomas

Colon Cancers

Colorectal Cancers

Cancer, Colorectal, Somatic

Cancer, Colon

Cancer, Colorectal, Susceptibility To

Colorectal Neoplasm

Colonic Neoplasm

Malignant Tumor Of Colon

Feingold Syndrome 1

Feingold Syndrome

Oculodigitoesophagoduodenal Syndrome

Oded Syndrome

Microcephaly-Oculo-Digito-Esophageal-Duodenal Syndrome

Digital Anomalies With Short Palpebral Fissures And Atresia Of Esophagus Or Duodenum

FGLDS1

Mmt Syndrome

Brunner-Winter Syndrome

Feingold Syndrome Type 1

Oded

Moded

Fglds

Microcephaly-Digital Anomalies-Normal Intelligence Syndrome

Moded Syndrome

Oculo-Digito-Esophageal-Duodenal Syndrome

Microcephaly, Mental Retardation, And Tracheoesophageal Fistula Syndrome

Microcephaly And Digital Abnormalities With Normal Intelligence

Digital Anomalies With Short Palpebral Fissures And Atresia Of Esophagus, Or Duodenum

Microcephaly-Mesobrachyphalangy-Tracheoesophageal Fistula Syndrome

Microcephaly-Oculo-Digito-Esophageal-Duodenal Syndrome

Oculo-Digito-Esophagoduodental Syndrome

Fs

Mmt

Microcephaly-Intellectual Disability-Tracheoesophageal Fistula Syndrome

Microcephaly-Oculo-Digito-Esophageal-Duodenal Syndrome Syndrome

Brunner-Winter Syndrome Type 1

Digital Anomalies With Short Palpebral Fissures And Atresia Of Esophagus Or Duodenum Type 1

Fs1

Mmt Type 1

Moded Syndrome Type 1

Microcephaly-Digital Anomalies-Normal Intelligence Syndrome Type 1

Microcephaly-Intellectual Disability-Tracheoesophageal Fistula Syndrome Type 1

Microcephaly-Oculo-Digito-Esophageal-Duodenal Syndrome Syndrome Type 1

Oded Syndrome Type 1

Oculo-Digito-Esophageal-Duodenal Syndrome Type 1

Feingold Syndrome, Type 1

Intellectual Developmental Disorder, X-Linked, Syndromic, Wilson-Turner Type

Wilson-Turner Syndrome

WTS

Mrxs6

X-Linked Intellectual Disability-Gynecomastia-Obesity Syndrome

Mrxswt

Wilson-Turner X-Linked Mental Retardation Syndrome

Mental Retardation, X-Linked, Syndromic 6

Mental Retardation, X-Linked, With Gynecomastia And Obesity

Intellectual Disability, X-Linked, Syndromic 6

Intellectual Disability, X-Linked, With Gynecomastia And Obesity

Wilson Turner Intellectual Disability Syndrome

X-Linked Intellectual Disability - Gynecomastia - Obesity

Wilms Tumor 1

Nephroblastoma

Wilms Tumor

WT1

Wilms' Tumor

Bilateral Wilms Tumor

Wilms Tumor, Type 1

Wilms Tumor, Somatic

Adult Nephroblastoma

Wt1 Disorder

Renal Embryonic Tumor

Adult Kidney Wilms Tumor

Childhood Kidney Wilms Tumor

Nonanaplastic Kidney Wilms Tumor

Leukemia, Chronic Lymphocytic

Chronic Lymphocytic Leukemia

B-Cell Chronic Lymphocytic Leukemia

CLL

B-Cell Chronic Lymphoid Leukemia

Chronic Lymphatic Leukemia

Chronic Lymphocytic Leukaemia

Lymphoplasmacytic Leukemia

Small Lymphocytic Lymphoma

Leukemia, Chronic Lymphatic

B-Cell Chronic Lymphocytic Leukaemia

Chronic Lymphatic Leukaemia

Lymphoplasmacytic Leukaemia

B Cell Chronic Lymphocytic Leukemia

Chronic B-Cell Lymphocytic Leukemia

Leukemia, Lymphocytic, Chronic

B-Cll

Chronic Lymphoid Leukemia

Leukemia Lymphocytic Chronic

Lymphoma Small Lymphocytic

Leukemia, Lymphocytic, Chronic, B-Cell

Amyotrophic Lateral Sclerosis 1

Amyotrophic Lateral Sclerosis

ALS

Lou Gehrig Disease

Amyotrophic Lateral Sclerosis Type 1

Charcot Disease

ALS1

Amyotrophic Lateral Sclerosis, Susceptibility To

Fals

Lou Gehrig'S Disease

Mnd

Motor Neuron Disease

Familial Amyotrophic Lateral Sclerosis

Amyotrophic Lateral Sclerosis 1, Familial

Amyotrophic Lateral Sclerosis 1, Autosomal Dominant

Motor Neuron Disease, Bulbar

Motor Neurone Disease

Amyotrophic Lateral Sclerosis With Dementia

Dementia With Amyotrophic Lateral Sclerosis

Motor Neuron Disease, Amyotrophic Lateral Sclerosis

Sclerosis, Lateral, Amyotrophic

Sclerosis, Lateral, Amyotrophic, Type 1

Amyotrophic Sclerosis

Als - [Amyotrophic Lateral Sclerosis]

Wasting Palsy

Amyotrophic Paralysis

Amyotrophy Lateral Sclerosis

Wasting Paralysis

Spinal Progressive Amyotrophy

Progressive Atrophic Paralysis

Diseases Alias
Waldenstroem'S Macroglobulinemia

Waldenstroem'S Macroglobulinemia

Waldenstroem'S Macroglobulinemia

Macroglobulinemia Of Waldenstrom

Lymphoplasmacytic Lymphoma With Igm Gammopathy

Lymphoplasmacytic Lymphoma

Waldenstroem'S Macroglobulinemia

Waldenstroem'S Macroglobulinemia

Macroglobulinemia Of Waldenstrom

Lymphoplasmacytic Lymphoma With Igm Gammopathy

Lymphoplasmacytic Lymphoma

Orthologs Information

Species Symbol Source ID
Bos taurus DROSHA VGNC VGNC:28211
Mus musculus DROSHA MGD MGI:1261425
Felis catus DROSHA VGNC VGNC:61632
Macaca mulatta DROSHA VGNC VGNC:71854
Rattus norvegicus DROSHA RGD RGD:1307626