RNH1 ribonuclease/angiogenin inhibitor 1 [Homo sapiens (human)] - Gene

Summary

Official Symbol: RNH1provided by HGNC
Official Full Name: ribonuclease/angiogenin inhibitor 1provided by HGNC
Primary source: HGNC:HGNC:10074
See related: Ensembl:ENSG00000023191 MIM:173320; AllianceGenome:HGNC:10074
Gene type: protein coding
RefSeq status: VALIDATED
Organism: Homo sapiens
Lineage: Eukaryota; Metazoa; Chordata; Craniata; Vertebrata; Euteleostomi; Mammalia; Eutheria; Euarchontoglires; Primates; Haplorrhini; Catarrhini; Hominidae; Homo
Also known as: RAI; RNH; IIAE12
Summary: Placental ribonuclease inhibitor (PRI) is a member of a family of proteinaceous cytoplasmic RNase inhibitors that occur in many tissues and bind to both intracellular and extracellular RNases (summarized by Lee et al., 1988 [PubMed 3219362]). In addition to control of intracellular RNases, the inhibitor may have a role in the regulation of angiogenin (MIM 105850). Ribonuclease inhibitor, of 50,000 Da, binds to ribonucleases and holds them in a latent form. Since neutral and alkaline ribonucleases probably play a critical role in the turnover of RNA in eukaryotic cells, RNH may be essential for control of mRNA turnover; the interaction of eukaryotic cells with ribonuclease may be reversible in vivo.[supplied by OMIM, Jul 2010]
Expression: Ubiquitous expression in skin (RPKM 36.6), lung (RPKM 36.0) and 25 other tissues See more
Orthologs: mouse all
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Genomic context

Location:: 11p15.5

Exon count:: 13

Annotation release	Status	Assembly	Chr	Location
RS_2023_10	current	GRCh38.p14 (GCF_000001405.40)	11	NC_000011.10 (494515..507242, complement)
RS_2023_10	current	T2T-CHM13v2.0 (GCF_009914755.1)	11	NC_060935.1 (540227..553016, complement)
105.20220307	previous assembly	GRCh37.p13 (GCF_000001405.25)	11	NC_000011.9 (494515..507242, complement)

Chromosome 11 - NC_000011.10 Genomic Context describing neighboring genes

Genomic regions, transcripts, and products

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Go to reference sequence details

Genomic Sequence:

Go to nucleotide: Graphics FASTA GenBank

Expression

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See details

Project title: HPA RNA-seq normal tissues
Description: RNA-seq was performed of tissue samples from 95 human individuals representing 27 different tissues in order to determine tissue-specificity of all protein-coding genes
BioProject: PRJEB4337
Publication: PMID 24309898
Analysis date: Wed Apr 4 07:08:55 2018

Bibliography

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GeneRIFs: Gene References Into Functions

What's a GeneRIF?

Biallelic variants in ribonuclease inhibitor (RNH1), an inflammasome modulator, are associated with a distinctive subtype of acute, necrotizing encephalopathy.
Title: Biallelic variants in ribonuclease inhibitor (RNH1), an inflammasome modulator, are associated with a distinctive subtype of acute, necrotizing encephalopathy.
Ribonuclease inhibitor 1 (RNH1) deficiency cause congenital cataracts and global developmental delay with infection-induced psychomotor regression and anemia.
Title: Ribonuclease inhibitor 1 (RNH1) deficiency cause congenital cataracts and global developmental delay with infection-induced psychomotor regression and anemia.
Functional status analysis of RNH1 in bladder cancer for predicting immunotherapy response.
Title: Functional status analysis of RNH1 in bladder cancer for predicting immunotherapy response.
LRR-protein RNH1 dampens the inflammasome activation and is associated with COVID-19 severity.
Title: LRR-protein RNH1 dampens the inflammasome activation and is associated with COVID-19 severity.
Angiogenin (ANG)-Ribonuclease Inhibitor (RNH1) System in Protein Synthesis and Disease.
Title: Angiogenin (ANG)-Ribonuclease Inhibitor (RNH1) System in Protein Synthesis and Disease.
This study first time revealed in vivo role of RNH1. This study report that RNH1 is important for embryonic development and erythropoiesis. RNH1 binds to ribosomes and regulates erythropoiesis by controlling translation of the erythroid transcription factor GATA1.
Title: Ribonuclease inhibitor 1 regulates erythropoiesis by controlling GATA1 translation.
RNH1 might represent the first direct regulator of IRE1 RNase activity
Title: Regulation of IRE1 RNase activity by the Ribonuclease inhibitor 1 (RNH1).
Results show that a low expression of RNH1 in metastasis of bladder cancer and suggest that it might regulate the function of ILK through mutual binding.
Title: Interplay between intergrin-linked kinase and ribonuclease inhibitor affects growth and metastasis of bladder cancer through signaling ILK pathways.
Data suggest that ribonuclease inhibitor (RNH1) protects HeLa cells from ribonuclease 1 (RNase 1).
Title: Knockout of the Ribonuclease Inhibitor Gene Leaves Human Cells Vulnerable to Secretory Ribonucleases.
a novel mechanism of ANG in regulating PI3K/AKT/mTOR signaling pathway via RI, is reported.
Title: Angiogenin interacts with ribonuclease inhibitor regulating PI3K/AKT/mTOR signaling pathway in bladder cancer cells.

Submit: New GeneRIF Correction See all GeneRIFs (26)

Phenotypes

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Find tests for this gene in the NIH Genetic Testing Registry (GTR)

Review eQTL and phenotype association data in this region using PheGenI

Associated conditions

Description	Tests
Encephalitis, acute, infection-induced, susceptibility to, 12 MedGen: CN372695 OMIM: 620461 GeneReviews: Not available	not available

Variation

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See variants in ClinVar

See studies and variants in dbVar

See Variation Viewer (GRCh37.p13)

See Variation Viewer (GRCh38)

HIV-1 interactions

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Replication interactions

Interaction	Pubs
Knockdown of ribonuclease/angiogenin inhibitor 1 (RNH1) by siRNA inhibits the early stages of HIV-1 replication in 293T cells infected with VSV-G pseudotyped HIV-1	PubMed

Protein interactions

Protein	Gene	Interaction	Pubs
Envelope surface glycoprotein gp160, precursor	env	HIV-1 gp160 interacts with RNH1	PubMed
nucleocapsid	gag	HIV-1 NC is identified to have a physical interaction with ribonuclease/angiogenin inhibitor 1 (RNH1) in human HEK293 and/or Jurkat cell lines by using affinity tagging and purification mass spectrometry analyses	PubMed
retropepsin	gag-pol	Positional proteomics analysis identifies the cleavage of human ribonuclease/angiogenin inhibitor 1 (RNH1) at amino acid residues 445-446 by the HIV-1 protease	PubMed

Go to the HIV-1, Human Interaction Database

Interactions

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Products	Interactant	Other Gene	Complex	Source	Pubs	Description

General gene information

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Markers

D11S2921 (e-PCR)
- UniSTS:152074

L18426 (e-PCR)
- UniSTS:34648

GDB:197903 (e-PCR)
- UniSTS:155996

RNH (e-PCR)
- UniSTS:66667

RH78931 (e-PCR)
- UniSTS:18580

SHGC-31515 (e-PCR)
- UniSTS:15068

Gene Ontology Provided by GOA

Function	Evidence Code	Pubs
enables protein binding	IPI Inferred from Physical Interaction more info	PubMed
enables ribonuclease inhibitor activity	IBA Inferred from Biological aspect of Ancestor more info
enables ribonuclease inhibitor activity	IDA Inferred from Direct Assay more info	PubMed

Process	Evidence Code	Pubs
involved_in cell migration	IBA Inferred from Biological aspect of Ancestor more info
involved_in mRNA catabolic process	NAS Non-traceable Author Statement more info	PubMed
involved_in regulation of Arp2/3 complex-mediated actin nucleation	IBA Inferred from Biological aspect of Ancestor more info
involved_in regulation of angiogenesis	IBA Inferred from Biological aspect of Ancestor more info
involved_in regulation of angiogenesis	IDA Inferred from Direct Assay more info	PubMed

Component	Evidence Code	Pubs
part_of angiogenin-PRI complex	IBA Inferred from Biological aspect of Ancestor more info
part_of angiogenin-PRI complex	IPI Inferred from Physical Interaction more info	PubMed
located_in cytosol	IDA Inferred from Direct Assay more info
located_in extracellular exosome	HDA more info	PubMed
is_active_in lamellipodium	IBA Inferred from Biological aspect of Ancestor more info
is_active_in nucleoplasm	IBA Inferred from Biological aspect of Ancestor more info
located_in nucleoplasm	IDA Inferred from Direct Assay more info
is_active_in plasma membrane	IBA Inferred from Biological aspect of Ancestor more info

General protein information

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Preferred Names: ribonuclease inhibitor

Names: placental RNase inhibitor; placental ribonuclease inhibitor; testicular tissue protein Li 164

NCBI Reference Sequences (RefSeq)

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RefSeqs maintained independently of Annotated Genomes

These reference sequences exist independently of genome builds. Explain

These reference sequences are curated independently of the genome annotation cycle, so their versions may not match the RefSeq versions in the current genome build. Identify version mismatches by comparing the version of the RefSeq in this section to the one reported in Genomic regions, transcripts, and products above.

mRNA and Protein(s)

NM_002939.4 → NP_002930.2 ribonuclease inhibitor

See identical proteins and their annotated locations for NP_002930.2

Status: VALIDATED

Description

Transcript Variant: This variant (1) represents the longest transcript. All eight variants of this gene encode the same protein.

Source sequence(s)

BC047730

Consensus CDS

CCDS7697.1

UniProtKB/Swiss-Prot

P13489, Q8IZK8, Q96FD7, Q9BQ80, Q9UDK6

UniProtKB/TrEMBL

A0A140VJT8

Related

ENSP00000380739.2, ENST00000397615.6

Conserved Domains (3) summary

smart00368
Location:397 → 424

LRR_RI; Leucine rich repeat, ribonuclease inhibitor type

cd00116
Location:6 → 325

LRR_RI; Leucine-rich repeats (LRRs), ribonuclease inhibitor (RI)-like subfamily. LRRs are 20-29 residue sequence motifs present in many proteins that participate in protein-protein interactions and have different functions and cellular locations. LRRs correspond ...

sd00033
Location:6 → 29

LRR_RI; leucine-rich repeat [structural motif]
NM_203383.2 → NP_976317.1 ribonuclease inhibitor

See identical proteins and their annotated locations for NP_976317.1

Status: VALIDATED

Description

Transcript Variant: This variant (2) uses a different splice site in the 5' UTR, compared to variant 1. All eight variants of this gene encode the same protein.

Source sequence(s)

BC011186, BG752774

Consensus CDS

CCDS7697.1

UniProtKB/Swiss-Prot

P13489, Q8IZK8, Q96FD7, Q9BQ80, Q9UDK6

UniProtKB/TrEMBL

A0A140VJT8

Conserved Domains (3) summary

smart00368
Location:397 → 424

LRR_RI; Leucine rich repeat, ribonuclease inhibitor type

cd00116
Location:6 → 325

LRR_RI; Leucine-rich repeats (LRRs), ribonuclease inhibitor (RI)-like subfamily. LRRs are 20-29 residue sequence motifs present in many proteins that participate in protein-protein interactions and have different functions and cellular locations. LRRs correspond ...

sd00033
Location:6 → 29

LRR_RI; leucine-rich repeat [structural motif]
NM_203384.2 → NP_976318.1 ribonuclease inhibitor

See identical proteins and their annotated locations for NP_976318.1

Status: VALIDATED

Description

Transcript Variant: This variant (3) lacks an alternate exon and uses a different splice site in the 5' UTR, compared to variant 1. All eight variants of this gene encode the same protein.

Source sequence(s)

BC003506, BC011186, BM019570

Consensus CDS

CCDS7697.1

UniProtKB/Swiss-Prot

P13489, Q8IZK8, Q96FD7, Q9BQ80, Q9UDK6

UniProtKB/TrEMBL

A0A140VJT8

Related

ENSP00000416589.2, ENST00000438658.6

Conserved Domains (3) summary

smart00368
Location:397 → 424

LRR_RI; Leucine rich repeat, ribonuclease inhibitor type

cd00116
Location:6 → 325

LRR_RI; Leucine-rich repeats (LRRs), ribonuclease inhibitor (RI)-like subfamily. LRRs are 20-29 residue sequence motifs present in many proteins that participate in protein-protein interactions and have different functions and cellular locations. LRRs correspond ...

sd00033
Location:6 → 29

LRR_RI; leucine-rich repeat [structural motif]
NM_203385.2 → NP_976319.1 ribonuclease inhibitor

See identical proteins and their annotated locations for NP_976319.1

Status: VALIDATED

Description

Transcript Variant: This variant (4) lacks an alternate exon in the 5' UTR, compared to variant 1. All eight variants of this gene encode the same protein.

Source sequence(s)

BC011186, BM019570, BQ954409

Consensus CDS

CCDS7697.1

UniProtKB/Swiss-Prot

P13489, Q8IZK8, Q96FD7, Q9BQ80, Q9UDK6

UniProtKB/TrEMBL

A0A140VJT8

Related

ENSP00000380738.1, ENST00000397614.5

Conserved Domains (3) summary

smart00368
Location:397 → 424

LRR_RI; Leucine rich repeat, ribonuclease inhibitor type

cd00116
Location:6 → 325

LRR_RI; Leucine-rich repeats (LRRs), ribonuclease inhibitor (RI)-like subfamily. LRRs are 20-29 residue sequence motifs present in many proteins that participate in protein-protein interactions and have different functions and cellular locations. LRRs correspond ...

sd00033
Location:6 → 29

LRR_RI; leucine-rich repeat [structural motif]
NM_203386.3 → NP_976320.1 ribonuclease inhibitor

See identical proteins and their annotated locations for NP_976320.1

Status: VALIDATED

Description

Transcript Variant: This variant (5) contains a different exon and uses a different splice site in its 5' UTR, compared to variant 1. All eight variants of this gene encode the same protein.

Source sequence(s)

AC137894, BC011186, BM465975

Consensus CDS

CCDS7697.1

UniProtKB/Swiss-Prot

P13489, Q8IZK8, Q96FD7, Q9BQ80, Q9UDK6

UniProtKB/TrEMBL

A0A140VJT8

Related

ENSP00000435594.1, ENST00000533410.5

Conserved Domains (3) summary

smart00368
Location:397 → 424

LRR_RI; Leucine rich repeat, ribonuclease inhibitor type

cd00116
Location:6 → 325

LRR_RI; Leucine-rich repeats (LRRs), ribonuclease inhibitor (RI)-like subfamily. LRRs are 20-29 residue sequence motifs present in many proteins that participate in protein-protein interactions and have different functions and cellular locations. LRRs correspond ...

sd00033
Location:6 → 29

LRR_RI; leucine-rich repeat [structural motif]
NM_203387.3 → NP_976321.1 ribonuclease inhibitor

See identical proteins and their annotated locations for NP_976321.1

Status: VALIDATED

Description

Transcript Variant: This variant (6) contains a different exon in its 5' UTR, compared to variant 1. All eight variants of this gene encode the same protein.

Source sequence(s)

AC137894, BC011186, DC296330

Consensus CDS

CCDS7697.1

UniProtKB/Swiss-Prot

P13489, Q8IZK8, Q96FD7, Q9BQ80, Q9UDK6

UniProtKB/TrEMBL

A0A140VJT8

Related

ENSP00000346402.2, ENST00000354420.7

Conserved Domains (3) summary

smart00368
Location:397 → 424

LRR_RI; Leucine rich repeat, ribonuclease inhibitor type

cd00116
Location:6 → 325

LRR_RI; Leucine-rich repeats (LRRs), ribonuclease inhibitor (RI)-like subfamily. LRRs are 20-29 residue sequence motifs present in many proteins that participate in protein-protein interactions and have different functions and cellular locations. LRRs correspond ...

sd00033
Location:6 → 29

LRR_RI; leucine-rich repeat [structural motif]
NM_203388.3 → NP_976322.1 ribonuclease inhibitor

See identical proteins and their annotated locations for NP_976322.1

Status: VALIDATED

Description

Transcript Variant: This variant (7) has multiple differences in its 5' UTR, compared to variant 1. All eight variants of this gene encode the same protein.

Source sequence(s)

AC137894, BC011500

Consensus CDS

CCDS7697.1

UniProtKB/Swiss-Prot

P13489, Q8IZK8, Q96FD7, Q9BQ80, Q9UDK6

UniProtKB/TrEMBL

A0A140VJT8

Related

ENSP00000380729.3, ENST00000397604.7

Conserved Domains (3) summary

smart00368
Location:397 → 424

LRR_RI; Leucine rich repeat, ribonuclease inhibitor type

cd00116
Location:6 → 325

LRR_RI; Leucine-rich repeats (LRRs), ribonuclease inhibitor (RI)-like subfamily. LRRs are 20-29 residue sequence motifs present in many proteins that participate in protein-protein interactions and have different functions and cellular locations. LRRs correspond ...

sd00033
Location:6 → 29

LRR_RI; leucine-rich repeat [structural motif]
NM_203389.3 → NP_976323.1 ribonuclease inhibitor

See identical proteins and their annotated locations for NP_976323.1

Status: VALIDATED

Description

Transcript Variant: This variant (8) has multiple differences in its 5' UTR, compared to variant 1. All eight variants of this gene encode the same protein.

Source sequence(s)

AC137894, BC014629

Consensus CDS

CCDS7697.1

UniProtKB/Swiss-Prot

P13489, Q8IZK8, Q96FD7, Q9BQ80, Q9UDK6

UniProtKB/TrEMBL

A0A140VJT8

Related

ENSP00000348515.5, ENST00000356187.9

Conserved Domains (3) summary

smart00368
Location:397 → 424

LRR_RI; Leucine rich repeat, ribonuclease inhibitor type

cd00116
Location:6 → 325

LRR_RI; Leucine-rich repeats (LRRs), ribonuclease inhibitor (RI)-like subfamily. LRRs are 20-29 residue sequence motifs present in many proteins that participate in protein-protein interactions and have different functions and cellular locations. LRRs correspond ...

sd00033
Location:6 → 29

LRR_RI; leucine-rich repeat [structural motif]

RefSeqs of Annotated Genomes: GCF_000001405.40-RS_2023_10

The following sections contain reference sequences that belong to a specific genome build. Explain

Reference GRCh38.p14 Primary Assembly

Genomic

NC_000011.10 Reference GRCh38.p14 Primary Assembly

Range

494515..507242 complement

Download

GenBank, FASTA, Sequence Viewer (Graphics)

mRNA and Protein(s)

XM_011520260.3 → XP_011518562.1 ribonuclease inhibitor isoform X1

See identical proteins and their annotated locations for XP_011518562.1

UniProtKB/Swiss-Prot

P13489, Q8IZK8, Q96FD7, Q9BQ80, Q9UDK6

UniProtKB/TrEMBL

A0A140VJT8

Conserved Domains (3) summary

smart00368
Location:397 → 424

LRR_RI; Leucine rich repeat, ribonuclease inhibitor type

cd00116
Location:6 → 325

LRR_RI; Leucine-rich repeats (LRRs), ribonuclease inhibitor (RI)-like subfamily. LRRs are 20-29 residue sequence motifs present in many proteins that participate in protein-protein interactions and have different functions and cellular locations. LRRs correspond ...

sd00033
Location:6 → 29

LRR_RI; leucine-rich repeat [structural motif]
XM_011520255.2 → XP_011518557.1 ribonuclease inhibitor isoform X1

See identical proteins and their annotated locations for XP_011518557.1

UniProtKB/Swiss-Prot

P13489, Q8IZK8, Q96FD7, Q9BQ80, Q9UDK6

UniProtKB/TrEMBL

A0A140VJT8

Conserved Domains (3) summary

smart00368
Location:397 → 424

LRR_RI; Leucine rich repeat, ribonuclease inhibitor type

cd00116
Location:6 → 325

LRR_RI; Leucine-rich repeats (LRRs), ribonuclease inhibitor (RI)-like subfamily. LRRs are 20-29 residue sequence motifs present in many proteins that participate in protein-protein interactions and have different functions and cellular locations. LRRs correspond ...

sd00033
Location:6 → 29

LRR_RI; leucine-rich repeat [structural motif]
XM_011520261.3 → XP_011518563.1 ribonuclease inhibitor isoform X1

See identical proteins and their annotated locations for XP_011518563.1

UniProtKB/Swiss-Prot

P13489, Q8IZK8, Q96FD7, Q9BQ80, Q9UDK6

UniProtKB/TrEMBL

A0A140VJT8

Conserved Domains (3) summary

smart00368
Location:397 → 424

LRR_RI; Leucine rich repeat, ribonuclease inhibitor type

cd00116
Location:6 → 325

LRR_RI; Leucine-rich repeats (LRRs), ribonuclease inhibitor (RI)-like subfamily. LRRs are 20-29 residue sequence motifs present in many proteins that participate in protein-protein interactions and have different functions and cellular locations. LRRs correspond ...

sd00033
Location:6 → 29

LRR_RI; leucine-rich repeat [structural motif]
XM_017018106.2 → XP_016873595.1 ribonuclease inhibitor isoform X1

UniProtKB/Swiss-Prot

P13489, Q8IZK8, Q96FD7, Q9BQ80, Q9UDK6

UniProtKB/TrEMBL

A0A140VJT8

Conserved Domains (3) summary

smart00368
Location:397 → 424

LRR_RI; Leucine rich repeat, ribonuclease inhibitor type

cd00116
Location:6 → 325

LRR_RI; Leucine-rich repeats (LRRs), ribonuclease inhibitor (RI)-like subfamily. LRRs are 20-29 residue sequence motifs present in many proteins that participate in protein-protein interactions and have different functions and cellular locations. LRRs correspond ...

sd00033
Location:6 → 29

LRR_RI; leucine-rich repeat [structural motif]
XM_011520263.2 → XP_011518565.1 ribonuclease inhibitor isoform X1

See identical proteins and their annotated locations for XP_011518565.1

UniProtKB/Swiss-Prot

P13489, Q8IZK8, Q96FD7, Q9BQ80, Q9UDK6

UniProtKB/TrEMBL

A0A140VJT8

Conserved Domains (3) summary

smart00368
Location:397 → 424

LRR_RI; Leucine rich repeat, ribonuclease inhibitor type

cd00116
Location:6 → 325

LRR_RI; Leucine-rich repeats (LRRs), ribonuclease inhibitor (RI)-like subfamily. LRRs are 20-29 residue sequence motifs present in many proteins that participate in protein-protein interactions and have different functions and cellular locations. LRRs correspond ...

sd00033
Location:6 → 29

LRR_RI; leucine-rich repeat [structural motif]
XM_011520259.4 → XP_011518561.1 ribonuclease inhibitor isoform X1

See identical proteins and their annotated locations for XP_011518561.1

UniProtKB/Swiss-Prot

P13489, Q8IZK8, Q96FD7, Q9BQ80, Q9UDK6

UniProtKB/TrEMBL

A0A140VJT8

Related

ENSP00000433999.1, ENST00000534797.5

Conserved Domains (3) summary

smart00368
Location:397 → 424

LRR_RI; Leucine rich repeat, ribonuclease inhibitor type

cd00116
Location:6 → 325

LRR_RI; Leucine-rich repeats (LRRs), ribonuclease inhibitor (RI)-like subfamily. LRRs are 20-29 residue sequence motifs present in many proteins that participate in protein-protein interactions and have different functions and cellular locations. LRRs correspond ...

sd00033
Location:6 → 29

LRR_RI; leucine-rich repeat [structural motif]