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LONRF2 LON peptidase N-terminal domain and ring finger 2 [ Homo sapiens (human) ]

Gene ID: 164832, updated on 4-Jan-2025

Summary

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Official Symbol
LONRF2provided by HGNC
Official Full Name
LON peptidase N-terminal domain and ring finger 2provided by HGNC
Primary source
HGNC:HGNC:24788
See related
Ensembl:ENSG00000170500 AllianceGenome:HGNC:24788
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
RNF192
Summary
Predicted to enable ubiquitin protein ligase activity. Predicted to be located in cytoplasm. [provided by Alliance of Genome Resources, Jan 2025]
Expression
Biased expression in adrenal (RPKM 18.3), brain (RPKM 16.1) and 10 other tissues See more
Orthologs
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Genomic context

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See LONRF2 in Genome Data Viewer
Location:
2q11.2
Exon count:
14
Annotation release Status Assembly Chr Location
RS_2024_08 current GRCh38.p14 (GCF_000001405.40) 2 NC_000002.12 (100271875..100322501, complement)
RS_2024_08 current T2T-CHM13v2.0 (GCF_009914755.1) 2 NC_060926.1 (100730785..100781377, complement)
RS_2024_09 previous assembly GRCh37.p13 (GCF_000001405.25) 2 NC_000002.11 (100888337..100938963, complement)

Chromosome 2 - NC_000002.12Genomic Context describing neighboring genes Neighboring gene ALF transcription elongation factor 3 Neighboring gene uncharacterized LOC105373504 Neighboring gene MPRA-validated peak3800 silencer Neighboring gene MPRA-validated peak3801 silencer Neighboring gene BRD4-independent group 4 enhancer GRCh37_chr2:100483776-100484975 Neighboring gene H3K27ac hESC enhancer GRCh37_chr2:100521553-100522052 Neighboring gene Sharpr-MPRA regulatory region 9249 Neighboring gene OCT4-NANOG hESC enhancer GRCh37_chr2:100735506-100736449 Neighboring gene fragile site, folic acid type, rare, fra(2)(q11.2) Neighboring gene long intergenic non-protein coding RNA 1104 Neighboring gene ATAC-STARR-seq lymphoblastoid active region 16290 Neighboring gene P300/CBP strongly-dependent group 1 enhancer GRCh37_chr2:100865373-100866572 Neighboring gene H3K4me1 hESC enhancer GRCh37_chr2:100871469-100871968 Neighboring gene ATAC-STARR-seq lymphoblastoid silent region 11813 Neighboring gene H3K27ac-H3K4me1 hESC enhancer GRCh37_chr2:100907393-100908033 Neighboring gene H3K4me1 hESC enhancer GRCh37_chr2:101007732-101008232 Neighboring gene H3K4me1 hESC enhancer GRCh37_chr2:101008233-101008733 Neighboring gene ATAC-STARR-seq lymphoblastoid active region 16291 Neighboring gene ATAC-STARR-seq lymphoblastoid active region 16292 Neighboring gene ATAC-STARR-seq lymphoblastoid active region 16293 Neighboring gene ATAC-STARR-seq lymphoblastoid active region 16294 Neighboring gene H3K4me1 hESC enhancer GRCh37_chr2:101021577-101022078 Neighboring gene OCT4-NANOG-H3K27ac-H3K4me1 hESC enhancer GRCh37_chr2:101028413-101028940 Neighboring gene ATAC-STARR-seq lymphoblastoid silent region 11814 Neighboring gene ATAC-STARR-seq lymphoblastoid silent region 11815 Neighboring gene CYCS pseudogene 7 Neighboring gene carbohydrate sulfotransferase 10

Genomic regions, transcripts, and products

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Go to nucleotide: Graphics FASTA GenBank

Expression

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  • Project title: Tissue-specific circular RNA induction during human fetal development
  • Description: 35 human fetal samples from 6 tissues (3 - 7 replicates per tissue) collected between 10 and 20 weeks gestational time were sequenced using Illumina TruSeq Stranded Total RNA
  • BioProject: PRJNA270632
  • Publication: PMID 26076956
  • Analysis date: Mon Apr 2 22:54:59 2018

Bibliography

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Related articles in PubMed

  1. LONRF2 is a protein quality control ubiquitin ligase whose deficiency causes late-onset neurological deficits. Li D, et al. Nat Aging, 2023 Aug. PMID 37474791
  2. Mapping the interactome of HPV E6 and E7 oncoproteins with the ubiquitin-proteasome system. Poirson J, et al. FEBS J, 2017 Oct. PMID 28786561
  3. CBFβ stabilizes HIV Vif to counteract APOBEC3 at the expense of RUNX1 target gene expression. Kim DY, et al. Mol Cell, 2013 Feb 21. PMID 23333304, Free PMC Article
  4. Coeliac disease-associated risk variants in TNFAIP3 and REL implicate altered NF-kappaB signalling. Trynka G, et al. Gut, 2009 Aug. PMID 19240061
  5. High-resolution photocatalytic mapping of SARS-CoV-2 spike interactions on the cell surface. Datta S, et al. Cell Chem Biol, 2023 Oct 19. PMID 37499664

See all (18) citations in PubMed

GeneRIFs: Gene References Into Functions

What's a GeneRIF?
  1. Observational study of gene-disease association. (HuGE Navigator)
    Title: Coeliac disease-associated risk variants in TNFAIP3 and REL implicate altered NF-kappaB signalling.
Submit: New GeneRIF Correction

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

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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Protein interactions

Protein Gene Interaction Pubs
Vif vif HIV-1 Vif downregulates the expression of LON peptidase N-terminal domain and ring finger 2 (LONRF2) in Vif-expression T cells 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

Homology

Clone Names

  • FLJ45273, MGC126711, MGC126713

Gene Ontology Provided by GOA

Function Evidence Code Pubs
enables metal ion binding IEA
Inferred from Electronic Annotation
more info
  
enables protein binding IPI
Inferred from Physical Interaction
more info
 PubMed 
Component Evidence Code Pubs
located_in cytoplasm IEA
Inferred from Electronic Annotation
more info
  

General protein information

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Preferred Names
LON peptidase N-terminal domain and RING finger protein 2
Names
RING finger protein 192
neuroblastoma apoptosis-related protease

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)

  1. NM_001371783.1 → NP_001358712.1  LON peptidase N-terminal domain and RING finger protein 2 isoform 2

    Status: VALIDATED

    Source sequence(s)
    AC012493, AC104782
    Consensus CDS
    CCDS92818.1
    UniProtKB/TrEMBL
    B7ZKY9
    Conserved Domains (4) summary
    COG5574
    Location:137 → 246
    PEX10; RING-finger-containing E3 ubiquitin ligase [Posttranslational modification, protein turnover, chaperones]
    sd00006
    Location:21 → 51
    TPR; TPR repeat [structural motif]
    cd16514
    Location:203 → 244
    RING2-HC_LONFs; RING finger 2, HC subclass, found in the LON peptidase N-terminal domain and RING finger proteins family
    pfam02190
    Location:295 → 495
    LON_substr_bdg; ATP-dependent protease La (LON) substrate-binding domain

  2. NM_198461.4 → NP_940863.3  LON peptidase N-terminal domain and RING finger protein 2 isoform 1

    See identical proteins and their annotated locations for NP_940863.3

    Status: VALIDATED

    Source sequence(s)
    AC012493, AC104782
    Consensus CDS
    CCDS2046.2
    UniProtKB/Swiss-Prot
    B9A006, Q1L5Z9, Q6ZSR4
    Related
    ENSP00000377086.3, ENST00000393437.8
    Conserved Domains (6) summary
    COG5574
    Location:380 → 489
    PEX10; RING-finger-containing E3 ubiquitin ligase [Posttranslational modification, protein turnover, chaperones]
    sd00006
    Location:200 → 225
    TPR; TPR repeat [structural motif]
    cd16513
    Location:143 → 174
    RING1-HC_LONFs; RING finger 1, HC subclass, found in the LON peptidase N-terminal domain and RING finger proteins family
    cd16514
    Location:446 → 487
    RING2-HC_LONFs; RING finger 2, HC subclass, found in the LON peptidase N-terminal domain and RING finger proteins family
    pfam02190
    Location:538 → 738
    LON_substr_bdg; ATP-dependent protease La (LON) substrate-binding domain
    pfam13432
    Location:27 → 92
    TPR_16; Tetratricopeptide repeat

RefSeqs of Annotated Genomes: GCF_000001405.40-RS_2024_08

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

Reference GRCh38.p14 Primary Assembly

Genomic

  1. NC_000002.12 Reference GRCh38.p14 Primary Assembly

    Range
    100271875..100322501 complement
    Download
    GenBank, FASTA, Sequence Viewer (Graphics)

mRNA and Protein(s)

  1. XM_047443537.1 → XP_047299493.1  LON peptidase N-terminal domain and RING finger protein 2 isoform X1

    UniProtKB/TrEMBL
    B7ZKY9
    Related
    ENSP00000386823.1, ENST00000409647.1

  2. XM_047443538.1 → XP_047299494.1  LON peptidase N-terminal domain and RING finger protein 2 isoform X2

Alternate T2T-CHM13v2.0

Genomic

  1. NC_060926.1 Alternate T2T-CHM13v2.0

    Range
    100730785..100781377 complement
    Download
    GenBank, FASTA, Sequence Viewer (Graphics)
Nucleotide Protein
Heading Accession and Version
Protein Accession Links
GenPept Link UniProtKB Link
Q1L5Z9.3 GenPept UniProtKB/Swiss-Prot:Q1L5Z9

Gene LinkOut

The following LinkOut resources are supplied by external providers. These providers are responsible for maintaining the links.

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