U.S. flag

An official website of the United States government

Format

Send to:

Choose Destination

PRORP protein only RNase P catalytic subunit [ Homo sapiens (human) ]

Gene ID: 9692, updated on 23-Nov-2023

Summary

Official Symbol
PRORPprovided by HGNC
Official Full Name
protein only RNase P catalytic subunitprovided by HGNC
Primary source
HGNC:HGNC:19958
See related
Ensembl:ENSG00000100890 MIM:609947; AllianceGenome:HGNC:19958
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
MRPP3; COXPD54; KIAA0391
Summary
Enables ribonuclease P activity. Involved in mitochondrial tRNA 5'-end processing. Located in mitochondrion and nucleoplasm. Part of mitochondrial ribonuclease P complex. [provided by Alliance of Genome Resources, Apr 2022]
Expression
Ubiquitous expression in testis (RPKM 10.2), kidney (RPKM 8.4) and 25 other tissues See more
Orthologs
NEW
Try the new Gene table
Try the new Transcript table

Genomic context

See PRORP in Genome Data Viewer
Location:
14q13.2
Exon count:
9
Annotation release Status Assembly Chr Location
RS_2023_10 current GRCh38.p14 (GCF_000001405.40) 14 NC_000014.9 (35121839..35277622)
RS_2023_10 current T2T-CHM13v2.0 (GCF_009914755.1) 14 NC_060938.1 (29318793..29465226)
105.20220307 previous assembly GRCh37.p13 (GCF_000001405.25) 14 NC_000014.8 (35591758..35746828)

Chromosome 14 - NC_000014.9Genomic Context describing neighboring genes Neighboring gene H3K27ac hESC enhancer GRCh37_chr14:35451517-35452218 Neighboring gene ATAC-STARR-seq lymphoblastoid active region 8259 Neighboring gene signal recognition particle 54 Neighboring gene ATAC-STARR-seq lymphoblastoid active region 8260 Neighboring gene ATAC-STARR-seq lymphoblastoid silent region 5673 Neighboring gene ATAC-STARR-seq lymphoblastoid active region 8261 Neighboring gene ATAC-STARR-seq lymphoblastoid active region 8262 Neighboring gene family with sequence similarity 177 member A1 Neighboring gene uncharacterized LOC101927178 Neighboring gene ReSE screen-validated silencer GRCh37_chr14:35571935-35572102 Neighboring gene NANOG-H3K27ac hESC enhancer GRCh37_chr14:35581755-35582536 Neighboring gene protein phosphatase 2 regulatory subunit B''gamma Neighboring gene MED14-independent group 3 enhancer GRCh37_chr14:35590400-35591599 Neighboring gene NANOG-H3K27ac-H3K4me1 hESC enhancer GRCh37_chr14:35591739-35592658 Neighboring gene PRORP-PSMA6 readthrough Neighboring gene H3K27ac hESC enhancer GRCh37_chr14:35622091-35622592 Neighboring gene Sharpr-MPRA regulatory region 10139 Neighboring gene ribosomal protein L23a pseudogene 70 Neighboring gene septin 7 pseudogene 1 Neighboring gene mitochondrial ribosomal protein L57 pseudogene 8 Neighboring gene divergent-paired related homeobox pseudogene 3 Neighboring gene ribosomal protein L7a pseudogene 3 Neighboring gene ATAC-STARR-seq lymphoblastoid active region 8266 Neighboring gene ATAC-STARR-seq lymphoblastoid active region 8267 Neighboring gene ATAC-STARR-seq lymphoblastoid active region 8268 Neighboring gene H3K27ac hESC enhancer GRCh37_chr14:35761289-35761875 Neighboring gene ATAC-STARR-seq lymphoblastoid active region 8271 Neighboring gene ATAC-STARR-seq lymphoblastoid active region 8272 Neighboring gene ribosomal protein L9 pseudogene 3 Neighboring gene proteasome 20S subunit alpha 6 Neighboring gene Sharpr-MPRA regulatory region 8744 Neighboring gene H3K27ac-H3K4me1 hESC enhancer GRCh37_chr14:35801329-35802250 Neighboring gene NANOG-H3K4me1 hESC enhancer GRCh37_chr14:35802261-35802870 Neighboring gene H3K27ac-H3K4me1 hESC enhancer GRCh37_chr14:35805755-35806276 Neighboring gene ATAC-STARR-seq lymphoblastoid active region 8273 Neighboring gene H3K4me1 hESC enhancer GRCh37_chr14:35811147-35812003 Neighboring gene H3K27ac-H3K4me1 hESC enhancer GRCh37_chr14:35816286-35816830 Neighboring gene ATAC-STARR-seq lymphoblastoid active region 8274 Neighboring gene ATAC-STARR-seq lymphoblastoid active region 8275 Neighboring gene H3K27ac-H3K4me1 hESC enhancer GRCh37_chr14:35836533-35837122 Neighboring gene H3K4me1 hESC enhancer GRCh37_chr14:35837123-35837710 Neighboring gene H3K27ac-H3K4me1 hESC enhancer GRCh37_chr14:35837711-35838299 Neighboring gene CRISPRi-validated cis-regulatory element chr14.550 Neighboring gene Sharpr-MPRA regulatory region 4470 Neighboring gene PSMA6-RPLP0P3 intergenic CAGE-defined low expression enhancer Neighboring gene ribosomal protein lateral stalk subunit P0 pseudogene 3

Genomic regions, transcripts, and products

Bibliography

GeneRIFs: Gene References Into Functions

What's a GeneRIF?

Phenotypes

Associated conditions

Description Tests
Combined oxidative phosphorylation deficiency 54
MedGen: C5676912 OMIM: 619737 GeneReviews: Not available
not available

EBI GWAS Catalog

Description
Genome-wide association analysis identifies three psoriasis susceptibility loci.
EBI GWAS Catalog

Pathways from PubChem

Interactions

Products Interactant Other Gene Complex Source Pubs Description

General gene information

Markers

Readthrough PRORP-PSMA6

Readthrough gene: PRORP-PSMA6, Included gene: PSMA6

Gene Ontology Provided by GOA

Function Evidence Code Pubs
enables metal ion binding IEA
Inferred from Electronic Annotation
more info
 
enables ribonuclease P activity IBA
Inferred from Biological aspect of Ancestor
more info
 
enables ribonuclease P activity IDA
Inferred from Direct Assay
more info
PubMed 
Process Evidence Code Pubs
involved_in mitochondrial tRNA 5'-end processing IBA
Inferred from Biological aspect of Ancestor
more info
 
involved_in mitochondrial tRNA 5'-end processing IDA
Inferred from Direct Assay
more info
PubMed 
involved_in tRNA 5'-leader removal IBA
Inferred from Biological aspect of Ancestor
more info
 
Component Evidence Code Pubs
located_in mitochondrial matrix TAS
Traceable Author Statement
more info
 
located_in mitochondrial nucleoid IEA
Inferred from Electronic Annotation
more info
 
part_of mitochondrial ribonuclease P complex IBA
Inferred from Biological aspect of Ancestor
more info
 
part_of mitochondrial ribonuclease P complex IDA
Inferred from Direct Assay
more info
PubMed 
part_of mitochondrial ribonuclease P complex IPI
Inferred from Physical Interaction
more info
PubMed 
part_of mitochondrial ribonuclease P complex TAS
Traceable Author Statement
more info
PubMed 
located_in mitochondrion IDA
Inferred from Direct Assay
more info
PubMed 
located_in nucleoplasm IDA
Inferred from Direct Assay
more info
 

General protein information

Preferred Names
mitochondrial ribonuclease P catalytic subunit
Names
mitochondrial RNase P protein 3
mitochondrial RNase P subunit 3
mitochondrial ribonuclease P protein 3
proteinaceous RNase P
NP_001243607.1
NP_001243608.1
NP_001243609.1
NP_001243610.1
NP_001401432.1
NP_055487.2

NCBI Reference Sequences (RefSeq)

NEW Try the new Transcript table

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_001256678.2NP_001243607.1  mitochondrial ribonuclease P catalytic subunit isoform 2

    See identical proteins and their annotated locations for NP_001243607.1

    Status: VALIDATED

    Description
    Transcript Variant: This variant (2) lacks an exon in the coding region, but maintains the reading frame, compared to variant 1. The encoded isoform (2) is shorter than isoform 1.
    Source sequence(s)
    AB002389, AK304066, AL121594, DA459368
    Consensus CDS
    CCDS58312.1
    UniProtKB/Swiss-Prot
    O15091
    Related
    ENSP00000250377.8, ENST00000250377.11
    Conserved Domains (2) summary
    sd00004
    Location:174204
    PPR; PPR repeat [structural motif]
    pfam16953
    Location:328562
    PRORP; Protein-only RNase P
  2. NM_001256679.2NP_001243608.1  mitochondrial ribonuclease P catalytic subunit isoform 3

    Status: VALIDATED

    Description
    Transcript Variant: This variant (3) differs in the 5' UTR, lacks a portion of the 5' coding region and initiates translation at a downstream, in-frame start codon, compared to variant 1. The encoded isoform (3) has a shorter N-terminus, compared to isoform 1.
    Source sequence(s)
    AI431599, AK301931, AL121594, BP373829
    Consensus CDS
    CCDS58313.1
    UniProtKB/Swiss-Prot
    O15091
    Related
    ENSP00000474620.1, ENST00000604948.5
    Conserved Domains (2) summary
    sd00004
    Location:79109
    PPR; PPR repeat [structural motif]
    pfam16953
    Location:247483
    PRORP; Protein-only RNase P
  3. NM_001256680.2NP_001243609.1  mitochondrial ribonuclease P catalytic subunit isoform 4

    See identical proteins and their annotated locations for NP_001243609.1

    Status: VALIDATED

    Description
    Transcript Variant: This variant (4) differs in the 5' UTR, lacks a portion of the 5' coding region and initiates translation at a downstream, in-frame start codon, compared to variant 1. Variants 4 and 5 encode the same isoform (4), which has a shorter N-terminus compared to isoform 1.
    Source sequence(s)
    AA838769, AL121594, BX161487, DC404874
    Consensus CDS
    CCDS58314.1
    UniProtKB/TrEMBL
    A0A0A0MTQ0
    Related
    ENSP00000474299.1, ENST00000605870.5
    Conserved Domains (1) summary
    pfam16953
    Location:1206
    PRORP; Protein-only RNase P
  4. NM_001256681.2NP_001243610.1  mitochondrial ribonuclease P catalytic subunit isoform 4

    See identical proteins and their annotated locations for NP_001243610.1

    Status: VALIDATED

    Description
    Transcript Variant: This variant (5) differs in the 5' UTR, lacks a portion of the 5' coding region and initiates translation at a downstream, in-frame start codon, compared to variant 1. Variants 4 and 5 encode the same isoform (4), which has a shorter N-terminus compared to isoform 1.
    Source sequence(s)
    AI431599, AL121594, BQ063741, DC404874, DT217089
    Consensus CDS
    CCDS58314.1
    UniProtKB/TrEMBL
    A0A0A0MTQ0
    Related
    ENSP00000324697.9, ENST00000321130.14
    Conserved Domains (1) summary
    pfam16953
    Location:1206
    PRORP; Protein-only RNase P
  5. NM_001414503.1NP_001401432.1  mitochondrial ribonuclease P catalytic subunit isoform 1 precursor

    Status: VALIDATED

    Source sequence(s)
    AL121594
    UniProtKB/Swiss-Prot
    B4DXD9, B4E0S8, B4E211, C4AM93, D3DS99, D3DSA1, O15091, Q86SZ4, Q86YB5, Q8N5L5
  6. NM_014672.4NP_055487.2  mitochondrial ribonuclease P catalytic subunit isoform 1 precursor

    See identical proteins and their annotated locations for NP_055487.2

    Status: VALIDATED

    Description
    Transcript Variant: This variant (1) encodes the longest isoform (1). Variants 1 and 6 both encode the same isoform (1).
    Source sequence(s)
    AA838769, AL121594, BX161394, DA459368
    Consensus CDS
    CCDS32063.1
    UniProtKB/Swiss-Prot
    B4DXD9, B4E0S8, B4E211, C4AM93, D3DS99, D3DSA1, O15091, Q86SZ4, Q86YB5, Q8N5L5
    Related
    ENSP00000440915.2, ENST00000534898.9
    Conserved Domains (2) summary
    sd00004
    Location:174204
    PPR; PPR repeat [structural motif]
    pfam16953
    Location:342578
    PRORP; Protein-only RNase P

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

  1. NC_000014.9 Reference GRCh38.p14 Primary Assembly

    Range
    35121839..35277622
    Download
    GenBank, FASTA, Sequence Viewer (Graphics)

Alternate T2T-CHM13v2.0

Genomic

  1. NC_060938.1 Alternate T2T-CHM13v2.0

    Range
    29318793..29465226
    Download
    GenBank, FASTA, Sequence Viewer (Graphics)