Seroatlas · Human Serome Atlas

PRORP

Mitochondrial ribonuclease P catalytic subunit

Also known as: KIAA0391, MRPP3, MRPP3_HUMAN

Cross-references: UniProt · Ensembl · Human Protein Atlas · GeneCards · NCBI Gene

Protein identityUniProt · HPA

UniProt accession: O15091
Gene: PRORP
Ensembl: ENSG00000100890
Chromosome: 14
Canonical length: 583 aa
Protein class: Disease related genes, Enzymes, Human disease related genes, Potential drug targets, Predicted intracellular proteins
Subcellular location: Nucleoplasm,Mitochondria

OverviewNCBI Gene

Enables ribonuclease P activity. Involved in mitochondrial tRNA 5'-end processing. Located in mitochondrion and nucleoplasm. Part of mitochondrial ribonuclease P complex. Implicated in combined oxidative phosphorylation deficiency 54. [provided by Alliance of Genome Resources, Jul 2025]

Canonical amino-acid sequenceUniProt

583 residues, UniProt reviewed canonical sequence.

>O15091|PRORP
     1  MTFYLFGIRS FPKLWKSPYL GLGPGHSYVS LFLADRCGIR NQQRLFSLKT MSPQNTKATN
    61  LIAKARYLRK DEGSNKQVYS VPHFFLAGAA KERSQMNSQT EDHALAPVRN TIQLPTQPLN
   121  SEEWDKLKED LKENTGKTSF ESWIISQMAG CHSSIDVAKS LLAWVAAKNN GIVSYDLLVK
   181  YLYLCVFHMQ TSEVIDVFEI MKARYKTLEP RGYSLLIRGL IHSDRWREAL LLLEDIKKVI
   241  TPSKKNYNDC IQGALLHQDV NTAWNLYQEL LGHDIVPMLE TLKAFFDFGK DIKDDNYSNK
   301  LLDILSYLRN NQLYPGESFA HSIKTWFESV PGKQWKGQFT TVRKSGQCSG CGKTIESIQL
   361  SPEEYECLKG KIMRDVIDGG DQYRKTTPQE LKRFENFIKS RPPFDVVIDG LNVAKMFPKV
   421  RESQLLLNVV SQLAKRNLRL LVLGRKHMLR RSSQWSRDEM EEVQKQASCF FADDISEDDP
   481  FLLYATLHSG NHCRFITRDL MRDHKACLPD AKTQRLFFKW QQGHQLAIVN RFPGSKLTFQ
   541  RILSYDTVVQ TTGDSWHIPY DEDLVERCSC EVPTKWLCLH QKT

LocalizationUniProt · AlphaFold · HPA

Whether an antibody against PRORP can act on the native protein depends on physical access: surface and secreted proteins are reachable by circulating antibodies, intracellular proteins usually are not.

Antibody reachability: Intracellular
Secreted: No
Transmembrane segments: 0
Mean surface accessibility (rSASA): 0.34
Highest tissue expression: 29 nTPM

Expression across tissuesHPA

Tissue

thymus: 29 nTPM
kidney: 23 nTPM
liver: 23 nTPM
choroid plexus: 22 nTPM
tonsil: 21 nTPM
lymph node: 20 nTPM

Single-cell type

choroid plexus epithelial cells: 114 nCPM
epicardial cells: 84 nCPM
oligodendrocytes: 69 nCPM
microglia: 63 nCPM
ependymal cells: 62 nCPM
oligodendrocyte progenitor cells: 62 nCPM

Immune cell

T-reg: 56 nTPM
memory CD4 T-cell: 47 nTPM
MAIT T-cell: 44 nTPM
naive CD4 T-cell: 42 nTPM
memory CD8 T-cell: 33 nTPM
basophil: 31 nTPM

Brain region

choroid plexus: 28 nTPM
white matter: 23 nTPM
medulla oblongata: 22 nTPM
thalamus: 21 nTPM
pons: 20 nTPM
cerebellum: 19 nTPM

DiseaseUniProt · ClinVar · IEDB · PubMed

Four sources answering four different questions about PRORP.

Disease | AllUniProt

Conditions PRORP is implicated in, by any mechanism.

Combined oxidative phosphorylation deficiency 54 (COXPD54) MIM:619737

Disease | GeneticClinVar

8 pathogenic / likely-pathogenic of 98 ClinVar records.

Conditions with pathogenic or likely-pathogenic variants.

Combined oxidative phosphorylation deficiency 54
Childhood onset sensorineural hearing impairment
6 conditions
Lactic acidosis
Leukoencephalopathy

Genetic constraint and essentialitygnomAD · DepMap

Does the body need this protein intact? Low LOEUF or a strong DepMap dependency means loss or blockade of the protein is likely to be felt.

gnomAD LOEUF (loss-of-function intolerance): 1.22
gnomAD pLI: 0
DepMap mean gene effect: -0.42
DepMap dependency class: selective

Cancer expressionTCGA

Across TCGA tumor cohorts, this protein is over-expressed in roughly 6% of surveyed tumor types (aggregate summary; per-cohort expression, alteration, and survival load in the interactive view).

OntologyGO

Biological processes

Molecular functions

Cellular components

Protein domainsUniProt · Pfam · InterPro

Tetratricopeptide-like helical domain superfamily
Protein-only RNase P, C-terminal
Mitochondrial ribonuclease P catalytic subunit, PIN domain
Protein-only RNase P

KeywordsUniProt

Antibody and autoantibody relevanceSeroatlas analysis

Seroatlas reads PRORP as an antibody target. Whether an autoantibody or antibody against PRORP could matter depends on whether native PRORP is physically reachable, whether the body needs it intact, and whether it acts in a disease-relevant tissue.

PRORP is annotated as predominantly intracellular. Intracellular proteins are common autoantibody markers, becoming visible to the immune system after cell injury or altered processing, but are usually markers of disease rather than direct drivers.

Annotation status

The present source text does not explicitly label PRORP as an autoantigen. Seroatlas presents hypothesis context only and does not manufacture a known-serology claim.

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