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Gene

PRORP

protein only RNase P catalytic subunit

Summary

Cytogenetic location
14q13.2
Coordinates
Chromosome 14: 35,122,552–35,277,622 (GRCh38)
Exons
8 exons across 155.1 kb
Protein
583 amino acids
Also known as
MRPP3
Previous symbols
KIAA0391
MANE Select transcript
ENST00000534898.9NM_014672.4
Locus type
Protein-coding
  • Very high
  • Confident
  • Low
  • Very low

Curated report relevant to this gene

  • Primary Mitochondrial Disease

    • Combined Oxidative Phosphorylation Deficiency

      Disorders, usually caused by nuclear genes involved in mitochondrial protein production, that reduce several respiratory chain complexes at once and often cause severe multisystem disease in childhood.

      • 1 GenCC submitter rates it definitive or strong
      Actionability

      Knowing can end a diagnostic search and guide surveillance and supportive care.

How PRORP is laid out

  • Coding sequence
  • Untranslated region
  • Intron
  • Direction of transcription
  • Known disease-causing sites
  • Stretches short reads find hard
Chromosome 14, with a bracket where PRORP sits. PRORP and 10 genes near it on chromosome 14, from 34,700,087 to 35,700,086.Chromosome 1414q13.2p13p12p11.2p11.1q11.1q11.2q12q13.1q13.2q13.3q21.1q21.2q21.3q22.1q22.2q22.3q23.1q23.2q23.3q24.1q24.2q24.3q31.1q31.2q31.3q32.11q32.12q32.13q32.2q32.31q32.32q32.3334.8 Mb35 Mb35.2 Mb35.4 Mb35.6 MbCFL2: 34,707,001–34,714,946, reverse strandCFL2BAZ1A: 34,752,028–34,875,647, reverse strandBAZ1ASRP54: 34,981,957–35,029,686, forward strandSRP54FAM177A1: 35,045,117–35,116,630, forward strandFAM177A1PPP2R3C: 35,085,467–35,122,547, reverse strandPPP2R3C: 35,122,549–35,317,474, forward strandPRORP: 35,122,552–35,277,622, forward strandPRORPPSMA6: 35,278,596–35,318,734, forward strandPSMA6NFKBIA: 35,401,079–35,404,749, reverse strandNFKBIAINSM2: 35,534,164–35,537,054, forward strandINSM2RALGAPA1: 35,538,352–35,809,325, reverse strandRALGAPA1Diagram of PRORP: 8 exons on the forward strand of chromosome 14Exon 6: 1 known disease-causing siteExon 7: 1 known disease-causing siteExon 1: 35,122,552–35,122,635, 84 basesExon 2: 35,122,952–35,124,231, 1,280 basesExon 3: 35,126,735–35,126,782, 48 basesExon 4: 35,127,479–35,127,611, 133 basesExon 5: 35,180,670–35,180,777, 108 basesExon 6: 35,266,727–35,266,875, 149 basesExon 7: 35,270,401–35,270,596, 196 basesExon 8: 35,273,435–35,277,622, 4,188 bases1585′3′

Exons of the gene's MANE Select transcript (release v1.5), from NCBI and EMBL-EBI, on the GRCh38 reference genome.

Chromosome bands from UCSC's GRCh38 ideogram (2022-10-29).

Nearby genes from Ensembl release 116, on GRCh38.

Exons make up 6.2 kb of the gene's 155.1 kb; 1,752 of those bases code for the protein.

Sequencing PRORP

1Low

How hard PRORP is to sequence with short reads, out of 100

  • Mappability0%
  • Homology0%
  • Repeats3.2%
  • GC content0.8%
  • Reference coverage0%

2

positions in PRORP where ClinVar classes a change as pathogenic or likely pathogenic

None of them falls in a region short reads find hard.

A whole genome covers all 155.1 kb of PRORP, introns included, not only the 6.2 kb of exons.

Genes linked to the same conditions

Traits linked to PRORP

The Human Phenotype Ontology lists these as features of the rare conditions PRORP can cause. Each trait's page lists the other genes linked to it.

External links

Where this comes from

  • HGNC: the gene's symbol, name, other symbols and location.
  • MANE Select v1.5, from NCBI and EMBL-EBI: the transcript, its exons and coding sequence.
  • GenCC and ClinGen, released 2026-10-17: which conditions the gene is linked to, and how strongly.
  • The ACMG secondary findings list, SF v3.2.
  • ClinVar and the GIAB stratifications: known disease-causing sites, and the regions short reads find hard.
  • The Genome in a Bottle genome stratifications, from NIST: the sequencing difficulty score.
  • The Human Phenotype Ontology, release 2026-09-01: the traits linked to the gene. Find out more at hpo.jax.org.
  • Data release 2026.10.17-8666dff2.

Cite this page

APA

Dante Labs. (n.d.). PRORP gene: protein only RNase P catalytic subunit. Retrieved 17 October 2026, from https://dantelabs.com/genes/prorp

Vancouver

Dante Labs. PRORP gene: protein only RNase P catalytic subunit [Internet]. [cited 17 October 2026]. Available from: https://dantelabs.com/genes/prorp

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