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Gene

LONP1

lon peptidase 1, mitochondrial

Summary

Cytogenetic location
19p13.2
Coordinates
Chromosome 19: 5,691,834–5,720,146 (GRCh38)
Exons
18 exons across 28.3 kb
Protein
959 amino acids
Also known as
LonHS, hLON, PIM1
Previous symbols
PRSS15
MANE Select transcript
ENST00000360614.8NM_004793.4
Locus type
gene with protein product
  • Very high
  • Confident
  • Low
  • Very low

Reference genomeGRCh38

Positions on gene and variant pages follow this choice, in this browser.

Curated reports relevant to this gene

No Genome Test report reads LONP1 yet. The test sequences your whole genome, so your data includes it all the same.

How LONP1 is laid out

  • Coding sequence
  • Untranslated region
  • Intron
  • Direction of transcription
  • Known disease-causing sites
  • Stretches short reads find hard
Chromosome 19, with a bracket where LONP1 sits. LONP1 and 24 genes near it on chromosome 19, from 5,205,990 to 6,205,989.Chromosome 1919p13.2p13.3p13.2p13.13p13.12p13.11p12p11q11q12q13.11q13.12q13.13q13.2q13.31q13.32q13.33q13.41q13.42q13.435.4 Mb5.6 Mb5.8 Mb6 Mb6.2 MbPTPRS: 5,158,495–5,340,941, reverse strandPTPRSZNRF4: 5,455,417–5,456,856, forward strandZNRF4TINCR: 5,552,723–5,578,349, reverse strandTINCRSAFB2: 5,586,981–5,624,046, reverse strandSAFB2SAFB: 5,622,963–5,668,478, forward strandSAFBRPL36: 5,674,947–5,691,881, forward strandRPL36MICOS13: 5,678,414–5,680,940, reverse strandMICOS13HSD11B1L: 5,680,604–5,688,524, forward strandHSD11B1LLONP1: 5,691,834–5,720,146, reverse strandLONP1CATSPERD: 5,720,637–5,778,734, forward strandCATSPERDPRR22: 5,782,960–5,784,752, reverse strandPRR22: 5,784,005–5,785,619, reverse strandDUS3L: 5,784,832–5,791,225, reverse strandDUS3LNRTN: 5,805,053–5,828,324, forward strandNRTNFUT6: 5,830,361–5,839,722, reverse strandFUT6FUT3: 5,842,257–5,851,535, reverse strandFUT3FUT5: 5,865,826–5,870,540, reverse strandFUT5: 5,866,171–5,903,787, reverse strandNDUFA11: 5,891,207–5,904,006, reverse strandNDUFA11VMAC: 5,904,723–5,910,853, forward strandVMAC: 5,904,841–5,914,707, forward strandCAPS: 5,912,339–5,916,211, forward strandRANBP3: 5,916,138–5,978,288, reverse strandRFX2: 5,993,164–6,199,572, reverse strandRFX2ACSBG2: 6,135,247–6,193,094, forward strandACSBG2Diagram of LONP1: 18 exons on the reverse strand of chromosome 19Exon 18: 1 known disease-causing siteExon 15: 1 known disease-causing siteExon 14: 2 known disease-causing sitesExon 13: 1 known disease-causing siteExon 8: 2 known disease-causing sitesExon 18: 5,691,834–5,692,208, 375 basesExon 17: 5,693,298–5,693,462, 165 basesExon 16: 5,693,552–5,693,769, 218 basesExon 15: 5,694,387–5,694,552, 166 basesExon 14: 5,694,761–5,694,901, 141 basesExon 13: 5,696,054–5,696,170, 117 basesExon 12: 5,696,249–5,696,371, 123 basesExon 11: 5,696,670–5,696,757, 88 basesExon 10: 5,699,027–5,699,205, 179 basesExon 9: 5,700,789–5,700,927, 139 basesExon 8: 5,705,772–5,705,992, 221 basesExon 7: 5,707,060–5,707,143, 84 basesExon 6: 5,707,697–5,707,826, 130 basesExon 5: 5,708,342–5,708,403, 62 basesExon 4: 5,711,771–5,712,002, 232 basesExon 3: 5,713,134–5,713,253, 120 basesExon 2: 5,714,183–5,714,271, 89 basesExon 1: 5,719,704–5,720,146, 443 bases1816141210864215′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 3.1 kb of the gene's 28.3 kb; 2,880 of those bases code for the protein.

How a genome test reads LONP1

7

positions in LONP1 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 28.3 kb of LONP1, introns included, not only the 3.1 kb of exons.

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.
  • ClinVar and the GIAB stratifications: known disease-causing sites, and the regions short reads find hard.
  • The Human Phenotype Ontology, release 2026-09-01: the traits linked to the gene. Find out more at hpo.jax.org.
  • Data release 2026.10.16-c6eea6bf.

Cite this page

APA

Dante Labs. (n.d.). LONP1 gene: lon peptidase 1, mitochondrial. Retrieved 16 October 2026, from https://dantelabs.com/genes/lonp1

Vancouver

Dante Labs. LONP1 gene: lon peptidase 1, mitochondrial [Internet]. [cited 16 October 2026]. Available from: https://dantelabs.com/genes/lonp1

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