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Gene

MIPEP

mitochondrial intermediate peptidase

Summary

Cytogenetic location
13q12.12
Coordinates
Chromosome 13: 23,730,189–23,889,400 (GRCh38)
Exons
19 exons across 159.2 kb
Protein
713 amino acids
Inheritance
Autosomal recessive
Also known as
MIP
MANE Select transcript
ENST00000382172.4NM_005932.4
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

  • Left Ventricular Noncompaction

    • MIPEP-Related Left Ventricular Non-Compaction SyndromeAutosomal recessive

      A severe condition caused by changes in MIPEP that combines left ventricular non-compaction or other cardiomyopathy with seizures, low muscle tone, cataracts and developmental delay.

      • 3 GenCC submitters rate it definitive or strong

  • 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.

      • 3 GenCC submitters rate it definitive or strong
      What knowing changes

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

Inheritance

Autosomal recessive
It takes a changed copy from each parent. With one, you are a carrier and usually unaffected; when both parents are carriers, each child has a one-in-four chance of inheriting two.

How MIPEP is laid out

  • Coding sequence
  • Untranslated region
  • Intron
  • Direction of transcription
  • Known disease-causing sites
  • Stretches short reads find hard
Chromosome 13, with a bracket where MIPEP sits. MIPEP and 7 genes near it on chromosome 13, from 23,309,795 to 24,309,794.Chromosome 1313q12.12p13p12p11.2p11.1q11q12.11q12.12q12.13q12.2q12.3q13.1q13.2q13.3q14.11q14.12q14.13q14.2q14.3q21.1q21.2q21.31q21.32q21.33q22.1q22.2q22.3q31.1q31.2q31.3q32.1q32.2q32.3q33.1q33.2q33.3q3423.4 Mb23.6 Mb23.8 Mb24 Mb24.2 MbSGCG: 23,160,011–23,325,958, forward strandSGCGSACS: 23,288,689–23,433,763, reverse strandSACSTNFRSF19: 23,570,194–23,676,105, forward strandTNFRSF19MIPEP: 23,730,189–23,889,400, reverse strandMIPEPC1QTNF9B: 23,891,098–23,897,502, reverse strandC1QTNF9BSPATA13: 23,979,677–24,307,074, forward strandSPATA13: 23,979,700–24,321,031, forward strandC1QTNF9: 24,307,166–24,322,535, forward strandC1QTNF9Diagram of MIPEP: 19 exons on the reverse strand of chromosome 13Exon 17: 2 known disease-causing sitesExon 16: 2 known disease-causing sitesExon 13: 1 known disease-causing siteExon 12: 1 known disease-causing siteExon 11: 1 known disease-causing siteExon 10: 1 known disease-causing siteExon 9: 1 known disease-causing siteExon 7: 2 known disease-causing sitesExon 5: 1 known disease-causing siteExon 2: 1 known disease-causing siteExon 19: 23,730,189–23,730,445, 257 basesExon 18: 23,756,545–23,756,618, 74 basesExon 17: 23,760,096–23,760,217, 122 basesExon 16: 23,805,950–23,806,069, 120 basesExon 15: 23,809,850–23,809,924, 75 basesExon 14: 23,836,240–23,836,349, 110 basesExon 13: 23,837,552–23,837,756, 205 basesExon 12: 23,839,649–23,839,726, 78 basesExon 11: 23,841,335–23,841,488, 154 basesExon 10: 23,858,860–23,858,912, 53 basesExon 9: 23,862,302–23,862,362, 61 basesExon 8: 23,864,141–23,864,189, 49 basesExon 7: 23,869,292–23,869,448, 157 basesExon 6: 23,870,013–23,870,195, 183 basesExon 5: 23,874,846–23,874,909, 64 basesExon 4: 23,879,268–23,879,354, 87 basesExon 3: 23,881,699–23,881,787, 89 basesExon 2: 23,886,333–23,886,506, 174 basesExon 1: 23,889,132–23,889,400, 269 bases191510515′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 2.4 kb of the gene's 159.2 kb; 2,142 of those bases code for the protein.

How a genome test reads MIPEP

16

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

1 of them (6.3%) falls in a region short reads find hard.

  • Segmental duplications (1 site)Copies of at least 1 kb sharing at least 90% identity with another part of the genome (paralogues, pseudogenes); reads may align to the wrong copy.

A whole genome covers all 159.2 kb of MIPEP, introns included, not only the 2.4 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.
  • GenCC and ClinGen, released 2026-10-16: 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 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.). MIPEP gene: mitochondrial intermediate peptidase. Retrieved 17 October 2026, from https://dantelabs.com/genes/mipep

Vancouver

Dante Labs. MIPEP gene: mitochondrial intermediate peptidase [Internet]. [cited 17 October 2026]. Available from: https://dantelabs.com/genes/mipep

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