MIEF1

Protein identityUniProt · HPA

UniProt accession

Q9NQG6

Gene

MIEF1

Ensembl

ENSG00000100335

Chromosome

22

Canonical length

463 aa

Protein class

Predicted intracellular proteins, Predicted membrane proteins

Subcellular location

Intermediate filaments,Mitochondria

Quaternary structure

Homodimer

OverviewNCBI Gene

Enables identical protein binding activity; mitochondrial large ribosomal subunit binding activity; and purine ribonucleotide binding activity. Involved in several processes, including mitochondrial large ribosomal subunit assembly; mitochondrion organization; and regulation of mitochondrion organization. Located in mitochondrial matrix and mitochondrial outer membrane. Implicated in optic atrophy. [provided by Alliance of Genome Resources, Jul 2025]

Canonical amino-acid sequenceUniProt

463 residues, UniProt reviewed canonical sequence.

>Q9NQG6|MIEF1
     1  MAGAGERKGK KDDNGIGTAI DFVLSNARLV LGVGGAAMLG IATLAVKRMY DRAISAPTSP
    61  TRLSHSGKRS WEEPNWMGSP RLLNRDMKTG LSRSLQTLPT DSSTFDTDTF CPPRPKPVAR
   121  KGQVDLKKSR LRMSLQEKLL TYYRNRAAIP AGEQARAKQA AVDICAELRS FLRAKLPDMP
   181  LRDMYLSGSL YDDLQVVTAD HIQLIVPLVL EQNLWSCIPG EDTIMNVPGF FLVRRENPEY
   241  FPRGSSYWDR CVVGGYLSPK TVADTFEKVV AGSINWPAIG SLLDYVIRPA PPPEALTLEV
   301  QYERDKHLFI DFLPSVTLGD TVLVAKPHRL AQYDNLWRLS LRPAETARLR ALDQADSGCR
   361  SLCLKILKAI CKSTPALGHL TASQLTNVIL HLAQEEADWS PDMLADRFLQ ALRGLISYLE
   421  AGVLPSALNP KVNLFAELTP EEIDELGYTL YCSLSEPEVL LQT

LocalizationUniProt · AlphaFold · HPA

Whether an antibody against MIEF1 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

Other membrane

Secreted

No

Transmembrane segments

1

Mean surface accessibility (rSASA)

0.36

Highest tissue expression

17 nTPM

Expression across tissuesHPA

DiseaseUniProt · ClinVar · IEDB · PubMed

Four sources answering four different questions about MIEF1.

Disease | AllUniProt

Conditions MIEF1 is implicated in, by any mechanism.

• Optic atrophy 14 (OPA14) MIM:620550

Disease | GeneticClinVar

2 pathogenic / likely-pathogenic of 82 ClinVar records.

Conditions with pathogenic or likely-pathogenic variants.

• Optic atrophy 14

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)

0.73

gnomAD pLI

0.01

gnomAD missense Z

0.82

DepMap mean gene effect

-0.16

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

• mitochondrial fission
• positive regulation of mitochondrial fission
• positive regulation of mitochondrial fusion
• positive regulation of protein targeting to membrane

Molecular functions

• ADP binding
• GDP binding
• identical protein binding

Cellular components

• mitochondrial matrix
• mitochondrial outer membrane
• mitochondrion

Protein domainsUniProt · Pfam · InterPro

• MAB21L/Cyclic GMP-AMP synthase-like receptor
• Mitochondrial dynamics protein MID49/MID51
• Mab-21-like, HhH/H2TH-like domain
• Mitochondrial dynamics protein MID51-like, C-terminal domain
• Mab-21 protein HhH/H2TH-like domain
• Mitochondrial dynamics protein MID51-like, C-terminal domain

KeywordsUniProt

• Alternative initiation
• Membrane
• Mitochondrion
• Mitochondrion outer membrane
• Nucleotide-binding
• Phosphoprotein
• Transmembrane
• Transmembrane helix

InteractionsUniProt · HPA

Protein binding partners of MIEF1 in the human serome: UniProt-annotated complex subunits plus reported interactors. Each links to its own Seroatlas record.

• DNM1L
• FIS1
• YWHAG