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Gene Review

IMMT  -  inner membrane protein, mitochondrial

Homo sapiens

Synonyms: Cell proliferation-inducing gene 4/52 protein, HMP, MIC60, MICOS complex subunit MIC60, MINOS2, ...
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Disease relevance of IMMT


Psychiatry related information on IMMT

  • HMP Whitemoor, Cambridgeshire, is currently piloting a programme for the assessment and treatment of severe personality disorders [6].
  • BACKGROUND: The aim of this study is to evaluate the value ofTc-99 HMP AO cerebral SPECT imaging to confirm brain death in patients with complex spinal automatism [7].

High impact information on IMMT

  • The CO2 generated by the HMP shunt is spontaneously hydrated and the protons (H+) are secreted upon the dissociation of carbonic acid [8].
  • Reaction of human neutrophils with aggregated immunoglobulin on nonphagocytosable surfaces results in secretion of granule enzymes (exocytosis of granules) and stimulation of glucose oxidation by the nexose monophosphate pathway (HMP) [9].
  • Aggregated immunoglobulin reacting with neutrophil Fc receptors thus induces both degranulation (exocytosis) and increased HMP activity [9].
  • (b) Removal of Ca++ and addition of agents which increased the intracellular levels of cyclic AMP (cAMP), however, prevented both activities, while colchicine had greater inhibitory activity on HMP stimulation than upon secretion [9].
  • It was found that (a) HMP stimulation could be selectively inhibited under conditions where release of granule enzymes remained unchanged or was enhanced, for example, by reduced glucose concentration or by 2-deoxyglucose [9].

Chemical compound and disease context of IMMT


Biological context of IMMT


Anatomical context of IMMT

  • Mitofilin is enriched in the narrow space between the inner boundary and the outer membranes, where it forms a homotypic interaction and assembles into a large multimeric protein complex [12].
  • Mitofilin is a transmembrane protein of the inner mitochondrial membrane expressed as two isoforms [1].
  • Mitofilin, also known as heart muscle protein, is a recently identified mitochondrial protein [1].
  • The nuclear matrix protein matrin 3, cytoskeletal motor protein HMP, and the circadian clock protein hlark were significantly decreased in fetal DS brain [2].
  • The role of HMP stimulation in the enzyme secretion and some requirements for the two neutrophil activities have been examined [9].

Associations of IMMT with chemical compounds

  • We find that, at biologically relevant O(2) concentrations, HMP preferentially binds NO (not O(2)), which it then reacts with oxygen to form nitrate (in essence NO(-) + O(2) --> NO(3)(-)) [13].
  • It has been alternatively suggested that HMP activity is inhibited at low pO(2) because the enzyme is then in the relatively inactive nitrosyl form [k(off)/k(on) for NO (0.000008 microM) k(off)/k(on) for O(2) (0.012 microM) and K(M) for O(2) = 30-100 microM] [13].
  • Based on analysis of co-occurrence of the thiamin biosynthetic genes in complete genomes, we predict that eubacteria, archaea, and eukaryota have different pathways for the HMP and hydroxyethylthiazole biosynthesis [4].
  • A previously undescribed high molecular mass protein (HMP) from human erythrocyte membranes was solubilized by Triton X-100 and purified on a calmodulin-agarose column in the presence of Ca2+ [14].
  • Vitamin B(1) in its active form thiamin pyrophosphate is an essential coenzyme that is synthesized by coupling of pyrimidine (hydroxymethylpyrimidine; HMP) and thiazole (hydroxyethylthiazole) moieties in bacteria [4].

Physical interactions of IMMT


Other interactions of IMMT


Analytical, diagnostic and therapeutic context of IMMT

  • Sequence analysis and our results suggest that mitofilin is anchored in the inner mitochondrial membrane with an amino-terminal transmembrane domain, while the majority of the protein is extruding into the intermembrane space [1].
  • HMP was found to cross-react in Western blots with an antibody raised against the rabbit multicatalytic proteinase [14].
  • These results show that oral administration of HMP preferentially induces exclusive Th2-type immune responses, which may prevent the development of HMP (S-IgA and Lf)-specific mucosally induced tolerance [21].
  • Gel filtration column chromatography indicated that the native protein exists as a dimer, while isoelectric focusing suggested that there were at least four HMP isotypes [22].
  • Similarly, in RBC of different ages, obtained by density gradient ultracentrifugation, the glucose-6-phosphate concentration range from 57 (young cells) to 18 (old cells) nmole/ml RBC but the rate at which glucose is utilized in the HMP is unchanged [23].


  1. Mitofilin is a transmembrane protein of the inner mitochondrial membrane expressed as two isoforms. Gieffers, C., Korioth, F., Heimann, P., Ungermann, C., Frey, J. Exp. Cell Res. (1997) [Pubmed]
  2. Manifold decreased protein levels of matrin 3, reduced motor protein HMP and hlark in fetal Down's syndrome brain. Bernert, G., Fountoulakis, M., Lubec, G. Proteomics (2002) [Pubmed]
  3. A human monoclonal IgG1 with anti-idiotypic activity against anti-human thyroglobulin autoantibody. Zouali, M., Fine, J.M., Eyquem, A. J. Immunol. (1984) [Pubmed]
  4. Comparative genomics of thiamin biosynthesis in procaryotes. New genes and regulatory mechanisms. Rodionov, D.A., Vitreschak, A.G., Mironov, A.A., Gelfand, M.S. J. Biol. Chem. (2002) [Pubmed]
  5. Anonymous HIV surveillance with risk factor elicitation at Scotland's largest prison, Barlinnie. Bird, A.G., Gore, S.M., Cameron, S., Ross, A.J., Goldberg, D.J. AIDS (1995) [Pubmed]
  6. Managing personality disorders: making positive connections. Duff, A. Nursing management (Harrow, London, England : 1994) (2003) [Pubmed]
  7. Tc-99 HMPAO cerebral SPECT imaging in brain death patients with complex spinal automatism. Kahveci, F., Bekar, A., Tamgac, F. Ulusal travma dergisi = Turkish journal of trauma & emergency surgery : TJTES. (2002) [Pubmed]
  8. Proton secretion by the sodium/hydrogen ion antiporter in the human neutrophil. Wright, J., Schwartz, J.H., Olson, R., Kosowsky, J.M., Tauber, A.I. J. Clin. Invest. (1986) [Pubmed]
  9. Stimulation of human neutrophils by soluble and insoluble immunoglobulin aggregates. Secretion of granule constituents and increased oxidation of glucose. Henson, P.M., Oades, Z.G. J. Clin. Invest. (1975) [Pubmed]
  10. Synthesis, characterization, and immunologic properties of detoxified lipooligosaccharide from nontypeable Haemophilus influenzae conjugated to proteins. Gu, X.X., Tsai, C.M., Ueyama, T., Barenkamp, S.J., Robbins, J.B., Lim, D.J. Infect. Immun. (1996) [Pubmed]
  11. Gerbils of a seizure-sensitive strain have a mitochondrial inner membrane protein with different isoelectric points from those of a seizure-resistant strain. Omori, A., Ichinose, S., Kitajima, S., Shimotohno, K.W., Murashima, Y.L., Shimotohno, K., Seto-Ohshima, A. Electrophoresis (2002) [Pubmed]
  12. The mitochondrial inner membrane protein mitofilin controls cristae morphology. John, G.B., Shang, Y., Li, L., Renken, C., Mannella, C.A., Selker, J.M., Rangell, L., Bennett, M.J., Zha, J. Mol. Biol. Cell (2005) [Pubmed]
  13. Flavohemoglobin denitrosylase catalyzes the reaction of a nitroxyl equivalent with molecular oxygen. Hausladen, A., Gow, A., Stamler, J.S. Proc. Natl. Acad. Sci. U.S.A. (2001) [Pubmed]
  14. Characterization of a novel high molecular mass protein with peptidase activity purified from the human erythrocyte membrane by calmodulin affinity chromatography. Khan, M.T., Wang, K.K., Villalobo, A., Roufogalis, B.D. J. Biol. Chem. (1994) [Pubmed]
  15. Mutations in the membrane anchor of yeast cytochrome c1 compensate for the absence of Oxa1p and generate carbonate-extractable forms of cytochrome c1. Hamel, P., Lemaire, C., Bonnefoy, N., Brivet-Chevillotte, P., Dujardin, G. Genetics (1998) [Pubmed]
  16. GRACILE syndrome, a lethal metabolic disorder with iron overload, is caused by a point mutation in BCS1L. Visapää, I., Fellman, V., Vesa, J., Dasvarma, A., Hutton, J.L., Kumar, V., Payne, G.S., Makarow, M., Van Coster, R., Taylor, R.W., Turnbull, D.M., Suomalainen, A., Peltonen, L. Am. J. Hum. Genet. (2002) [Pubmed]
  17. Uncoupling proteins 2 and 3: potential regulators of mitochondrial energy metabolism. Boss, O., Hagen, T., Lowell, B.B. Diabetes (2000) [Pubmed]
  18. Uncoupling protein 3: its possible biological role and mode of regulation in rodents and humans. Muzzin, P., Boss, O., Giacobino, J.P. J. Bioenerg. Biomembr. (1999) [Pubmed]
  19. Mitochondrial control of cell death induced by HIV-1-encoded proteins. Ferri, K.F., Jacotot, E., Blanco, J., Esté, J.A., Kroemer, G. Ann. N. Y. Acad. Sci. (2000) [Pubmed]
  20. Amish lethal microcephaly: a new metabolic disorder with severe congenital microcephaly and 2-ketoglutaric aciduria. Kelley, R.I., Robinson, D., Puffenberger, E.G., Strauss, K.A., Morton, D.H. Am. J. Med. Genet. (2002) [Pubmed]
  21. Human milk proteins including secretory IgA fail to elicit tolerance after feeding. Yuki, Y., Fujihashi, K., Yamamoto, M., McGhee, J.R., Kiyono, H. Int. Immunol. (1998) [Pubmed]
  22. A Tudor protein with multiple SNc domains from pea seedlings: cellular localization, partial characterization, sequence analysis, and phylogenetic relationships. Abe, S., Sakai, M., Yagi, K., Hagino, T., Ochi, K., Shibata, K., Davies, E. J. Exp. Bot. (2003) [Pubmed]
  23. Relationship between the rate of erythrocyte hexose monophosphate pathway and the glucose 6-phosphate concentration. Magnani, M., Stocchi, V., Fazi, A., Dachà, M., Fornaini, G. Biochem. Biophys. Res. Commun. (1984) [Pubmed]
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