Skip to main content

Advertisement

SpringerLink
Log in

Mitochondrial Biogenesis and Dynamics in Neurodegeneration: A Causative Relationship

  • Comments
  • Published:
Neurochemical Research Aims and scope Submit manuscript

This is a preview of subscription content, log in via an institution to check access.

Access this article

Log in via an institution

Price excludes VAT (USA)
Tax calculation will be finalised during checkout.

Instant access to the full article PDF.

Institutional subscriptions

Fig. 1

Abbreviations

AD:

Alzheimer’s disease

CMT2A:

Charcot-Marie-Tooth 2A

DOA:

Dominant optic atrophy

DRG:

Dorsal root ganglia

FCLS:

French Canadian Leigh syndrome

LHON:

Leber’s hereditary optic neuropathy

LRP130:

Leucine rich protein 130 kDa

MFN1:

Mitofusin1

MFN2:

Mitofusin2

mtDNA:

Mitochondrial DNA

OPA1:

Optic atrophy 1

PD:

Parkinson’s disease

PGC-1α:

Peroxisome proliferator-activated receptor coactivator 1-α

RGCs:

Retinal ganglion cells

References

  1. Bratic I, Trifunovic A (2010) Mitochondrial energy metabolism and ageing. Biochim Biophys Acta 1797(6–7):961–967. doi:10.1016/j.bbabio.2010.01.004

    Article  CAS  PubMed  Google Scholar 

  2. David DC, Hauptmann S, Scherping I, Schuessel K, Keil U, Rizzu P, Ravid R, Drose S, Brandt U, Muller WE, Eckert A, Gotz J (2005) Proteomic and functional analyses reveal a mitochondrial dysfunction in P301L tau transgenic mice. J Biol Chem 280(25):23802–23814. doi:10.1074/jbc.M500356200

    Article  CAS  PubMed  Google Scholar 

  3. Perry EK, Perry RH, Tomlinson BE, Blessed G, Gibson PH (1980) Coenzyme A-acetylating enzymes in Alzheimer’s disease: possible cholinergic ‘compartment’ of pyruvate dehydrogenase. Neurosci Lett 18(1):105–110

    Article  CAS  PubMed  Google Scholar 

  4. Rhein V, Baysang G, Rao S, Meier F, Bonert A, Muller-Spahn F, Eckert A (2009) Amyloid-beta leads to impaired cellular respiration, energy production and mitochondrial electron chain complex activities in human neuroblastoma cells. Cell Mol Neurobiol 29(6–7):1063–1071. doi:10.1007/s10571-009-9398-y

    Article  CAS  PubMed  Google Scholar 

  5. Abou-Sleiman PM, Muqit MM, Wood NW (2006) Expanding insights of mitochondrial dysfunction in Parkinson’s disease. Nat Rev Neurosci 7(3):207–219. doi:10.1038/nrn1868

    Article  CAS  PubMed  Google Scholar 

  6. Wallace DC, Singh G, Lott MT, Hodge JA, Schurr TG, Lezza AM, Elsas LJ 2nd, Nikoskelainen EK (1988) Mitochondrial DNA mutation associated with Leber’s hereditary optic neuropathy. Science 242(4884):1427–1430

    Article  CAS  PubMed  Google Scholar 

  7. Delettre C, Lenaers G, Pelloquin L, Belenguer P, Hamel CP (2002) OPA1 (Kjer type) dominant optic atrophy: a novel mitochondrial disease. Mol Genet Metab 75(2):97–107. doi:10.1006/mgme.2001.3278

    Article  CAS  PubMed  Google Scholar 

  8. Alexander C, Votruba M, Pesch UE, Thiselton DL, Mayer S, Moore A, Rodriguez M, Kellner U, Leo-Kottler B, Auburger G, Bhattacharya SS, Wissinger B (2000) OPA1, encoding a dynamin-related GTPase, is mutated in autosomal dominant optic atrophy linked to chromosome 3q28. Nat Genet 26(2):211–215. doi:10.1038/79944

    Article  CAS  PubMed  Google Scholar 

  9. Delettre C, Lenaers G, Griffoin JM, Gigarel N, Lorenzo C, Belenguer P, Pelloquin L, Grosgeorge J, Turc-Carel C, Perret E, Astarie-Dequeker C, Lasquellec L, Arnaud B, Ducommun B, Kaplan J, Hamel CP (2000) Nuclear gene OPA1, encoding a mitochondrial dynamin-related protein, is mutated in dominant optic atrophy. Nat Genet 26(2):207–210. doi:10.1038/79936

    Article  CAS  PubMed  Google Scholar 

  10. Elachouri G, Vidoni S, Zanna C, Pattyn A, Boukhaddaoui H, Gaget K, Yu-Wai-Man P, Gasparre G, Sarzi E, Delettre C, Olichon A, Loiseau D, Reynier P, Chinnery PF, Rotig A, Carelli V, Hamel CP, Rugolo M, Lenaers G (2011) OPA1 links human mitochondrial genome maintenance to mtDNA replication and distribution. Genome Res 21(1):12–20. doi:10.1101/gr.108696.110

    Article  CAS  PubMed  Google Scholar 

  11. Baloh RH, Schmidt RE, Pestronk A, Milbrandt J (2007) Altered axonal mitochondrial transport in the pathogenesis of Charcot-Marie-Tooth disease from mitofusin 2 mutations. J Neurosci 27(2):422–430. doi:10.1523/JNEUROSCI.4798-06.2007

    Article  CAS  PubMed  Google Scholar 

  12. Detmer SA, Vande Velde C, Cleveland DW, Chan DC (2008) Hindlimb gait defects due to motor axon loss and reduced distal muscles in a transgenic mouse model of Charcot-Marie-Tooth type 2A. Hum Mol Genet 17(3):367–375. doi:10.1093/hmg/ddm314

    Article  CAS  PubMed  Google Scholar 

  13. Chen S, Zhang Y, Wang Y, Li W, Huang S, Chu X, Wang L, Zhang M, Liu Z (2007) A novel OPA1 mutation responsible for autosomal dominant optic atrophy with high frequency hearing loss in a Chinese family. Am J Ophthalmol 143(1):186–188. doi:10.1016/j.ajo.2006.06.049

    Article  CAS  PubMed  Google Scholar 

  14. Lawson VH, Graham BV, Flanigan KM (2005) Clinical and electrophysiologic features of CMT2A with mutations in the mitofusin 2 gene. Neurology 65(2):197–204. doi:10.1212/01.wnl.0000168898.76071.70

    Article  CAS  PubMed  Google Scholar 

  15. Verhoeven K, Claeys KG, Zuchner S, Schroder JM, Weis J, Ceuterick C, Jordanova A, Nelis E, De Vriendt E, Van Hul M, Seeman P, Mazanec R, Saifi GM, Szigeti K, Mancias P, Butler IJ, Kochanski A, Ryniewicz B, De Bleecker J, Van den Bergh P, Verellen C, Van Coster R, Goemans N, Auer-Grumbach M, Robberecht W, Milic Rasic V, Nevo Y, Tournev I, Guergueltcheva V, Roelens F, Vieregge P, Vinci P, Moreno MT, Christen HJ, Shy ME, Lupski JR, Vance JM, De Jonghe P, Timmerman V (2006) MFN2 mutation distribution and genotype/phenotype correlation in Charcot-Marie-Tooth type 2. Brain 129(Pt 8):2093–2102. doi:10.1093/brain/awl126

    Article  PubMed  Google Scholar 

  16. Zuchner S, Mersiyanova IV, Muglia M, Bissar-Tadmouri N, Rochelle J, Dadali EL, Zappia M, Nelis E, Patitucci A, Senderek J, Parman Y, Evgrafov O, Jonghe PD, Takahashi Y, Tsuji S, Pericak-Vance MA, Quattrone A, Battaloglu E, Polyakov AV, Timmerman V, Schroder JM, Vance JM (2004) Mutations in the mitochondrial GTPase mitofusin 2 cause Charcot-Marie-Tooth neuropathy type 2A. Nat Genet 36(5):449–451. doi:10.1038/ng1341

    Article  PubMed  Google Scholar 

  17. Coombs JL, Van Der List D, Chalupa LM (2007) Morphological properties of mouse retinal ganglion cells during postnatal development. J Comp Neurol 503(6):803–814. doi:10.1002/cne.21429

    Article  PubMed  Google Scholar 

  18. Delettre C, Griffoin JM, Kaplan J, Dollfus H, Lorenz B, Faivre L, Lenaers G, Belenguer P, Hamel CP (2001) Mutation spectrum and splicing variants in the OPA1 gene. Hum Genet 109(6):584–591. doi:10.1007/s00439-001-0633-y

    Article  CAS  PubMed  Google Scholar 

  19. Dimmer KS, Papic D, Schumann B, Sperl D, Krumpe K, Walther DM, Rapaport D (2012) A crucial role for Mim2 in the biogenesis of mitochondrial outer membrane proteins. J Cell Sci 125(Pt 14):3464–3473. doi:10.1242/jcs.103804

    Article  CAS  PubMed  Google Scholar 

  20. Lopez-Domenech G, Serrat R, Mirra S, D’Aniello S, Somorjai I, Abad A, Vitureira N, Garcia-Arumi E, Alonso MT, Rodriguez-Prados M, Burgaya F, Andreu AL, Garcia-Sancho J, Trullas R, Garcia-Fernandez J, Soriano E (2012) The Eutherian Armcx genes regulate mitochondrial trafficking in neurons and interact with Miro and Trak2. Nat Commun 3:814. doi:10.1038/ncomms1829

    Article  PubMed  Google Scholar 

  21. DuBoff B, Gotz J, Feany MB (2012) Tau promotes neurodegeneration via DRP1 mislocalization in vivo. Neuron 75(4):618–632. doi:10.1016/j.neuron.2012.06.026

    Article  CAS  PubMed Central  PubMed  Google Scholar 

  22. Lehman JJ, Barger PM, Kovacs A, Saffitz JE, Medeiros DM, Kelly DP (2000) Peroxisome proliferator-activated receptor gamma coactivator-1 promotes cardiac mitochondrial biogenesis. J Clin Invest 106(7):847–856. doi:10.1172/JCI10268

    Article  CAS  PubMed Central  PubMed  Google Scholar 

  23. Wu Z, Puigserver P, Andersson U, Zhang C, Adelmant G, Mootha V, Troy A, Cinti S, Lowell B, Scarpulla RC, Spiegelman BM (1999) Mechanisms controlling mitochondrial biogenesis and respiration through the thermogenic coactivator PGC-1. Cell 98(1):115–124. doi:10.1016/S0092-8674(00)80611-X

    Article  CAS  PubMed  Google Scholar 

  24. Lin J, Wu PH, Tarr PT, Lindenberg KS, St-Pierre J, Zhang CY, Mootha VK, Jager S, Vianna CR, Reznick RM, Cui L, Manieri M, Donovan MX, Wu Z, Cooper MP, Fan MC, Rohas LM, Zavacki AM, Cinti S, Shulman GI, Lowell BB, Krainc D, Spiegelman BM (2004) Defects in adaptive energy metabolism with CNS-linked hyperactivity in PGC-1alpha null mice. Cell 119(1):121–135. doi:10.1016/j.cell.2004.09.013

    Article  CAS  PubMed  Google Scholar 

  25. Ma D, Li S, Lucas EK, Cowell RM, Lin JD (2010) Neuronal inactivation of peroxisome proliferator-activated receptor gamma coactivator 1alpha (PGC-1alpha) protects mice from diet-induced obesity and leads to degenerative lesions. J Biol Chem 285(50):39087–39095. doi:10.1074/jbc.M110.151688

    Article  CAS  PubMed  Google Scholar 

  26. Cooper MP, Qu L, Rohas LM, Lin J, Yang W, Erdjument-Bromage H, Tempst P, Spiegelman BM (2006) Defects in energy homeostasis in Leigh syndrome French Canadian variant through PGC-1alpha/LRP130 complex. Genes Dev 20(21):2996–3009. doi:10.1101/gad.1483906

    Article  CAS  PubMed  Google Scholar 

Download references

Acknowledgments

The authors wish to acknowledge funding from the European Research Council (ERC), the Marie Curie Fellowship Programme and the European Commission Framework Programmes. V. N. is supported by a European Molecular Biology Organization (EMBO) long term postdoctoral fellowship.

Author information

Authors and Affiliations

  1. Institute of Molecular Biology and Biotechnology, Foundation for Research and Technology, 71110, Heraklion, Crete, Greece

    Vassiliki Nikoletopoulou & Nektarios Tavernarakis

Authors
  1. Vassiliki Nikoletopoulou
    View author publications

    You can also search for this author in PubMed Google Scholar

  2. Nektarios Tavernarakis
    View author publications

    You can also search for this author in PubMed Google Scholar

Corresponding author

Correspondence to Nektarios Tavernarakis.

Rights and permissions

Reprints and permissions

About this article

Cite this article

Nikoletopoulou, V., Tavernarakis, N. Mitochondrial Biogenesis and Dynamics in Neurodegeneration: A Causative Relationship. Neurochem Res 39, 542–545 (2014). https://doi.org/10.1007/s11064-013-0997-0

Download citation

  • Received:

  • Revised:

  • Accepted:

  • Published:

  • Issue Date:

  • DOI: https://doi.org/10.1007/s11064-013-0997-0

Keywords

Search

Navigation