Progressive parkinsonism in mice with respiratory-chain-deficient dopamine neurons
- Mats I. Ekstrand, M. Terzioğlu, N. Larsson
- BiologyProceedings of the National Academy of Sciences
- 23 January 2007
It is demonstrated that respiratory chain dysfunction in DA neurons may be of pathophysiological importance in PD and reduced mtDNA expression and respiratory chain deficiency in midbrain DA neurons leads to a parkinsonism phenotype.
Somatic progenitor cell vulnerability to mitochondrial DNA mutagenesis underlies progeroid phenotypes in Polg mutator mice.
- K. Ahlqvist, R. Hämäläinen, A. Suomalainen
- BiologyCell Metabolism
- 4 January 2012
Parkinson’s disease: genetic versus toxin‐induced rodent models
- M. Terzioğlu, D. Galter
- BiologyThe FEBS Journal
- 1 April 2008
Three types of rodent animal models used to study different aspects of PD are compared: animal models using neurotoxins; genetically modified mouse models reproducing findings from PD linkage studies or based on ablation of genes necessary for the development and survival of dopamine neurons; and (c) tissue‐specific knockouts in mice targeting dopamine neurons.
High brain lactate is a hallmark of aging and caused by a shift in the lactate dehydrogenase A/B ratio
The mtDNA mutator mouse allows us to conclude that the increased LDH-A/LDH-B ratio causes high brain lactate levels, which, in turn, are predictive of aging phenotypes.
MTERF1 binds mtDNA to prevent transcriptional interference at the light-strand promoter but is dispensable for rRNA gene transcription regulation.
- M. Terzioğlu, Benedetta Ruzzenente, N. Larsson
- BiologyCell Metabolism
- 2 April 2013
Mitochondrial DNA Replication Defects Disturb Cellular dNTP Pools and Remodel One-Carbon Metabolism.
- Joni Nikkanen, S. Forsström, A. Suomalainen
- BiologyCell Metabolism
- 12 April 2016
The Molecular Basis of Glycogen Storage Disease Type 1a
- J. Shieh, M. Terzioğlu, J. Chou
- Biology, ChemistryJournal of Biological Chemistry
- 15 February 2002
The critical roles of transmembrane helices in the stability and activity of this phosphatase are established, and it is shown that G6Pase is a substrate for proteasome-mediated degradation.
Mitofusin 2 is necessary for striatal axonal projections of midbrain dopamine neurons.
- Seungmin Lee, F. Sterky, N. Larsson
- BiologyHuman Molecular Genetics
- 15 November 2012
It is found that Mfn1 is dispensable for DA neuron survival and motor function, and Mfn2, but not Mfn 1, is required for axonal projections of DA neurons in vivo.
Molecular analysis of Turkish mucopolysaccharidosis IVA (Morquio A) patients: identification of novel mutations in the N‐acetylgalactosamine‐6‐sulfate sulfatase (GALNS) gene
- M. Terzioğlu, A. Tokatlı, T. Coşkun, S. Emre
- BiologyHuman Mutation
- 1 December 2002
Mucopolysaccharidosis IVA is a lysosomal storage disorder caused by the deficiency of N‐acetylgalactosamine‐6‐sulfate sulfatase, and 6 different gene mutations and 2 polymorphisms were identified in 10 severely affected MPS IVA patients.
Loss of mtDNA activates astrocytes and leads to spongiotic encephalopathy
- O. Ignatenko, D. Chilov, A. Suomalainen
- BiologyNature Communications
- 4 January 2018
It is shown that astrocytes, the most abundant cerebral cell type, are chronically activated upon mtDNA loss, leading to early-onset spongiotic degeneration of brain parenchyma, microgliosis and secondary neurodegeneration.
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