Original article<br>Mitofusin 2 expression dominates over mitofusin 1 exclusively in mouse dorsal root ganglia – a possible explanation for peripheral nervous system involvement in Charcot-Marie-Tooth 2A

Maria Kawalec; Barbara Zabłocka; Dagmara Kabzińska; Jacek Neska; Małgorzata Beręsewicz

doi:10.5114/fn.2014.47845

eISSN: 1509-572x
ISSN: 1641-4640

Folia Neuropathologica

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4/2014
vol. 52

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abstract:

Original article
Mitofusin 2 expression dominates over mitofusin 1 exclusively in mouse dorsal root ganglia – a possible explanation for peripheral nervous system involvement in Charcot-Marie-Tooth 2A

Maria Kawalec

,

Barbara Zabłocka

,

Dagmara Kabzińska

,

Jacek Neska

,

Małgorzata Beręsewicz

Folia Neuropathol 2014; 52 (4): 436-442

DOI: https://doi.org/10.5114/fn.2014.47845

Online publish date: 2014/12/30

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AMA

Kawalec M, Zabłocka B, Kabzińska D, Neska J, Beręsewicz M. Original articleMitofusin 2 expression dominates over mitofusin 1 exclusively in mouse dorsal root ganglia – a possible explanation for peripheral nervous system involvement in Charcot-Marie-Tooth 2A. Folia Neuropathologica. 2014;52(4):436-442. doi:10.5114/fn.2014.47845.

APA

Kawalec, M., Zabłocka, B., Kabzińska, D., Neska, J.,  & Beręsewicz, M. (2014). Original articleMitofusin 2 expression dominates over mitofusin 1 exclusively in mouse dorsal root ganglia – a possible explanation for peripheral nervous system involvement in Charcot-Marie-Tooth 2A. Folia Neuropathologica, 52(4), 436-442. https://doi.org/10.5114/fn.2014.47845

Chicago

Kawalec, Maria, Barbara Zabłocka, Dagmara Kabzińska, Jacek Neska,  and Małgorzata Beręsewicz. 2014. "Original articleMitofusin 2 expression dominates over mitofusin 1 exclusively in mouse dorsal root ganglia – a possible explanation for peripheral nervous system involvement in Charcot-Marie-Tooth 2A". Folia Neuropathologica 52 (4): 436-442. doi:10.5114/fn.2014.47845.

Harvard

Kawalec, M., Zabłocka, B., Kabzińska, D., Neska, J.,  and Beręsewicz, M. (2014). Original articleMitofusin 2 expression dominates over mitofusin 1 exclusively in mouse dorsal root ganglia – a possible explanation for peripheral nervous system involvement in Charcot-Marie-Tooth 2A. Folia Neuropathologica, 52(4), pp.436-442. https://doi.org/10.5114/fn.2014.47845

MLA

Kawalec, Maria et al. "Original articleMitofusin 2 expression dominates over mitofusin 1 exclusively in mouse dorsal root ganglia – a possible explanation for peripheral nervous system involvement in Charcot-Marie-Tooth 2A." Folia Neuropathologica, vol. 52, no. 4, 2014, pp. 436-442. doi:10.5114/fn.2014.47845.

Vancouver

Kawalec M, Zabłocka B, Kabzińska D, Neska J, Beręsewicz M. Original articleMitofusin 2 expression dominates over mitofusin 1 exclusively in mouse dorsal root ganglia – a possible explanation for peripheral nervous system involvement in Charcot-Marie-Tooth 2A. Folia Neuropathologica. 2014;52(4):436-442. doi:10.5114/fn.2014.47845.

PlumX metrics:

Mitofusin 2 (Mfn2), a protein of the mitochondrial outer membrane, is essential for mitochondrial fusion and contributes to the maintenance and operation of the mitochondrial network. Mutations in the mitofusin 2 gene cause axonal Charcot-Marie-Tooth type 2A (CMT2A), an inherited disease affecting peripheral nerve axons. The precise mechanism by which mutations in MFN2 selectively cause the degeneration of long peripheral axons is not known. There is a hypothesis suggesting the involvement of reduced expression of a homologous protein, mitofusin 1 (Mfn1), in the peripheral nervous system, and less effective compensation of defective mitofusin 2 by mitofusin 1. We therefore aimed to perform an analysis of the mitofusin 1 and mitofusin 2 mRNA and protein expression profiles in different mouse tissues, with special attention paid to dorsal root ganglia (DRGs), as parts of the peripheral nervous system. Quantitative measurement relating to mRNA revealed that expression of the Mfn2 gene dominates over Mfn1 mainly in mouse DRG, as opposed to other nervous system samples and other tissues studied. This result was further supported by Western blot evaluation. Both these sets of data confirm the hypothesis that the cellular consequences of mutations in the mitofusin 2 gene can mostly be manifested in the peripheral nervous system

keywords:

Original articleMitofusin 2 expression dominates over mitofusin 1 exclusively in mouse dorsal root ganglia – a possible explanation for peripheral nervous system involvement in Charcot-Marie-Tooth 2A

Maria Kawalec , Barbara Zabłocka , Dagmara Kabzińska , Jacek Neska , Małgorzata Beręsewicz

mitofusins, peripheral nervous system, CMT2A, Mfn2 mutation manifestation

Original article
Mitofusin 2 expression dominates over mitofusin 1 exclusively in mouse dorsal root ganglia – a possible explanation for peripheral nervous system involvement in Charcot-Marie-Tooth 2A

Maria Kawalec

,

Barbara Zabłocka

,

Dagmara Kabzińska

,

Jacek Neska

,

Małgorzata Beręsewicz