Mitofusins Mfn1 and Mfn2 coordinately regulate mitochondrial fusion and are essential for embryonic development.

Mitofusins Mfn1 and Mfn2 coordinately regulate mitochondrial fusion and are essential for embryonic development. Research Abstract Details 

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Mitofusins Mfn1 and Mfn2 coordinately regulate mitochondrial fusion and are essential for embryonic development. Abstract Text:

Hsiuchen Chen,Scott A Detmer,Andrew J Ewald,Erik E Griffin,Scott E Fraser,David C Chan,

Mitochondrial morphology is determined by a dynamic equilibrium between organelle fusion and fission, but the significance of these processes in vertebrates is unknown. The mitofusins, Mfn1 and Mfn2, have been shown to affect mitochondrial morphology when overexpressed. We find that mice deficient in either Mfn1 or Mfn2 die in midgestation. However, whereas Mfn2 mutant embryos have a specific and severe disruption of the placental trophoblast giant cell layer, Mfn1-deficient giant cells are normal. Embryonic fibroblasts lacking Mfn1 or Mfn2 display distinct types of fragmented mitochondria, a phenotype we determine to be due to a severe reduction in mitochondrial fusion. Moreover, we find that Mfn1 and Mfn2 form homotypic and heterotypic complexes and show, by rescue of mutant cells, that the homotypic complexes are functional for fusion. We conclude that Mfn1 and Mfn2 have both redundant and distinct functions and act in three separate molecular complexes to promote mitochondrial fusion. Strikingly, a subset of mitochondria in mutant cells lose membrane potential. Therefore, mitochondrial fusion is essential for embryonic development, and by enabling cooperation between mitochondria, has protective effects on the mitochondrial population.

Mitofusins Mfn1 and Mfn2 coordinately regulate mitochondrial fusion and are essential for embryonic development. Publishing Authors By Initials

H Chen,SA Detmer,AJ Ewald,EE Griffin,SE Fraser,DC Chan,

For similar proteins: cytoskeletal proteins: utrophin research abstracts see: proteins: cytoskeletal proteins: utrophin research

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Mitofusins Mfn1 and Mfn2 coordinately regulate mitochondrial fusion and are essential for embryonic development. Journal Published:

PUBLICATION TYPE: Research Support, U.S. Gov't,

Journal: The Journal of cell biology

VOLUME: 160

Page Numbers: 189-200

Journal Abbreviation: J. Cell Biol.

ISSN: 0021-9525

DAY: 13

MONTH: 01

YEAR: 2003

Mitofusins Mfn1 and Mfn2 coordinately regulate mitochondrial fusion and are essential for embryonic development. Information

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LANGUAGE: eng

NlmUniqueID: 375356

Mitofusins Mfn1 and Mfn2 coordinately regulate mitochondrial fusion and are essential for embryonic development. Keywords Mesh Terms:

KEYWORDS: Utrophin

MESH TERMS: pathology

Chemical & Substance for Abstract: Mitofusins Mfn1 and Mfn2 coordinately regulate mitochondrial fusion and are essential for embryonic development. Information

Substance Name: Mfn1 protein, human

Registry Number: EC 3.6.5.-

Grant and Affiliation Information for Mitofusins Mfn1 and Mfn2 coordinately regulate mitochondrial fusion and are essential for embryonic development.

AFFILIATION: Division of Biology, Beckman Institute, California Institute of Technology, Pasadena, CA 91125, USA.

Country: United States

AGENCY: United States NIGMS

GRANT: GM07616

ACRONYM: GM

MEDLINETA: J Cell Biol

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