SUMMARY:
Presence of the R364W-MFN2 mutation makes cells susceptible towards stress, thus negatively affecting cellular health.
TITLE:
CMT2A-linked mitochondrial hyperfusion-driving mutant MFN2 perturbs ER-mitochondrial associations and Ca2+ homeostasis
DESCRIPTION:
Mitofusin2 (MFN2), an important molecular player that regulates mitochondrial fusion, also helps maintain the inter-organellar contact sites, referred as mitochondria associated membranes (MAMs) that exist between the ER and mitochondria. Here we show that a mutant of MFN2, R364W-MFN2, linked with the Charcot Marie Tooth disease, promotes mitochondrial hyperfusion, alters ER mitochondrial associations at the MAM junctions and perturbs inter-organellar calcium homeostasis. Such hyperfused…
CONTENT:
Biol Cell. 2022 Aug 4. doi: 10.1111/boc.202100098. Online ahead of print.
ABSTRACT
Mitofusin2 (MFN2), an important molecular player that regulates mitochondrial fusion, also helps maintain the inter-organellar contact sites, referred as mitochondria associated membranes (MAMs) that exist between the ER and mitochondria. Here we show that a mutant of MFN2, R364W-MFN2, linked with the Charcot Marie Tooth disease, promotes mitochondrial hyperfusion, alters ER mitochondrial associations at the MAM junctions and perturbs inter-organellar calcium homeostasis. Such hyperfused mitochondria are also predisposed towards stress and undergo rapid fission upon induction of mild stress. Thus, here we report that presence of the R364W-MFN2 mutation makes cells susceptible towards stress, thus negatively affecting cellular health. This article is protected by copyright. All rights reserved.
PMID:35924634 | DOI:10.1111/boc.202100098
SOURCE:
Biology of the cell
TAGS:
R364WMFN2
CATEGORY:
Research
SUBCATEGORY:
n/a
DATE – PUBLISHED:
2022-08-04T09:28:57Z
DATE – DOI: 2022-08-04T09:28:57Z
DATE – PUBMED: 2022 Aug 4
DATE OUTPUT MATCHED: True
DATE – ADDED:
Thu, 04 Aug 2022 06:00:00 -0400
DATE – RETRIEVED:
08/04/22 12:15PM
2022-08-04T12:15:07-04:00
FEATURED IMAGE:
Media Uploaded (image/png)
IDENTIFIER:
pmid:35924634,doi:10.1111/boc.202100098
PUBMED ID:
pubmed:35924634
DOI:
10.1111/boc.202100098
LINK – PUBMED:
https://pubmed.ncbi.nlm.nih.gov/35924634/
LINK – DOI:
https://doi.org/10.1111/boc.202100098
LINK – PUBLISHER:
https://onlinelibrary.wiley.com/doi/10.1111/boc.202100098
REFERENCES:
CMT Treatment Report, Urgent Research, 2022-08-04T12:15:07-04:00, https://www.cmttreatmentreport.com.
