Methylene Blue Alleviates Nuclear And Mitochondrial Abnormalities In Progeria

Ras

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From their summary:

Hutchinson–Gilford progeria syndrome (HGPS), a fatal premature aging disease, is caused by a single-nucleotide mutation in the LMNA gene. Previous reports have focused on nuclear phenotypes in HGPS cells, yet the potential contribution of the mitochondria, a key player in normal aging, remains unclear. Using high-resolution microscopy analysis, we demonstrated a significantly increased fraction of swollen and fragmented mitochondria and a marked reduction in mitochondrial mobility in HGPS fibroblast cells. Notably, the expression of PGC-1α, a central regulator of mitochondrial biogenesis, was inhibited by progerin. To rescue mitochondrial defects, we treated HGPS cells with a mitochondrial-targeting antioxidant methylene blue (MB). Our analysis indicated that MB treatment not only alleviated the mitochondrial defects but also rescued the hallmark nuclear abnormalities in HGPS cells. Additional analysis suggested that MB treatment released progerin from the nuclear membrane, rescued perinuclear heterochromatin loss and corrected misregulated gene expression in HGPS cells. Together, these results demonstrate a role of mitochondrial dysfunction in developing the premature aging phenotypes in HGPS cells and suggest MB as a promising therapeutic approach for HGPS.

acel12434-fig-0004.png

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haidut

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Ras said:
post 115852 From their summary:

Hutchinson–Gilford progeria syndrome (HGPS), a fatal premature aging disease, is caused by a single-nucleotide mutation in the LMNA gene. Previous reports have focused on nuclear phenotypes in HGPS cells, yet the potential contribution of the mitochondria, a key player in normal aging, remains unclear. Using high-resolution microscopy analysis, we demonstrated a significantly increased fraction of swollen and fragmented mitochondria and a marked reduction in mitochondrial mobility in HGPS fibroblast cells. Notably, the expression of PGC-1α, a central regulator of mitochondrial biogenesis, was inhibited by progerin. To rescue mitochondrial defects, we treated HGPS cells with a mitochondrial-targeting antioxidant methylene blue (MB). Our analysis indicated that MB treatment not only alleviated the mitochondrial defects but also rescued the hallmark nuclear abnormalities in HGPS cells. Additional analysis suggested that MB treatment released progerin from the nuclear membrane, rescued perinuclear heterochromatin loss and corrected misregulated gene expression in HGPS cells. Together, these results demonstrate a role of mitochondrial dysfunction in developing the premature aging phenotypes in HGPS cells and suggest MB as a promising therapeutic approach for HGPS.

acel12434-fig-0004.png

Full study

About the disease

This is actually a really good study because it not only shows that MB reverses progeria, but MB can also have anti-aging abilities in normally aging organisms. The concentration used was only 100nM, which is in line with the other studies showing life extension benefits from MB doses in the range of 1mg, as Peat recommends as well.
 
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I want to express my appreciation for your posts here haidut, they are remarkable and I really am grateful.
 

haidut

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ecstatichamster said:
post 115920 I want to express my appreciation for your posts here haidut, they are remarkable and I really am grateful.

Thank you, it means the world to me!
:hattip
 
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haidut

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Vinero said:
post 115944 He also posts on reddit apparently

I used to but not so much in the last 2 years or so. Most people who comment on that site are looking to pick up a fight with somebody, and in general are insanely pro-establishment. Not surprising, given their self-admitted regular use of SSRI.
 
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Vinero

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Yeah but it was still fun to read those posts as most peoples minds are blown away to see such radical posts that actually make sense, are based on scientific studies, and are more intelligent than 99% of other posters.
 

Soren

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Does this study indicate that MB actually enters the nucleus of a cell? Or just that it has a beneficial effect on it?

Also does anyone know of any studies that show MB entering the cell nucleus?
 

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