https://doi.org/10.1186/s13023-021-02164-x
https://pubmed.ncbi.nlm.nih.gov/35012599
Dear Editor,
We read with appreciation the succinct and informative systematic review by Zweers et al. [1] on the efect of the Ketogenic Diet and/or the Modifed Atkins Diet on various mitochondrial disease associated phenotypes.
While this review covered studies pertaining to epilepsy, skeletal and heart muscle, tonus dysregulation, movement disorder, developmental delay/intellectual disability, food intake, weight gain/growth, and hair growth, we note that the multiple symmetric lipomatosis phenotype, frequently associated with the MT-TK genetic mutation, was not covered.
We would like to highlight that a version of the ketogenic diet was employed successfully, along with other simultaneous lifestyle interventions, in treating a disabling multiple symmetric lipomatosis phenotype. This successful intervention was reported in 2020 [2] and warrants further consideration by care providers whose patients are in a similar predicament, and similarly motivated.
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Open Access: True
Authors: Andre Mattman - Elizabeth Nadeau - Michelle M. Mezei - Mark Cresswell - Sida Zhao - Taryn Bosdet - Don D. Sin - Jordan A. Guenette - Isabelle Dupuis - Emily Allin - David C. Clarke -
Additional links:
https://ojrd.biomedcentral.com/track/pdf/10.1186/s13023-021-02164-x
https://www.longevity.technology/harnessing-the-power-of-mitochondria-to-beat-parkinsons/?utm_source=reddit&utm_medium=social&utm_campaign=vincere
Hey everyone, after struggling for 3 years with this unknown mystery illness that I’ve been battling, I have finally gotten some clarity. I am A 23 year old female who is 5’6 and 145lbs with apple body fat distribution. I currently taking metformin. I didn’t know if I had pcos, NCAH, hyper or hypothyroidism, Cushings, or something else. My menstrual cycle stopped, my hair began falling out, I got horrible jaw line acne; hirsutism, etc. I also experienced symptoms of hypoglycemia, blurred vision, tingling hands and feet and high ALT liver enzymes upon blood work. I knew these were symptoms of diabetes and rest assured I was tested positive for type 2 diabetes and pcos.
My endocrinologist put me on metformin to help with the insulin resistance and pcos which are linked and I may also try spironolactone in the future. However, something strange began happening to me and I think I know what is. I began losing muscle mass in my arms and legs and could not exercise anymore. I used to lift weights be fit, and resistance training helps diabetes. But, I could not physically exercise and my muscles atrophied. I saw a few studies that did liver catabolism induces muscle atrophying in t2dm patients. I do have elevated liver enzymes and high CRP levels.
The article I will share talks about a solution in which the ALT enzymes are “silenced” and thereby stops gluconeogenesis in the liver and decrease hyperglycemia and muscle wasting is reversed. However I don’t know how the liver enzymes are “silenced” and targeted as the article states. Can anyone help? Has anyone gone through the same thing? I just want to get my life back and be fit and healthy again instead of frail weak and diabetic. I don’t even know if this is my real issue or if I have glucocorticoid receptor resistance syndrome, Cushings but I have no moon face and im skinny so no doctor will take me seriously. Maybe mitochondrial disease? I’m so tired of fighting this. Please help. Thank you all advice welcome.
https://medivizor.com/blog/2021/03/22/type-2-diabetes-liver-function-and-muscle-loss/
I'm looking to see if anyone has experience or insight into long-term fasting with 2+ hours of "aerobic" exercise per day. This is extreme, yes, but it's the only way to correct the disease (Leigh syndrome due to complex I deficiency).
I have tried keto about 11 times. It usually does nothing, even fasting. I'm 275 from steroids, and it won't lose, because mitochondria are necessary for burning fat through beta-oxidation. Instead of my body burning the excess fat, I'll simply be unconscious for 20+ hours per day. This last time, muscles were destroyed and turned into glucose. I went into kidney failure from the rhabdomyolysis.
I've worked really hard to be able to go into ketosis from exercise, but the reliance on glycolysis at the end of the day after the gym is life-threatening now too. In fact, I ONLY go into ketosis while exercising.
It seems I won't really lose weight with keto or fasting, or with exercise and intermittent fasting. The lack of weight loss is indicative of my severe energy disorder. It's really dangerous, but the only cure for my disease is to replace old, damaged mitochondria with wild-type, through caloric restriction and exercise.
I'm about to undergo total fasting with only bone broth in the morning to absorb my medications, with my usual exercise which is competitive swimming and triathlon training 2+ hours per day. I cannot burn fat with anything less. If my mitochondria cannot burn the fat, I will end up in the ICU again on a sugar drip.
When I do this fast (maybe 60 days or so), my mitochondria will have to burn the fat or else I will die, which is a real possibility, but with anything less than this extreme, they wait for sugar, or dietary fats instead of inducing lipolysis. My body will do whatever it can to reduce energy expenditure to save my life. But it's also paradoxically ending my life. I've activated the ability to enter ketosis with a PPAR-alpha agonist, and I will fast with thyroid hormone which is downregulated in starvation.
If this doesn't work, the only option left is massive liposuction.
PERSPECTIVE article
Front. Nutr. | doi: 10.3389/fnut.2021.744398
Can ketogenic diet improve aging brain? Vision of Alzheimer Disease
Provisionally acceptedThe final, formatted version of the article will be published soonNotify me****đź“·Jose Enrique D. OrtĂ1**,** đź“·David Fernández-GarcĂa1**,** đź“·FĂ©lix Platero Armero2* and đź“·MARIA PILAR GARCIA PARDO3*
- 1Department of Nursing, Catholic University of Valencia San Vicente Mártir, Spain
- 2Department of Medicine, University of Valencia, Spain
- 3Department of Psychology and Sociology, University of Zaragoza, Spain
Alzheimer’s disease is characterized by presenting an aged brain, which is linked to neuropsychiatric symptoms such as anxiety and depression that worsen quality of life and disease course. This aging process, is mediated in turn at a central level, by the presence of β amyloid plaques and by alterations in the synaptic and extrasynaptic activity of the neurotransmitter glutamate, which are linked events to a hypometabolism of the glucose as the main source of cerebral energy. In this sense, pharmacologic treatments of these symptoms seem to be counterproductive, worsening the disease prognosis. This is why the alternative of consuming ketogenic diets as a mitochondrial energy source is raised and discussed, since they could improve that hypometabolism by acting both at the synaptic level: improving the glutamate synapsis glutamate in the NMDA synaptic receptors (sNMDAR), blocking the toxicity resulting from the formation of amyloid plaques after the activation of mGluR2 / 3 glutamate receptors, and enhancing glutamate function as a consequence of a greater ATP contribution which favours cognitive and emotional capacity; and extrasynaptic: since ketone bodies act as glutamate inhibitors in the NMDA extrasynaptic receptor (eNMDAR) decreasing inflammation, added to the characteristic neuroprotector effect of these metabolites precisely because they better the electron chain functioning which leads to a lower oxidative stress and inflammation.
Keywords: Alzheimer's disease, ketogenic diets, Anxiety, Depression, Glutamate
Received: 20 Jul 2021; Accepted: 20 Sep 2021.
[https://www.frontiersin.org/articles/10.3389/fnut.202
... keep reading on reddit ➡Ketogenic diet for mitochondrial disease: a systematic review on efficacy and safety
https://www.ncbi.nlm.nih.gov/labs/pmc/articles/PMC8254320/
Abstract
Background
No curative therapy for mitochondrial disease (MD) exists, prioritizing supportive treatment for symptom relief. In animal and cell models ketones decrease oxidative stress, increase antioxidants and scavenge free radicals, putting ketogenic diets (KDs) on the list of management options for MD. Furthermore, KDs are well-known, safe and effective treatments for epilepsy, a frequent symptom of MD. This systematic review evaluates efficacy and safety of KD for MD.
Methods
We searched Pubmed, Cochrane, Embase and Cinahl (November 2020) with search terms linked to MD and KD. From the identified records, we excluded studies on Pyruvate Dehydrogenase Complex deficiency. From these eligible reports, cases without a genetically confirmed diagnosis and cases without sufficient data on KD and clinical course were excluded. The remaining studies were included in the qualitative analysis.
Results
Only 20 cases (14 pediatric) from the 694 papers identified met the inclusion criteria (one controlled trial (n = 5), 15 case reports). KD led to seizure control in 7 out of 8 cases and improved muscular symptoms in 3 of 10 individuals. In 4 of 20 cases KD reversed the clinical phenotype (e.g. cardiomyopathy, movement disorder). In 5 adults with mitochondrial DNA deletion(s) related myopathy rhabdomyolysis led to cessation of KD. Three individuals with POLG mutations died while being on KD, however, their survival was not different compared to individuals with POLG mutations without KD.
Conclusion
Data on efficacy and safety of KD for MD is too scarce for general recommendations. KD should be considered in individuals with MD and therapy refractory epilepsy, while KD is contraindicated in mitochondrial DNA deletion(s) related myopathy. When considering KD for MD the high rate of adverse effects should be taken into account, but also spectacular improvements in individual cases. KD is a highly individual management option in this fragile patient group and requires an experienced team. To increase knowledge on this—individually—promising management option more (prospective) studies using adequate outcome measures are crucial.
Supplementary Information
The online version contains supplementary material available at 10
... keep reading on reddit ➡Front Med (Lausanne). 2021; 8: 689042.Published online 2021 Aug 9. doi: 10.3389/fmed.2021.689042PMCID: PMC8381051PMID: 34434943
Role of Carnitine in Non-alcoholic Fatty Liver Disease and Other Related Diseases: An Update
Na Li 1 , 2 , †and Hui Zhao 3 , * , †Author information Article notes Copyright and License information DisclaimerGo to:
Abstract
Carnitine is an amino acid-derived substance that coordinates a wide range of biological processes. Such functions include transport of long-chain fatty acids from the cytoplasm to the mitochondrial matrix, regulation of acetyl-CoA/CoA, control of inter-organellar acyl traffic, and protection against oxidative stress. Recent studies have found that carnitine plays an important role in several diseases, including non-alcoholic fatty liver disease (NAFLD). However, its effect is still controversial, and its mechanism is not clear. Herein, this review provides current knowledge on the biological functions of carnitine, the “multiple hit” impact of carnitine on the NAFLD progression, and the downstream mechanisms. Based on the “multiple hit” hypothesis, carnitine inhibits β-oxidation, improves mitochondrial dysfunction, and reduces insulin resistance to ameliorate NAFLD. L-carnitine may have therapeutic role in liver diseases including non-alcoholic steatohepatitis, cirrhosis, hepatocellular carcinoma, alcoholic fatty liver disease, and viral hepatitis. We also discuss the prospects of L-carnitine supplementation as a therapeutic strategy in NAFLD and related diseases, and the factors limiting its widespread use.
Keywords: carnitine, non-alcoholic fatty liver disease, L-carnitine supplementation, targeted therapy, therapeutic diet
https://www.nature.com/articles/s41380-021-01068-3
- Article
- Open Access
- Published: 16 April 2021
Brain cells derived from Alzheimer’s disease patients have multiple specific innate abnormalities in energy metabolism
- Woo-In Ryu,
- Mariana K. Bormann,
- Minqi Shen,
- Dohoon Kim,
- Brent Forester,
- Yeongwon Park,
- Jisun So,
- Hyemyung Seo,
- Kai-C. Sonntag &
- Bruce M. Cohen
Molecular Psychiatry (2021)Cite this article
- 431 Accesses
- 8 Altmetric
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Abstract
Altered energy metabolism has been implicated both in aging and the pathogenesis of late-onset Alzheimer’s disease (LOAD). However, it is unclear which anomalies are acquired phenotypes and which are inherent and predispose to disease. We report that neural progenitor cells and astrocytes differentiated from LOAD patient-derived induced pluripotent stem cells exhibit multiple inter-related bioenergetic alterations including: changes in energy production by mitochondrial respiration versus glycolysis, as a consequence of alterations in bioenergetic substrate processing and transfer of reducing agents, reduced levels of NAD/NADH, diminished glucose uptake and response rates to insulin (INS)/IGF-1 signaling, decreased INS receptor and glucose transporter 1 densities, and changes in the metabolic transcriptome. Our data confirm that LOAD is a “multi-hit” disorder and provide evidence for innate inefficient cellular energy management in LOAD that likely predisposes to neurod
... keep reading on reddit ➡Hi! I am a lab tech leaving my first job of over 2 years in a couple months and I really want to get my PIs and coworkers thank you gifts before leaving. I am the only Bachelor’s holder in the lab, everyone else is either a postdoc or MD and I always bother them with a lot of stuff thats probably trivial to them.
Everyone is super helpful, patient and genuinely kind especially during the pandemic and I couldn’t have asked for a better first job. I am usually very shy and quiet and really want to show my gratitude before leaving.
My coworkers are in their early 30s, one in his 40s and another in her 50s. I have 2 PIs and they are in their 40s and 50s. I am planning to get 2 bigger gifts for my PIs, a medium gift for my postdoc supervisor and smaller gifts for the rest.
All ideas are appreciated, thank you!
https://www.longevity.technology/harnessing-the-power-of-mitochondria-to-beat-parkinsons/?utm_source=reddit&utm_medium=social&utm_campaign=vincere
https://www.longevity.technology/harnessing-the-power-of-mitochondria-to-beat-parkinsons/?utm_source=reddit&utm_medium=social&utm_campaign=vincere
