Ok, So I figured I would take the time to post this as it seems to be a rather unique opportunity to explore this objectively utilizing research equipment here at work. Granted this probably bears little weight as the sample size is n=1, regardless it may have some carry over.
The Background: I've had Lyme's Disease and Babesia Duncati for at least 4-5 years, it went undiagnosed for at least 3 of those years in which I experienced significant inflammation and was characterized in a myriad of traditional Lyme/CFS symptoms. I suspect that this brought about damage to my mitochondrial cells as vascular dynamics are quite unique as evidenced by near Infrared Spectroscopy and looking at relative concentration changes of O2 (HbO) and deoxy Hb (HbR).
The Paradigm: A typical exercise physio cycling paradigm in which exertion is varied and relative concentrations of HbO and HbR are observed. Optodes were placed on vastus lateralis (lateral) quadriceps.
Periods are coded as follows:
E- End of warm-up and start of moderate intensity
R- Rest
H- High intensity period
C- Cool down
S- Stop
Relative Concentrations.png 94.39KB 2 downloads
Red- HbO
Blue- HbR
Green- total Hb
In comparison here is a more normal response.
Normative Data.png 117.89KB 1 downloads
In my opinion it seems that there is a bottleneck somewhere in my Electron Transport Chain, most likely COX, and as a result instead of oxygen decreasing it paradoxically increases after the lactic acid threshold. I still need to do some more research but ran across this article which explains this phenomena.
Mol Cell Biochem. 1997 Sep;174(1-2):7-10.
Diagnosis of defects in oxidative muscle metabolism by non-invasive tissue oximetry.
Abstract
The dynamics of oxygen delivery and utilization are examined in a variety of mitochondrial disorders during rest, exercise and post exercise. We used a non-invasive optical technique to measure the oxygen consumption in the exercising limb in normal subjects and 5 patients with cytochrome c oxidase deficiency. We also examined 6 patients with MELAS and MERRF syndrome. We measured near-infrared spectra of hemoglobin in the gastrocnemius muscle during treadmill exercise. Normal subjects demonstrated a sustained deoxygenation during exercise, indicating an efficient utilization of delivered oxygen. Patients with cytochrome c oxidase deficiency demonstrated consistent oxygenation during exercise indicating an under utilization of delivered oxygen. Patients with MELAS and MERRF syndrome showed similar under utilization of oxygen during exercise. Non-invasive tissue oximetry during exercise demonstrates specific abnormalities in a variety of mitochondrial disorders, indicating abnormal oxygen utilization, and will be a useful addition to the clinical investigation of such disorders.
I am trying to come up with a suitable form of therapy and I believe Methylene Blue would be an ideal candidate given it increases COX expression. Any thoughts??
Low-concentration methylene blue maintains energy production and strongly improves survival of Leigh syndrome French Canadian skin fibroblasts.
Abstract
Leigh syndrome French Canadian (LSFC) is a recessive disease caused by mutations in the LRPPRC gene (leucine-rich pentatricopeptide repeat containing protein). These mutations induce a cytochrome c oxidase (COX) deficiency resulting in episodes of acute acidotic crisis that will often lead to death. There is no effective treatment. Methylene blue (MB) is a redox dye that increases COX content and activity in vitro and in vivo suggesting that MB could prevent and treat LSFC. In this study, the protective effect of low-concentration MB was tested on two LSFC cell lines, including LSFC-F1, homozygous for the mutation A354V, and LSFC-F2 a compound heterozygous for the mutations A354V and C12775STOP. MB effect on metabolic activity was assessed on both LSFC cells in stable and acidotic conditions. For LSFC-F1, results showed that metabolic activity drastically decline after 96 hours in both conditions but not LSFC-F2 and normal cells. MB completely prevents the decrease of metabolic activity in LSFC-F1. Intracellular ATP content was also measured in both culture media. After 96 hours in acidotic medium, ATP content was almost completely depleted for both LSFC cells. Interestingly, MB completely restores ATP content in LSFC-F1 and LSFC-F2 cells. Finally, MB strongly improves the survival of both LSFC cells.
Edited by Infinite1, 01 May 2014 - 04:36 PM.