Guest guest Posted January 27, 2006 Report Share Posted January 27, 2006 Steve, I'm no expert, so I can only quote the experts. Here's a few links with brief quotes that might help answer some of your questions and give food for metabolic thought. http://www.spiralnotebook.org/inthelongrun/index.html , MD: " Although fatty acids are not used at the beginning of exercise, they become increasingly important after 20-30 minutes of endurance exercise, and after an hour, they represent the major energy supply. Consequently defects in lipid metabolism give rise to symptoms after sustained activity. " http://www.spiralnotebook.org/splitthedifference/index.html Symptom comparison chart of carbohydrate defect (McArdles) with FAO defect (CPT def). Note that " second wind " is associated with carbohydrate defect rather than FAO defect. This makes sense, given the way muscle uses fuel. http://www.spiralnotebook.org/batterynotincluded/index.html , MD: " It should be noted that a tissue in which ATP levels are depleted is probably dead tissue. Even in the metabolic myopathies, the muscle seldom reaches this critical state. What does happen, however, is that most of the support pathways are overworked with the production of unwelcome by-products which are probably responsible for the symptoms. " [ Adds interesting dimension and complexity to interpreting individual response to exercise.] Agreed, your lipomas would surely be an accumulation of fatty acids--that's nature of the beast, isn't it?---but do " we " (the royal scientific " we " ) really know that all your symptoms are coming from the same source--MERRF? There are a significant number of cases where people have two defects in different metabolic cycles or are carriers for two different defects or have one primary defect and are also carriers for another, or have one genetic defect and some other acquired non-genetic pathology that may impact on response to exercise. Also cases where a defect in one metabolic cycle secondarily impairs another metabolic cycle. Many possibilities.....including anomalies. Maybe you're just an anomaly? :-) Small comfort, I know. Barbara > I guess it's safe to say that there are a wide variety of metabolic " locations " for the > breakage when mito function goes wrong, either IN the mitochondria (resp. chain) > or in various transportation across membranes. That's why I'm so interested in the > specific clusters for SOB reports. And why I'm describing my own cluster, i.e. > looking for similiarities and differences amongst us, that might especially lead to > better coping. > > I've always assumed that I have a FAOD, partly because of my " second wind " > symptoms and partly because I also have MSL whose physical presentation is > large lipomas (filled with brown-fat cells, as currently understood, I think). Not much > is known as to why these cells " sequester " so, instead of the usual body storage > patterns (i.e. more muscle-interspersed), in fact I guess that's the central issue. > These are proliferating but not " cancer " because it doesn't metastatize. But they > certainly enlarge, and even start in new places, so I question the VALUE of making > that distinction. After all, it DOES kill by mass effect, just like many cancers. > > But back to another point, for me this second wind could be due to FAO working > correctly, but the " easier " glucose metabolism could be broken, if I put your > comments together correctly. So perhaps my lipomas are the result of glucose > problems after all? Hmmm. I also have " unexplained " syndrome X (is that the right > word? aka the metabolic syndrome?) and questionable glucose tolerance numbers. > And fatty liver (hepatic steatosis), and high blood triglycerides. CLEARLY it's all > related, but I don't understand enough of HOW. > > By the way, this reported second-wind behavior is for someone with MERRF (me). > Seems that the ME (myoclonus epilepsy) part of MERRF implies some problem > with nerve conduction, either sensory or motor. Sensory problems would relate to > PN, and motor problems would be the single body jerks. These happen for me > every once in a while, typically once at night when very relaxed. Funny, when I was > little (and the jerks were rare) I thought everyone had these. Does anyone know > more about PN causation in mito, as compared to diabetic PN? I think I read > somewhere about demyelinating action, related to certain fats (as in the " Lorenzo's > Oil " disease). For ME, I would suppose it's the same underlying mechanism, but in > motor nerves. > > Please anyone, comment on above if inaccurate, or you have more information, > etc. > > Steve D. > Some questions > > > > > > > > > > > > Hi, I just joined your group. I received your welcome letter and > > > > responded. I would like to know if anyone can answer some questions? > > > > Is this where I would go and ask them? One of my questions is about > > > > breathing. Any suggestions. My doctors say, my lungs are clear and > > my > > > > heart is not causing this shortness of breath. But upon reading some > > > > sites about this mito disease it affects breathing. I feel short of > > > > breath upon any type of exertion. I have not seen my neurologist yet > > > > to discuss this disease. We just got back the biopsy and blood work > > to > > > > determine this is what I have. I also have trouble getting around > > when > > > > walking, between the breathing and the stiffness and hurting > > muscles, > > > > even pushing the shopping cart kills me. Any ideas? > > > Quote Link to comment Share on other sites More sharing options...
Guest guest Posted January 27, 2006 Report Share Posted January 27, 2006 The way I remember is that fat is hard to lose, so it must burn last, alass, leaving me overweight. :-) Steve wrote: >The way I " use " to remember the order, is to realize that glucose is a simple sugar, that burns first (like kindling, fast but not producing a lot of energy). The metabolic route that fatty acids take, in order to get burned in the mitochondria, is fraught with hazards, because the fat molecule is much larger, and also needs lipid solubility (at least) for transport. Worse, the long chain fatty acids need carnitine assist. All worth it of course, because (a) the body can store a LOT of fat, and ( burning fat is high energy. But of course, Barbara is inserting into the order an even-earlier source of energy, whereas the order we're discussing is between two substance categories, both blood-borne. If I understand it, muscle glycogen is converted to glucose at some point before cellular utilization, so the " hazards " should be similiar for blood glucose. > >I guess it's safe to say that there are a wide variety of metabolic " locations " for the breakage when mito function goes wrong, either IN the mitochondria (resp. chain) or in various transportation across membranes. That's why I'm so interested in the specific clusters for SOB reports. And why I'm describing my own cluster, i.e. looking for similiarities and differences amongst us, that might especially lead to better coping. > >I've always assumed that I have a FAOD, partly because of my " second wind " symptoms and partly because I also have MSL whose physical presentation is large lipomas (filled with brown-fat cells, as currently understood, I think). Not much is known as to why these cells " sequester " so, instead of the usual body storage patterns (i.e. more muscle-interspersed), in fact I guess that's the central issue. These are proliferating but not " cancer " because it doesn't metastatize. But they certainly enlarge, and even start in new places, so I question the VALUE of making that distinction. After all, it DOES kill by mass effect, just like many cancers. > >But back to another point, for me this second wind could be due to FAO working correctly, but the " easier " glucose metabolism could be broken, if I put your comments together correctly. So perhaps my lipomas are the result of glucose problems after all? Hmmm. I also have " unexplained " syndrome X (is that the right word? aka the metabolic syndrome?) and questionable glucose tolerance numbers. And fatty liver (hepatic steatosis), and high blood triglycerides. CLEARLY it's all related, but I don't understand enough of HOW. > >By the way, this reported second-wind behavior is for someone with MERRF (me). Seems that the ME (myoclonus epilepsy) part of MERRF implies some problem with nerve conduction, either sensory or motor. Sensory problems would relate to PN, and motor problems would be the single body jerks. These happen for me every once in a while, typically once at night when very relaxed. Funny, when I was little (and the jerks were rare) I thought everyone had these. Does anyone know more about PN causation in mito, as compared to diabetic PN? I think I read somewhere about demyelinating action, related to certain fats (as in the " Lorenzo's Oil " disease). For ME, I would suppose it's the same underlying mechanism, but in motor nerves. > >Please anyone, comment on above if inaccurate, or you have more information, etc. > >Steve D. > Some questions > > > > > > > > > > > > Hi, I just joined your group. I received your welcome letter and > > > > responded. I would like to know if anyone can answer some questions? > > > > Is this where I would go and ask them? One of my questions is about > > > > breathing. Any suggestions. My doctors say, my lungs are clear and > > my > > > > heart is not causing this shortness of breath. But upon reading some > > > > sites about this mito disease it affects breathing. I feel short of > > > > breath upon any type of exertion. I have not seen my neurologist yet > > > > to discuss this disease. We just got back the biopsy and blood work > > to > > > > determine this is what I have. I also have trouble getting around > > when > > > > walking, between the breathing and the stiffness and hurting > > muscles, > > > > even pushing the shopping cart kills me. Any ideas? > > > Quote Link to comment Share on other sites More sharing options...
Recommended Posts
Join the conversation
You are posting as a guest. If you have an account, sign in now to post with your account.
Note: Your post will require moderator approval before it will be visible.