iron engineers in this video we're going to continue on with our discussion about fatty acid oxidation so if you guys already watched the first part of this video it was specifically discussing the process of being oxidation that was occurring in different tissue cells like the heart or like the muscles or the skeletal muscles or even the liver are many many tissues that this could be occurring in but what we didn't get to describe yet is how much energy is actually being produced a little mnemonic that we're going to discuss also to remember the beta oxidative pathways and then other pathways that it can occur specifically with peroxisomes and odd chain fatty acids okay let's get back here and since we've already covered this we don't really need to do a serious serious in-depth topic conversation with us because we've already gone through it so we're going to kind of fly through this really quick guys so if you guys remember we have palmitoleic acid which is again how many carbons 16 carbon fatty acid what do we do we converted him into palma - let politics palma Khalil Cole a so what I'm going to do here is I'm going to say that we're going to have over here palma toil co a how was this reaction happening if you remember there was an enzyme that was doing this reaction that enzyme was called fatty a seal co a synthetase so that an enzyme was called fatty a seal cull a synthetase enzyme and what was this enzyme doing it was taking and adding na coenzyme a but in order to do that it cost energy so because it costs energy what do we have to do we have to specifically utilize the breaking of ATP so what did we do in that step we took ATP and we broke it into ADP and inorganic phosphate and whenever we did that we utilize that energy from breaking that bond to add the coenzyme a to make palmitoyl pala total CO a then we said that there was a specific transporter here and that was called cat1 or you remember you can call it car team palmitoyl transfers one or carnitine acyltransferase one it's all dependent upon preference and you guys remember that it added on a specific molecule and pulled off this coenzyme a so if you guys remember I'll draw the reaction here here's the reaction that's moving into the cell when it moves into the cell what does this molecule do it adds on carnitine but when it adds on the carnitine what does it get rid of as a result it gets rid of the coenzyme a and then it gets brought in as what now this molecule is called fatty or palmitoyl carnitine so it's called palmitoyl carnitine this palmitoyl carnitine molecule what happens with him remember there was another a specific type of transporter that was on the inside of the mitochondrial membrane closer to the mitochondrial matrix while this was on the cytosol Exide this one was called cat 2 or CPT 2 because remember you can call it carnitine acyltransferase type 2 or carnitine palmitoyl transferase type 2 and what does this one do remember that it pushes the carnitine back out to get recycled and then instead it takes and adds on a coenzyme a so it adds on a coenzyme a when it does that what's the overall result of this reaction out of this we get palmitoyl co a then what do we do we went through a series of steps four steps and this was the steps now let me give you a little mnemonic to remember all of these steps so we're going to four steps here let's do that first let's put one two three four okay I like to remember this mnemonic let's write this one out first one is going to be O H Oh tea I like to remember Oh hot so what is oh hot stand for o stands for oxidation hot stands for hydration o stands for another oxidation step and then T stands for Silas and if you guys remember what was happening in each one of these series of reactions if you guys remember in the first step of the reaction we had what molecule coming into here we had and I'm sorry F ad to fadh2 now you might be saying okay well Zack I remember that this one had NAD+ to NADH if they're both getting oxidized how do I remember which one goes which or which one goes first you know there's an F in f ad and there's an F in first so FA ad is going to come first in this reaction and then what was happening in this second step and as a result you guys remember from this reaction from this reaction you get the trans Delta to the oil co a then what then we add it in water and when we added the water into this reaction we got what we got specifically beta hydroxy a co co a beta hydroxy a seal a then what happened he got acted on by an ad positive to NADH to form a carbonyl remember that carving it was a ketone so we called it beta keto is seal cull a beta key to a seal Co a and then what happened and this last step this is where that thiolase enzyme comes in remember the thiolase enzyme what was he doing he was splitting the bit between alpha and the beta carbon when he was splitting between alpha and the beta carbon what happened as a result one thing is I make a new specific Asadi sto a so I make another fatty a CoA but this Fatiha Co Co a is going to be two carbons short remember how specifically what was happening here remember I had a 16 carbon this Paul material palmitoleic away with 16 carbons what's going to happen is I'm going to I'm going to regenerate another fatty a CO CO a but the problem is is that that fatty of CoA is no longer going to be 16 carbons he's going to be 14 carbons but then guess what he'll go back into this process and when he goes back in and gets recycled he'll go through the same process again and break off into another 12 and then into 10 8 6 4 to 0 okay you keep breaking these guys down but what did I tell you was the most significant part of this what was the other product here that we got that was really really important acetyl co a a ctul Co a ok now you're probably wondering ok this was an acetyl co a and let's actually make that green so that we stay consistent with the cola is here Co a with that Sai all group you're saying okay this was an acetyl co a and this is a fatty acyl Co a but I only had one Co a here this violates adds a color into this reaction so this file ace not only cleaves between Alvin the beta carbon to give you a 14 carbon fatty s Co Co a but it also gives you a seagull Co a now the question is is what can happen with that acetyl co a ok if I keep doing that beta oxidation like we talked about before how many acetyl co a is what I produce from a 16 carbon fatty acid as a result I would produce a total of let's take this over here think about it 16 carbons I'm breaking them into two carbon fragments what's 16 divided by 2 8 so I'm producing a total of 8 acetyl co ladies now you guys have to come back for a second and remember the Krebs cycle remember in the Krebs cycle whenever we went through the Krebs cycle you produced a bunch of nadh is in fadh2s remember for one acetyl co a how many nadh did you produce guys you produced two I'm sorry three nadh s3 nadh s and then you produce one fadh2 and you also produce one ATP by substrate phosphorylation four from one acetyl co AG you make three nadh s if you have a acetyl co lays how many nadh is when i make i would make 24 because 8 times 3 is gonna give me 24 then if i have 1 fadh2 for one acetyl co aids a besito quiz would give me 8 fadh2s and this would also give me 8 ATP's ok now the next question is this is only from that acetyl co a what about the nadh is 2m fadh2s that we generate from this part here ok so this happens you're going to undergo how many rounds of beta-oxidation that's the key question remember I told you you're only undergoing seven rounds of beta-oxidation when you undergo those seven rounds of beta oxidation how many fadh2s and how many NADH is am I actually going to make from this reaction from here I'm going to get a specific amount of NADH s and I'm going to get a specific amount of fadh2s from this beta oxidative pathway okay so how many how many fadh2s and how many NADH is am I going to get out of this okay whatever I told you have seven rounds of eight oxidation so in seven rounds of beta oxidation these reactions are only going to occur seven times so I'm going to get seven of these guys and seven of these guys so that's going to give me seven fadh2s and that's going to give me seven nadh --is hmm this is awesome okay but here's the next thing we can't forget about that one ATP that we used up in order to activate the fatty acid so we'll come back to that and we'll tally it all up okay so say we take a 16 carbon fatty acid how much energy would we be able to produce let's tally everything up all right so now if I add together right here look how many nad issues we have we have 24 and we produce from the sua to see the Kawai and then we have seven nadh s that we generated from that beta oxidative pathway so seven plus 24 is going to be a total of 31 nadh s so now from this I'm going to have a total of 31 and a DHS okay I get 8 fa da ch2s from the acetyl co a from these a to see the Caillat and then i get seven fadh2s blooms bait oxygen pathways so 7 plus 8 is going to be 15 then I get 15 fadh2s then you're also going to have to account for the 8 ATP that I made by substrate level phosphorylation so now I'm going to add in those 8 ATP now I got from substrate level phosphorylation but then here's the next thing you have to subtract the ATP that you utilize in order to activate the fatty acid so now we have to subtract one ATP from all of this whenever we get from here we're going to have to subtract this one ATP from it ok so let's put a line across here ok now the next question is one NADH is going to be specifically 3 ATP so if I take this and I multiply this by 3 because that's how many ATP I'll get so what is 31 times 3 that's going to give me 3 it's going to give me how many it's going to give me specifically 93 ATP so this is going to be 3 times 1 3 3 times 3 is 9 I'm going to get 93 ATP from those 31 and a DHS then 15 fadh2s if you remember fadh2 gives you 2 ATP so again what's 15 times 2 that's going to get me how many 30 ATP ok so now I'm going to have 30 ATP here Plus don't forget about the 880 P that I got by substrate level phosphorylation so now I have 93 plus 30 plus 8 so if we take all of that and sum that up what's 93 plus 30 plus 8 so 93 plus 30 plus 8 wearing it 3 plus 8 is going to be 1 carry that 1 over 9 plus 1 is going to be 10 10 plus there is going to be 131 so that's going to give me how many 131 ATP but then as how much we get we'll say gross all right but then don't forget to subtract the ATP that it required in order to what is this for activate the fatty acid so in order to activate the fatty acid so now you have to subtract that one ATP so now what is completely left over as a result for your net gain your net gain of ATP from this process is going to be how much 130 ATP that is absolutely insane to make 130 ATP from this actual 16 carbon fatty acid which is palmitate palmitate kolay that's why they say that specifically fats are such a high concentrated source of energy because they can produce significantly large amounts of ATP okay so from that we were able to calculate how much ATP we generated from the 16 carbon fatty acid we did a little quick review of the beta oxidative pathway now what we're going to do is we're going to come over here and we're going to look at another pathway which is going to be oxidizing specifically odd chain fatty acids okay so now we understand specifically how much ATP that we totally produce and again here's the thing guys certain textbooks in certain literature will say that the NADH actually accounts for not exactly 3 it's like 2. 5 and they'll also say that fadh2 actually doesn't account for - it's actually 1. 5 now if I'm just rounding it to 3 and I'm rounding this fadh2 - - then I'll get 138 TP by rounding it but if we were to be really particularly because some of you guys might get into your textbook and say oh is AK that doesn't make any sense well just in case you look in your textbook if I were to do this is 31 times 2.
5 so I'm making here red if I made this 2. 5 and then I made this one instead of making it 3 I made it 1. 5 then actually after I subtract everything my overall total will keep this one here as the rounded will keep this one here's the rounded but I'll write down below it if you were to utilize those values of 2.
5 and 1. 5 you're actually going to get a hundred and eight a TP okay okay so just in case you guys are looking into your literature you might see 108 ATP from this actual reaction here accounting for the 2. 5 inside of 3 and the 1.
5 is set of 2 and then again if you actually have to subtract this ATP you're also going to have to get about 107 ATP because you're going to subtract it from that point there - ok so now that we've done that now what we're going to do is we're going to take and look at these odd chain fatty acids and how they're being oxidized because they're a little bit different so our chain fatty acids obviously you know our chain means it just means that it's not you know 2 4 6 8 10 12 these ones could be instead of like 16 it could be a 15 carbon fatty acid so let's say I have a 15 carbon fatty acid here if I have a 15 carbon fatty acid and I bring this 15 carbon fatty acid into the actual mitochondria and then as a result I take this 15 carbon fatty acid through rounds of beta oxidation and when I go through this process of beta oxidation I give off a 3 carbon group at the end of it and that 3 carbon group that I form is called propia Niall Co a now here's the thing most of them were going into what when you were undergoing the data oxidative process most of them were producing acetyl co a most of them were being produced into this but what happens is is whenever you're breaking one of those actual last bonds you're giving rid of us acetylcholine and a propia not going that poopie and I'll call a is going to have to go through a different process to be utilized for energy so what happens is the probing on Co a is actually going to be acted on by special enzyme this process is going to require ATP so actually gonna have to utilize ATP in this process and the reason I'm going to have to utilize ATP okay is I'm going to have to do tons some type of special mechanism we're going to add another carbon into this guy so this is I'm going to what I'm going to do is I'm going to take this protein knock away and I'm going to add another carbon into it so in order for me to do that I have to have a special enzyme that can do that process usually enzymes that are adding carbon dioxide's or carbons in are going to be called carboxylation so this enzyme is called propia niall co a carboxylase now what's interesting about this enzyme is this enzyme not only is he catalyzing this step in adding a carbon in the form of usually the co2 so usually going to add a carbon in the form of co2 this enzyme he's stimulating this step right he also a consisting of a very important vitamin called biotin so this is one of our complex B vitamins and it's very very necessary in order for this enzyme to function so the propiedad co a carboxylic is going to be doing what it's going to be adding in any carbon and breaking this ATP in to the ADP and inorganic phosphate and they're also going to be some bicarb that can go into this reaction also so I can even put that if I need to I'll put bicarbonate actually going into this reaction also usually that's the form of how we're actually adding the co2 is we're actually adding in the form of the bicarb now this propia knock away when it reacts with the bicarbonate EP to give you ADP and inorganic phosphate in the presence of this enzyme propiedad kool-aid carboxylase which needs biotin is to be present what it's going to do is it's going to convert this propia malko a and to methyl mal anil : it's going to break it into its called methyl mal anil co-pay and usually there's two different forms technically there's a D form and then what will happen is that methylmalonyl coenzyme u tation reaction where it'll get converted into the L form so there is two different forms of the methyl malonyl-coa one in the D form one of the L form not really relevant here what is relevant is this next step I'm going to take this methyl Melanie Ocoee and I'm going to feed it into a specific step of the Krebs cycle and you guys have probably seen this krebs cycle intermediate before I would assume the enzymes of catalyzing this step is very very dependent upon vitamin b12 but this enzyme is called methyl malonyl coenzyme Allendale co a mutase enzyme and this enzyme what he's going to be doing is he's going to shuffle around the structure to be able to produce a very important molecule in this molecule that he'll be producing is called Sox little Co a and I already told you guys that this guy is a Krebs cycle intermediate so what can happen with that Sox intellect I can't technically take this succinylcholine and I can convert him into two different things one is I can convert him eventually into oxaloacetate and if you guys remember anything about oxaloacetate remember he can be hidden in the form of malate and when he's hidden in the form of malate he can get shuffled out here so here's our let's say this is the form of malli remember that malli can get converted back into oxaloacetate and then what can happen without oxaloacetate if you guys remember there's a special enzyme called Pepsi k and Pepsi k was converting the oxaloacetate into phospho enol pyruvate and then what can happen he can eventually go up to make glucose and if he eventually goes up to make glucose what is that called when I took an on carbohydrate source and turned it into glucose this is called gluconeogenesis so this can lead to gluco neo Genesis okay so now we have gluconeogenesis occurring here which is coming from these odd chain fatty acids right so this 15 carbon or let me put here right next to it what is this again this is a odd chain fatty acid this reaction is producing acetylcholine molecules but as a result it's producing a three carbon fragment called propia knock on a propia Navaho is being acted on by propionaldehyde slaves which needs biotin it's adding them bicarbonate for that extra carbon that's going to be adding in and this reaction requires ATP it produces methyl malonyl-coa which can shuffle between the D form Dexter Audrey into the L form level Road reform not significant in this process but it is acted on by a mutase enzyme a methyl Melanie Oh Co a mutase enzyme okay so now in order for this to happen we require vitamin b12 so b12 is very very important for this reaction to occur another thing that can happen with this occidental co a is you know that you can continue throughout the krebs cycle so if he continues throughout the krebs cycle what's another such significant function of this guy so technically he can go to form ATP he can go into forming ATP right because you can continue to go through the Krebs cycle and be utilized to make nadh and fadh2 take those two electron transport chain and make ATP one more interesting function of this guy he can also be utilized to make porphyrins specifically porphyrins porphyrins and heme groups and the team and porphyrins are important because where have you heard the word team or porphyrin molecules before team clothing so this isn't and this actually can help to make what molecule hemoglobin so you guys can realize how significant this process is any break down in this process of succinylcholine being able to make he more porphyrins which is consistent with in hemoglobin structure it cause detrimental effects on the body one of them you probably heard of its called pernicious anemia which is the lack of it by a vitamin b12 usually due to the lack of a protein produced by our stomach called intrinsic factor okay so this is one of the ways that we can deal with these odd chain fatty acids so the ultimate result of a chain fatty acid oxidation is what I can eventually make sucks in Akko a what are the destinations of socks on taco a1 is I can make glucose through gluconeogenesis the second one is I could actually continue throughout the Krebs cycle and produce ATP and then the other one is I could take it and actually use it as a precursor to make heme groups and porphyrins which is necessary for hemoglobin and again remember that this step here to do that convert the methylmalonic away in to suction Okoye requires vitamin b12 okay now the last thing that we're going to do is we're going to talk about the metabolism of these fats within peroxisomes okay so let's come over here now so this structure is specifically a paroxysm this is a paroxysm now peroxisomes play a very very tiny role but they do have they do nonetheless they are important within the fatty acid oxidation but they're not as significant as compared to the amount of fatty acid oxidation that's occurring within the mitochondria now the steps in this peroxisome are almost exactly identical there's just one step that really differs significantly and that's the one that we'll talk about it's actually the first step of beta oxidation so let's go straight to that step but what here's something that's got to realize this peroxisomes and they do have transporters to bring specific types of fatty acids into this structure which is kind of interesting but again this is important that these do have transporters on the peroxisome to bring the fatty acids in so let's say that I bring these fatty acids in so let's say I put F fatty acids but specifically this should be what it should technically be this fatty acids that I'm going to have here I should have a cull a on them right but they're going to come in fatty a seal cull a in once we have this fatty of silico a and what's going to happen you're just going to undergo the similar steps of beta oxidation but with the exception of one specific step in the first step if you guys remember remember we had F ad and f ad was reacting with this patio silikal and picking up the hydride ions and when it was picking up the hydride ions it was being converted into fadh2 and then if you remember fadh2 generally and the actual mitochondria will take those electrons to the electron transport chain to be utilized for energy in the peroxisomes are something different they actually take oxygen okay plus water and react that with the fadh2 and what's happening is the fadh2 has those electrons right he's going to react with those guys and regenerate the F ID as a result of when this oxygen and water gain those electrons they get converted into a new molecule which is called h2 o2 which is called hydrogen peroxide so this molecule is called hydrogen peroxide hydrogen peroxide is extremely you know it can be extremely dangerous on our body and the reason why is because it can act like a free radical and it can cause a lot of damage to our DNA and proteins and cause a lot of oxidative damage so what happens is inside of these peroxisomes they have special enzymes to deal with that hydrogen peroxide so say I take this hydrogen peroxide I bring it down here what happens is is I take this h2 o2 this hydrogen peroxide and I'm going to have a special enzyme this enzyme is called catalase so let's add here an enzyme and this enzyme is called catalase and what this catalase enzyme is going to do is it's going to work your specific reaction to break up the hydrogen peroxide and convert it back into oxygen and water so I'm going to take this hydrogen peroxide and convert it back into oxygen and water and then what will happen they'll go back in remove that if they remove the electrons off the fadh2 and convert back into hydrogen peroxide that's the whole purpose and that's because paroxysms do generate a lot of hydrogen peroxide for certain types of cellular functions but this is the main significant difference is that within the first step of fatty acid oxidation inside of the proximal real difference is instead whenever they generate these fi da choosing the peroxisomes they don't have an electronic transport chain they take and drop it on to oxygen and h2o to make hydrogen peroxide which can perform certain cellular functions with inside of the body but to prevent this from causing excessive damage to ourselves because it can act like a free radical what happens we take that hydrogen peroxide and we reacted with catalysis when we react it with a catalase enzyme that converts it back into oxygen and water okay now last thing I want to talk about guys in this videos I want to talk about certain types of disorders and which certain enzymes within the body are not functioning correctly the most common one that I want to talk about that is extremely dangerous is actually called medium-chain Asil dehydrogenase deficiency we actually refer to it as M CAD deficiency and what happens in in CAD deficiency is that they're obviously not having an enzyme called what medium-chain Aseel dehydrogenase there will be a CoA dehydrogenase enzyme is very very important with inside of this pathway if they're lacking this enzyme think about what can happen inside of the body so let's come over here to this actual pathway over here imagine we're lacking a specific enzyme in this pathway specifically right around this point this a CO CO a dehydrogenase if we're lacking that then we can't generate this trans Delta - in oil and we can't carry out the rest of the data oxidative processes so what happens is these fatiah Co Co wastes are building up and they start building up and building up and building up within the liver as these fatty of silica waves build up in the liver they cause extreme damage of the body right so what's some of the signs of someone who has MCAD one is they're going to have lipid accumulation so they're going to have high lipid accumulation mainly in the tissues of the liver this is usually going to be in the liver but it can occur in different cells but one of the dangerous ones is lipid accumulation in the liver and again what is this for this is for MCAD deficiency okay that's one dangerous thing the next dangerous thing is they have hypoglycemia these individuals also have hypoglycemia because it throws off other reactions in the body due to this MCAD deficiency and you know what can happen whenever you have severe hypoglycemia and lipid accumulation in the liver it can cause extreme vomiting so some of the signs of these people is that they're going to have signs of vomiting they're going to be extremely sleepy very very sleepy and they can get so bad to where it can actually put them into comatose okay that's one another one that I want to mention is over here with the peroxisomes now with the peroxisome remember I told you these are these transporters are responsible for bringing certain types of fatty acids into this actual peroxisome there is a condition in which there is a deficiency within those transporters if you have a deficiency in these transporters can you bring the fatty acid in no and so the lipid start actually accumulated in the blood and whenever the what is this actual condition called this condition is called x-linked Adreno Luco dystrophy now as you can tell it x-linked what does that mean it's more common in boys usually it's a common and more common in boys before the age of 10 so it's more common in boys before the age of 10 years old now what happens with these people well one thing I told you they don't have the transporters to bring the fatty acids in not only does that can happen that they can't bring the fatty acid in and undergo beta oxidation but what can happen is that these actual fats can start building up in the blood so as these fatty acids so some of the actual signs of this person is that they're going to have high fatty acids in the blood hyperlipidemia when there's high fatty acid levels in the blood this can cause disastrous effects on the body some of the effects that it can actually cause and cause of visual disturbances it can actually cause visual disturbances it can also cause significant behavioral disturbances and it can also cause coma and even significant death and I'll obviously have untreated it can lead to death now another thing that you can actually derive from this name look at this name that can actually can come from them which you can explain some of the neurological problems you hear the word adrenal leuco leuco means white so there's white matter within the actual central nervous system in this condition there is actually not going to be as heavily myelination so you know the white matter is actually myelinated axons and actually the adrenal leukodystrophy they're actually not having myelination of their axons which can cause significant damage another one is adrenal their adrenal gland is also going to be damaged because the adrenal gland being damaged that also can affect a lot of hormones being produced from that organ so there's actually two Drna leukodystrophy which can occur in boys below the ten years old or MCAD deficiency oh one other thing with MCAD deficiency let's come back over here for a second because they have high lipid accumulation right and they're lacking a specific enzyme that can actually break down these fatty acids you want to avoid this person from ever having to have to utilize or break down fatty acids so what is the treatment for these people usually one of the treatments is that you try to give them a high carb diet put them on a high carb diet to avoid and stay away from fats if you put them on a high carb diet which avoids taking in the fats that's going to help to prevent this breakdown of fats the next thing that can also you want to do is you're going to want to specifically be able to actually have decreasing time intervals between eating and that's going to help with this process also last but not least there is one last disorder we're not going to spend a lot of time but there is a condition which you can't make these peroxisomes and whenever you're not making these peroxisomes it can cause multiple disastrous effects on the body not just with relates to fatty oxidation oxidation but other different types of conditions and this whenever you're not able to make these peroxisomes functionally it's called Zellweger syndrome okay high injured so in this video we covered a lot of information we went over just basically review of the beta oxidation pathway in the activation the transport we went over the energy tally with respect to just rounding I remember I rounded it within this video to give us the simpler idea of how much ATP but if you did take into consideration the 2. 5 in the 1.
