As I mentioned earlier, there are 6 elongases of which ELOVL1 concentrates solely on saturates, while ELOVL3 is divided between elongating saturates and monounsaturates.
Therefore it is relatively easy to cut off ELOVL3's elongation of saturated VLCFA by simply adding an excess of monounsaturates (the oleic and erucic acid in mustard oil or Lorenzo's oil). This excess therefore divert's ELOVL3's attention and resources into elongating these monounsaturated VLCFA instead. As monounsaturates don't need ABCD1 or ABCD2 transporters, they can be slowly carried across the membrane and eaten up by the peroxisomes even without a functioning ALD gene.
Yesterday I said that nothing can be done about ELOVL1, which only elongates saturated fatty acids, and therefore its attention cannot be diverted. I have been thinking about this, and today I had a "Eureka" moment ! Now I think that there is a way to stop ELOVL1 from extending the length of very long chain saturated fatty acids (VLCSFA). But first I must digress so that you can get a clearer picture of how it all works:-
1. A saturated straight chain fatty acid is just a string of carbon atoms attached to each other like pearls on a necklace, with an acid on the end (like a clasp).
2. Each of these carbons also has two hydrogens which stick out like little diamonds on a ruby necklace.
3. A monounsaturated straight chain fatty acid has one double bond at one specific part of the chain, with two adjacent hydrogens missing. (Its like where two adjacent diamonds fall off the necklace, and it is reinforced at that point by a second bit of thread).
Straight chain fatty acids are classified according to the number of carbons along the chain:
SHORT CHAIN (1-6 carbons) SCFA
MEDIUM CHAIN (8-14 carbons) MCFA
LONG CHAIN (16-20 carbons) LCFA
VERY LONG CHAIN (22-40 carbons) VLCFA
The enzyme system of elongases and desaturases is located on a membrane swirled throughout the cell. Elongases are responsible for elongating the chain by two carbons at a time, and the desaturases just add double bonds at specific points when required.
Therefore these fatty acids are also classified according to how many double bonds they have: e.g: Saturated fatty acids (SFA) with no double bonds, monounsaturated with only one double bond (MUFA), and poyunsaturated fatty acids with two or more double bonds (PUFA), and that's it !!!
Because of the differences in electronic structures imposed by double bonds, we have different enzymes being used for elongation of these different types of fatty acids, resulting in a total of six elongase systems (ELOVL 1 through 6).
BUT: It is only ELOVL1 and 3 that we are interested in, as they directly influence the progression of AMN and ALD symptoms.
Q: SO HOW CAN WE STOP ELOVL1 ???
A: We can't. But we can do something to stop it elongating VLCFA.
I suddenly realised that ELOVL1 doesn't just elongate VLCSFA, it also elongates all the other saturated fatty acids as well. Moreover, the medium and long chain saturates are not handled by peroxisomes but by the mitochondria. (One of the mitochondrias' jobs is to break down fatty acids that are shorter than 22 carbons in length, while peroxisomes are responsible for breaking down VLCFA (whose chain length ranges from 22 to 40 or more carbons).
The peroxisomes only shorten VLCFA down to 18 carbons, then they pass these shortened fatty acids to the mitochondria, which decide what to do with them.
Therefore the solution to the ELOVL1 problem is to divert its attention away from elongating VLCFA into longer and longer chains, by giving it short chains to play with instead.
Any excess short, medium and long chain saturated fatty acids are broken down by the mitochondria; and AMN and ALD sufferers have perfectly working mitochondria, so nearly all of these acids will be disposed of by the mitochondria before even reaching a length of 22 carbons or more (i.e: before they become saturated VLCFA).
In essence, this fools ELOVL1 into diverting all its energy and resources into elongating shorter saturated fatty acids that will be removed by mitochondria before they become a threat. This should reduce the rate of VLCFA elongation and accumulation to almost zero, gaining time for the PPAR-activated ALDR genes to produce transporters that will begin to eliminate the pre-existing accumulation of VLCFA.
By luck, there is already an existing source of medium chain saturates readily available, that will do this job of ELOVL1 diversion.
VIRGIN COCONUT OIL has been used as a cooking oil for decades, and is a great source consisting purely of Capryllic (8:0), Capric (10:0), Lauric (12:0), Myristic (14:0), Palmitic (16:0), and Stearic acid (18:0). Over 50% of it is Lauric acid (12:0), which would need at least five elongations by ELOVL1 before it reached VLCFA length.
Therefore it is very likely that nearly all of this oil would be carried off by the mitochondria despite ELOVL1's efforts to lengthen the chains of its constituent acids. The mitochondria therefore act as the policeman thwarting the efforts of the criminal, making the criminal's best efforts redundant.
If you want to give it a try, they sell Virgin Coconut Oil at Holland and Barrett's. It's not very expensive, and apparently it makes a good flavoured cooking oil. If you use this when frying or as an addition, it may help to reduce the strain on your newly activated ABCD2 system, enabling it to get to grips with those accumulated VLCFA more quickly.
Let me know if you are going to try this, so we can keep track of the results.
Also, if anyone gets their blood tested regularly, please let me know so that I can chart your VLCFA over the past few years. I will send you a copy via email.
All the best to you
ROB.
