THE “TRANSSULFURATION PATHWAY”…AMMONIA and TROUBLE CBS and MTHFR can be very complicated: This is my best explanation at this point in time. This is for those of you who want to go into some detail. If this confuses you don’t worry…just go to the bottom and read… THE BOTTOM LINE. ……………… Regarding the CBS snps...they are not all alike. CBS ENZYME The Cystathionine Beta Synthase [CBS] C699T snp speeds up the CBS enzyme significantly faster than any other of the potential CBS snps. That would make more Cystathionine and the enzyme CBS doesnt go in the reverse direction. CYSTATHIONINE The Cystathionine would need to be acted upon by the enzyme Cystathionine Gamma-Lyase [CGL] which is an enzyme that breaks down Cystathionine into Cysteine and α-KetoButyrate as a part of the “Transsulfuration Pathway”. CYSTATHIONINE GAMMA-LYASE [CGL] The Cystathionine Gamma-Lyase [CGL] enzyme takes part in generating hydrogen sulfide [H2S] which is actually GOOD. HYDROGEN SULFIDE [H2S] is very helpful as it: 1. Suppresses Inflammation 2. Scavenges free radicals 3. Promotes angiogenesis [the making of blood vessels] 4. Suppresses atherosclerosis [possibly] 5. Reduces risk of stroke [possibly] Cystathionine Gamma-Lyase [CGL] also catalyzes the following ELIMINATION REACTIONS: 1. L-Homoserine to form… water [H2O], NH3 [AMMONIA] and 2-oxobutanoate 2. L-Cystine producing… thiocysteine, pyruvate and NH3 [AMMONIA] 3. L-Cysteine producing… pyruvate, NH3 [AMMONIA] and Hydrogen Sulfide [H2S] NOTICE ALL THE… AMMONIA [NH3] that can be produced as a result of the enzyme Cystathionine Gamma-Lyase [CGL]. AMMONIA AMMONIA generated in the LIVER requires TetraHydroBiopterin [BH4] to help get eliminated. The using up of Tetrahydrobiopterin [BH4] is the main problem because the lack of available BH4 ends up causing the production of a lot of “FREE RADICALS” and essentially reduces the availability of BH4 for the making of neurotransmitters. TetraHydroBiopterin [BH4] is a critical co-factor in the making of neurotransmitters. Having the MethylTetraHydroFolate Reductase [MTHFR] C677T and A1298C snps will likely reduce the persons L-Methyl Folate levels and L-Methyl Folate is needed to recycle BH2 into BH4. BOTTOM LINE…as the theory goes If the “Transsulfuration Pathway” is moving along too quickly and generating a lot of AMMONIA then the BH4 gets used up and “Free Radicals” get generated, neurotransmitters don’t get made and the body cannot keep up because the MTHFR mutations interfere with the production of BH4. ..... In regard to Nitric Oxide Synthase [NOS] enzyme BH4 is also needed to couple with Nitric Oxide Synthase [NOS] enzyme to activate Nitric Oxide [NO]. NOS is also involved in ammonia detoxification. When ammonia needs to be eliminated then Arginine doesn’t get converted in to Nitric Oxide, but instead Arginine is involved in the undesirable production of Free Radical species such as: Superoxide Peroxynitrite When NOS is eliminating ammonia there is less of it available to generated Nitric Oxide from Arginine. The detoxification of ammonia therefore ends up wasting the available BH4.
Posted on: Sat, 22 Nov 2014 02:03:26 +0000
Trending Topics
Recently Viewed Topics
© 2015