PNAS paper detailed! The lack of biotin can lead to some forms of severe neurodegeneration, and supplementation with biotin is expected to reverse

January 15, 2021 // --- Carbon-based life is derived from carbidification.
, that is, the coupling of carbon dioxide and sugar in the atmosphere.
is also the key to mitochondrial function.
mitochondrials have five xenonases that have one thing in common--- they are all operated by co-price-connected biotin cofactors.
biotin, also known as vitamin H, is named after the German words "Haar" and "Haut (skin).
this is because even a slight lack can lead to thinning hair, rashes or brittle nails.
In a new study, researchers from research institutions such as Harvard Medical School in the United States found that some forms of severe neurodegeneration, such as alzheimer's disease and Parkinson's disease, can be directly caused by a lack of adequate biotin.
study was recently published in the journal PNAS under the title "Biotin rescues mitochondrial dysfunction and neurotoxicity in a tauopathy model".
picture from Wikipedia.
researchers found this by looking at fruit flies with dementia.
today, fruit flies are actually a good model of Alzheimer's disease or other diseases when given the right genes before anyone sneers.
version of the defective MAPT (tau) gene causes these fruit flies to develop tau protein disease similar to the one that occurs in our own brains.
to delve deeper into tau's neurotoxicity, they studied more than 7,000 fruit fly genes in positive gene screening and then focused on a significant toxic modification of tau R406W mutants.
the gene Btnd encodes biotinidase, which extracts biotin from food sources or recovers biotin from used enzymes.
biocytin, which is simply a biotin linked to lysine.
Btnd isolates biotin from biocytins, or from the connection between biotin and lysine under the role of pyrenease.
when Btnd is weakened in fruit flies, their dementia gets worse.
, their mitochondrials also become deformed and longer.
researchers were able to remedy all of these effects by simply supplementing them with biotin, suggesting that some people with dementia could also benefit.
they were able to fully reveal the mechanisms that connect tau and Btnd functionally.
Although tau variants often bind together in the form of high phosphate tangles and cause damage in the cytogenes of neurons, other tau isomers are located in the nucleus of the cell, where they can destroy the structure of isochromatin.
isochromosomes consist mainly of tightly folded chromosome fragments, where idle genes are tightly stacked together.
destruction of these ordered structures could lead to abnormal expression of many genes.
in the current situation, the Btnd gene on chromosome 25 p appears to have a particularly fragile spot.
, that's how everything these researchers can prove happens.
, however, there are potential ways to see directly whether the Btnd region has been disturbed by the abnormal tau.
by sequencing the bit in full space, the gene can not only be located in the nuclei of the cell, but also reconstruct the folding state of the entire chromosome based on data.
, while such amazing capabilities would undoubtedly be convenient, the bigger question now is, who could benefit from biotin therapy? Recently, we discussed mitochondrial misconduct in the context of cancer and noted that so-called rare mutations are not very rare.
when people are not symptomatic or relatively healthy, it is rare to know what variations they have.
a good example is the biotinase Btnd.
about 100 variants are known to affect the function of the gene in some way.
, these mutations are constantly tracked in a dedicated variation database that records the insertion, repetition, deletion, clipping exceptions, or righteous, senseless, and misalmed changes of Brand.
some of these mutations produce insecuric enzymes, or block them entirely, while others appear to have little effect.
Wikipedia tells us, "Because the disease is inherited in a recessive pattern of the normal chromosome, two copies of a gene in each cell must change so that a person can be affected by the disease."
"Wikipedia is lying to us.
any doctor who says something like that is lying to us.
fact, science is deceiving itself in spreading the myth of this unaffected carrier.
fact, if you only carry a problematic variant, whether you realize it exists or not, you will be affected to some extent.
, for example, if your hair is rapidly thinning at age 23 and your hairline shrinks by 50%, how do you make sure you're not affected? You may measure your biotinase activity in blood or skin cells and find it to be better than 50%, but is this really good enough for you? As mentioned above, mitochondrials have five key biotin-driven carbamolases, which are listed as worthwhile β: acetylCoA carbasin 1 and 2 (or A and B), respectively, for the synthesis and oxidation of fatty acids;
to know how much you are affected by biotinase mutations, you also need to understand the activity of any mutations that may exist in these enzymes that use biotin.
, any further downstream variation in these pathways may also need to be calculated into the rapid dispersion equation.
another dimension of acetylCoA carbase, which is interesting to us.
mitochondrial acetylCoA carbasing enzyme 1 (ACC1) is necessary for the synthesis of thioic acid, another strange cofactor with many similarities to biotin.
For example, structurally similar sulphate-containing biotin and thioic acid are the only co-priced co-factors with parent enzymes: in both cases, they are combined at lysine.
synthesis of sulphate is specially arranged in our cells.
recently found that mitochondrials maintain bacterial-derived fatty acid synthesis to produce preludes to thioic acid.
for now, it remains an open question which of us might benefit from taking biotin and how much biotin we should take.
We do know that if you lack biotin or suspect that you lack biotin, avoid raw egg whites that contain a lot of pure affinity, which binds to biotin.
(Bioon.com) Reference: 1. Kelly M. Lohr et al. Biotin rescues mitochondrial dysfunction and neurotoxicity in a tauopathy model. PNAS, 2020, doi:10.1073/pnas.1922392117.2.Biotin, mitochondria, and dementia: Research reveals a connection