The name of the disease that has deficiency of the enzyme called sphingomyelin ace it’s called niemann-pick disease one of the sphingolipid o C’s it’s also one of the lysosomal storage diseases if you are a medical student and you actually enjoy studying the lysosomal storage diseases you’re weird vitamins are essential therefore you have to eat them in the diet vitamins are cofactor for enzymes and today’s topic mister biotin is a cofactor for carboxylation reactions because it adds co2 biotin is b7 therefore it’s water-soluble vitamin deficiency is more likely toxicity is less likely relatively speaking but luckily biotin deficiency is really rare fight is the same thing as b7 is the same thing as bios vitamin H or coenzyme r or you can call it anti egg-white injury factor.
Because egg whites actually interfere with b7 bio availability because egg whites contain evidence which avidly binds biotin we have discussed anti vitamin C’s before in this plane is called biochemistry definition chemical kabobs with inhibits absorption of vitamins such as evident protein in a raw egg whites not egg yolk egg yolk is fine egg white is a disaster when it comes to biotin inhibits the absorption of vitamin b7.
Egg white has avidin which will decrease the absorption of vitamin b7 which is Biden but technically to be honest you need to eat lots of raw eggs like 20 or 30 per day in order to develop vitamin b7 deficiency the main problem with eating raw eggs is Salmonella infection baby why do we need 5 min for dehydrogenase enzymes why we need to ripe a flavor for redox reactions nice and is the same thing Panasonic acid for Co a pie reduction for trans emanation and carboxylation what is decarboxylation to remove the co2 what is b7 is for carboxylation to actually add a co2 because vitamin b7 is a carrier and a donor of co2 b9 to transfer one carbon unit what are the benefits of biotin.
We have direct benefits and indirect and direct Memphis you can trade vitamin b7 deficiency which is extremely rare and you can treat something called multiple arboxylase deficiency that’s a genetic diseases and it can be treated using bite like lots of it indirect cofactor or carboxylation reactions co2 fixation ecause by it is a carrier as well as a donor of co2 biotin okay it has to acquire and to carry soot first and thenit can donate it you have to have some money first before you can donate vitamin b7 is the same thing it has to carry co2 first before it can donate it o let let’s make biotin carry co2 you add hco3 because hco3 I haven’t I don’t know if you have noticed it contains co2 so now we have carboxy biotin which is biotin plus co2 now biotin can go everywhere and give the co2 to many substrates and the enzyme is responsible for that it’s gonna be carboxylase and this is very good for protein synthesis and cell replication as well as a gluconeogenesis fatty acid synthesis fatty acid oxidation etc what are the sources of biotin you have vegetables we have media VST have egg yolk but not egg white and milk as fine art of e-resources enriched food and vitamin b-complex supplements that you get in the pharmacy functions of vitamin b7 again carboxylation.
There is another option for pyruvate it’s called pyruvate carboxylase here is pyruvate here is oxaloacetate ok the enzyme is a carboxylate it needs co2 whenever you need co2 you can count on biotin because it comes carrying co2 water give the co2 from from hco3 and ATP it also requires manganese so here is mr. pyruvate by pyruvate dehydrogenase we can get Estill Co a but by pyruvate carboxylase we can get oxaloacetate and this one needs biotin because it’s a carboxylate its co2 let’s go back to the TCA cycle you know it’s a 3-2 citrate of a keto good rate of smoke way successful rate malate oxaloacetate back to acetyl co a this is a carboxylase it requires biotin because whenever you need co2 ask biotin because it has co2 however pyruvate dehydrogenase needs 5 cofactors you remember the Teflon company TfL n curl what does the teeth fireman what’s the F F ad what’s the L lipoic acid was the end.
Vitamin b7 building up fat is the job of insulin breaking down fat is the function of glucagon and your lovely pancreas can influence either now we have talked about building of fat now let’s break down the fat triglycerides into cholesterol and free fatty acid go through will become whatever Callisto 6-phosphate gluconeogenesis thank you so much now let’s metabolize fatty acid to get some energy baby beta oxidation will give you assets of kuwait is a cycle ATP and you will get ketone bodies which can lead to high anion gap metabolic acidosis and acidosis can interfere with your nerve endings with your synapses and synaptic nerve transmission that’s why many people who are on the keto diet are tired there are no solutions in life there are trade-offs anyone who rolls up on you until you oh this dot is the best in the world nonsense anything has pros and cons we’re still breaking down the fat fatty oxidation we three steps activation quarantine salt and beta oxidation of this even seeps in saturated fatty acid so fatty acid fatty acyl Co a fatty acyl CoA will leave the cytoplasm to the mitochondria in a shuttle and then beta oxidation s local a TCA cycle n ketone bodies and glucagon.
Biochemistry playlist and succinylcholine enter into the TCA cycle succinylcholine aid from a rate malate oxaloacetate back to SLE co 8 and then acyl Co a can become glucose and this process called gluconeogenesis look at this here’s a sloka glucose from glucose to s Luka a this is a glycolysis but from acetyl co a back to glucose this is gluconeogenesis I love this word I just love it it’s awesome look at this Genesis which is formation knew you knew no glucose formation yes indeed because you have made glucose from fatty acids and this is new the old way is to make glucose from carbohydrate but to make glucose from fatty acid this is neo Genesis baby now let’s talk about this enzyme methylmalonic a mutase it requires vitamin b12 so if there is a patient who has vitamin b12 deficiency they will accumulate the methylmalonic.
It is rare causes primary causes secondary causes primary increased consumption of frogs it has to be lots of eggs not just one raw egg dana has to e lots of them but please don’t eat raw eggs because of salmonella infection there is a risk eviden will avidly bind by you 10 decreasing its absorption many interesting can cause the biotin deficiency was very rare because biotin is widely available in food secondary due to deficiency of by you today’s biotin days who name these things. prescribe antivirus for a very long period of time it’s a very good idea to add vitamins to the patient’s regimen what’s gonna happen in vitamin deficiency decreased function of pyruvate carboxylase which will lead to decrease gluconeogenesis decrease function of prop único a carboxylase which will decrease gluconeogenesis decrease function of acetyl co a car boxes which will decrease fatty acid nthesis also when you have no glucose and no energy dermatitis alopecia color scientist enteritis diagnosis decreased vitamin b7 in blood and urine treatment if the patient has no biotin give the patient biotin some pearls for the pros pharmacological doses of vitamin b7 can actually interfere with essays that measure the T SH and therefore it can mimic Graves disease the patient doesn’t have Graves but you are fooled why because you knew give vitamin b7 they can give you a result that’ similar to Graves disease even though the patient doesn’t have Graves disease question of the day this is question number 6 the previous five questions are this glorious playlist what enzyme or enzymes are deficient in cases of multiple carboxylase deficiency let me know the answer in the comment.