Metabolism of galactose: Classic Galactosemia, Galactokinase deficiency
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- Опубліковано 25 кві 2019
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Metabolism of galactose: Classic Galactosemia, Galactokinase deficiency
Galactose is a monosaccharide sugar that is a C-4 epimer of glucose.
Principally there are 2 enzymes involved in the metabolism of galactose, they are galactokinase (GAL-K) and galactose-1-phosphate uridyltransferase (GAL-T). Note there are also UDP-galactose-4’-epimerase (GAL-E) and mutarotase (GAL-M) which are involved in the process but are less clinically significant.
Dietary galactose enters glycolysis/gluconeogenesis by first being phosphorylated to galactose-1-phosphate by galactokinase. This enzyme is present in many tissues but is most active in the liver.
Clinical correlation: A deficiency of galactokinase leads to non-classical galactosemia, a rare and mild galactosemia.
The galactose in galactose-1-phosphate is then transferred to UDP-glucose by galactose-1-phosphate uridyltransferase to yield UDP-galactose and glucose 1-phosphate. UDP-galactose is then converted back to UDP-glucose by GAL-E and the cycle repeats.
Clinical correlation: A deficiency of galactose-1-phosphate uridyltransferase leads to the genetic disease Classic Galactosemia, which is a severe disorder if not caught early by infant screening.
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Great sir.. Ua videos r good for review as they contain evry info plus there is dr mungli who builds concepts that why evry sx is there... It feels great that my cncpts r clear so that ua videos become easy to grab
so...can we say that UDP Galactose has 3 fates :-
1. Become lactose after condensation with glucose (lactose synthase)
2. Become an active donor for compounds like glycoprots, glucosaminoglycans, cerebrosides, etc
3. Get converted to UDP-glucose then further in glycogen synthesis (UDP-hexose-4-epimerase)
Excellent and outstanding lecture
Thank you so much you saved me a lot of time 👍🏻
Thank you for this videos
Bohot Accha sir.
Interesting, because I was told I would have to restrict lactose my whole life.
What product is formed as a result of methylation of galactose?
Is there 100%surity of galactose 1phospate uridyltransferes will become active after 4-5yr of life and we can give milk and other dairy products.
Ah, I think I see how my early life was affected by galactosemia, and how it ultimately disappeared from my life before I reached kindergarten age. I think my body started creating the right enzymes around that time, since I was then able to eat things like cheese. I went a long time not knowing why I was told I used to be unable to consume dairy until my mother actually gave me a name to it because of my older sister's new son.
Are you sure you got galatose?
What is galactosuria
thank you
Welcome!
Nice video sir but I have a question is that -
How galactose 1 phosphate inhibit gluconeogenesis????
Bcz now rxn cannot go further with galactose 1 phosphate so phosphate gets trapped and unavailable for other reactions and hence ATP will also not be formed and gluconeogenesis requires ATP
Hi all,
My newborn baby is suffering from galactosemia disorder, docter stop normal formula milk and start soya made formula milk, isomil (abbot) is best available in my country as I am from India
Whenever we feed baby soya formula milk, immediately or after 5-10 minutes baby start poop (yellow color semi formed type).
I have asked docter for the same and docter provide some good protiotics medicine i.e. protectis and some digestive enyzme but none of them work.
Baby weight gain process is also very slow like after one mnth born my baby weight is 3KG. After birth only 500 gm weight is increased.
Can you please suggest how to stop baby loose motions? Current baby poop approx 15 times a day.
Thanks in advance.
Hi...my son has diagnosed with the same...Wat all are the things we have to avoid other than milk products
Soy milk cause cancer
@@Interestingworld4567 shut up, liar
IsrAze so you don't like to hear the truth idiot?
Hello brother, my son is 49 days old and has been diagnosed with type 1 galactosemia. Can you plz guide us a little about it regarding the diet
Did u use the dm vasudevan book as reference
yeah vasudevan and U satyanarayana