Writing these down so when I inevitably come back. Thanks man!! 1. Von Gierke Disease- "Von Geek Disease" ---Lack of G-6-phosphatase - Hepatomegaly - Hyperlipidemia/ hyperuricemia (fat/protein catabolism) 2. Cori Disease- "Coral Disease" ---Lack of debranching enzyme (alpha-1,6-glucosidase) - Same symptoms as Von Gierke, but with abnormal glycogen structure 3. McArdle's- Muscle --- Lack of glycogen phosphorylase - Myoglobinuria - Muscle cramps on exertion 4. Her's- Hepatic --- Lack of glycogen phosphorylase - Hepatomegaly - Fasting hypoglycemia 5. Anderson Disease- "Anderson Cooper gets straight to the point" --- Lack of branching enzyme -Cirrhosis (fatal) 6. Pompe Disease- "Pompe affects the pump" -- Lack of debranching enzyme specific to lysosomes - Cardiomegaly
@zxcvbnmasdfghjkl99604 жыл бұрын
Thnx alot 💐
@7ranj4 жыл бұрын
U nailed it
@mercy15262 жыл бұрын
copied thanks a lot
@hi-this-is-stephen Жыл бұрын
Great
@huzaifasiddiqui97037 ай бұрын
good work highly appreciated
@waqasashraf65524 жыл бұрын
You can also memorise it as A->B =Andersons Branching C->D = Coris Debranching Others H and H = Hers for Hepatic M and M = McArdles for Muscle
@debbie99624 жыл бұрын
Yooo thanks for this!
@amanali99124 жыл бұрын
This is so helpful
@memersharma15514 жыл бұрын
Ninja nerd 😄😄👍👍
@quratulainmuhammad35224 жыл бұрын
Ninga Nerds here✋✋
@kaouther30763 жыл бұрын
@Brycen Timothy yeah no one gives a damn
@cassiemorgan88985 жыл бұрын
You are officially MVP of the internet. You deserve a streamy.
@robertobobby76462 жыл бұрын
How is it ? Short cut simple hot Water base ( 1 spoon coffee +1 spoon cocoa+ 1spoon sugar it should be little bitter taste mm not sweet (dilute the old fatty acid glucose fructose branching enzyme repair any vital organs damage including incidence of scars wound++(lryny,esapagus lungs liver bile appendix abdomen kidney illium Blood vessels top is the brain cortex eye vessels spinal chor Youghurt garlic compound simple natural repair doctor do it your self ignorance is deadly
@memersharma15514 жыл бұрын
Hyperuricemia is due to increased conversion of Glucose 6 phosphate into prpp which Ultimately contribute to the increased production of the purines and the increased catabokism of purines leads to hyperuricemia that increases chances of gout
@DraakMD4 жыл бұрын
I remember something from uworld vaguely. It said that the increase in uric acid levels in VGD is due to build up of pyruvate that shunts to lactate. The increased lactate competes with the same transporter as that of uric acid. Hence its build up. On the other hand hypoglycemia stimulates epinephrine that activates lipoprotein lipase which then increases FFAs and TGs. Great video, thanks.
@sleepytraveler3693 жыл бұрын
Thanks for that, I was kind of confused because I thought uric acid is from purine breakdown, not amino acids
@coolabe872 жыл бұрын
@@sleepytraveler369 Lactate is a organic anion, and is a counterion for uric acid at the URAT1 antiporter on the apical surface of the collecting duct; thus the more lactate secreted into the urine, more uric acid reabsorbed into the blood
@11ramyatulsitejasria42 Жыл бұрын
glucose 6 phosphate that accumulates is diverted to pentose phosphate pathway increased synthesis of ribose phosphates increases the levels of phosphoribosyl pyrophosphates and enhance metabolism of purines...increases the uric acid..
@hi-this-is-stephen Жыл бұрын
Great
@bronxbomma718 Жыл бұрын
This is correct but it has more to do with the metabolic knee-jerk reaction of shunting G-6-P into HMP shunt pathway, which leads to purine salvage and nucleotide synthesis (all of which require ↑uric acid production). Here is the algorithm: VGD = glucose 6 phosphate deficiency → ↑G-6-P SUBSTRATE accumulates → G-6-P enters HMP shunt pathway → ↑PRPP | NADPH production → PRPP used in nucleotide synthesis and salvage of adenine | hypoxanthine | guanine | thymine | uracil | orotate (but not cytosine) → ↑purines metabolized via xanthine oxidase → ↑hypoxanthine → ↑xanthine → ↑uric acid produced and subsequently consumed by macrophages → GOUT
@fromahappy55364 жыл бұрын
I finally found the person who will get me to 260 in step 1. Thanks man. Not all heroes wear capes
@fromahappy55363 жыл бұрын
@@michigan1085 good luck bro. I'm still in prep. I'm going for pass and fail
@mellnick68382 жыл бұрын
I've always struggled with biochem and all of the pathways seemed to abstract when given in 1st year medicine school, but now I am getting used to them and it is all because of your amazing content! Thank you so much!
@asmaa.rahman61004 жыл бұрын
Thnk you The only video on youtube regarding glycogen storage disease that you feel like listening
@MerrBusra6 ай бұрын
This is the best video about glycogen storage diseases. Thanks a lot!
@kevonmccurdy30983 ай бұрын
By far the best Glycogen storage disease lecture. Thank you👌
@sohniya76815 жыл бұрын
I know you’ve heard this a million times but you rock!!!!!
@maximumovermuslim63373 жыл бұрын
For Pompe Disease, the enzyme involved is acid alpha glucosidase. I remember this by the fact that Pompeiian (Mediterranean) diets include a lot of vinegar (acid). Plus, the fact that Pompeiians were killed pairs conveniently with the "-cide" sound in "glucosidase"
@AlanaOkun4 ай бұрын
I feel like I am going to fly into the ceiling. I have tried so many ways to remember these diseases to no avail, somehow with your video it just clicks. you have the perfect mnemonics. THANK YOU!!!!
@arslanahmed629511 ай бұрын
This is the best video on KZbin. Nothing comes close to it
@hinawajid15753 жыл бұрын
No explanation can be more helpful than this!!! Seriously a great job. Thank you so much...
@jaxsenball89013 жыл бұрын
This was PERFECT, thank you. Except Anderson...I'm gonna have trouble associating Anderson with straight. I'm sure he'd love to see your Anderson slide lol
@frameshift_mutation8 ай бұрын
Debranching enzyme does NOT convert G-1-P to G-6-P, that is done by phosphoglucomutase. Instead, debranching enzyme participates in breaking down glycogen to G-1-P by breaking those 1,6 linkages.
@abdulahadahsan77266 ай бұрын
same I was also thinking this
@ananyajha99312 жыл бұрын
I have been following your channel since medschool. You are so amazing it's surprising 🥺 Thankyou for making these videos and especially for keeping them free 🥺💯
@giceliacarvalho74574 жыл бұрын
This guy is FANTASTIC!!!! I am impressed how he can transformer difficult things in very easy and understandable. Congratulations.
@keinyienchong19914 жыл бұрын
One thing I want to clarify : Lacking of glucose-6-phosphatase can affect glycogenolysis and also gluconeogenesis because gluconeogenesis also involves this enzyme. Then, how does hyperuricemia happen if gluconeogenesis(breaking down of amino acid) is affected? Btw, excellent and straight-to-the-point explanation.
@fernandonateros69893 жыл бұрын
Hyperuricemia occurs due to phosphate sequestration (in G6P) which interferes with the conversion of AMP to ATP, so AMP is degraded (uric acid) and also due to hyperlactacidemia which interferes with the renal secretion of uric acid.
@janvisadhwani5 жыл бұрын
Great job!!👍 You've been uploading all these videos together, all of them about the topics on my biochem test!!! Thank you,helped me a lot!💗
@Moali-ys8vy Жыл бұрын
The best explanation i saw for glycogen storage diseases thank you very much
@thepeacekeeper30132 жыл бұрын
This was the most helpful and summarised med video I've ever watched. Thank you so much. The hard work really paid off. ❤❤❤
@jagmeetsingh012 жыл бұрын
additional difference between Cori and Von Gierke is that Von Gierke results in severe fasting hypoglycemia, whereas Cori results in mild fasting hypoglycemia because gluconeogenesis is compromised in VG but not in Cori
@ailsaarthur13632 жыл бұрын
Not always true though. Please check your comment
@munahiljaved2028 Жыл бұрын
This really made the concepts so easy to understand. Thank you sir!
@RizaAli1198 ай бұрын
Thanks!
@kaitlinsears17676 ай бұрын
COMBANK had a question on this. The question asked what we'd see after exercise, and the answer choices included both "hypoglycemia and myoglobinuria" and "increased serum creatine kinase and myoglobinuria." The correct answer was the increased serum creatine kinase, and the reasoning said that they wouldn't have hypoglycemia since the liver glycogen phosphorylase is still functional (even though the question specifically said after exercise).
@Lo2ay5ory2 жыл бұрын
You just saved my ass from failing this topic. I love you man.
@kaiveen095 жыл бұрын
Thank god you re-uploaded this! Just in time for my step 2. Thank you
@meowmeowgirliecow11 ай бұрын
Please correct me if I'm wrong, but I'm pretty sure there is a mistake in this video. I believe glycogen phosphorylase liberates free G1P until there are 4 glucose units remaining on the glycogen branch, then debranching enzymes have to come in and liberate the remaining G1P. Ultimately it is a different enzyme, phosphoglucomutase, that then yields G6P from the liberated G1P's. In this video, you say that debranching enzyme turns G1P into G6P.
@issacgeorgy82899 ай бұрын
Yeahh
@waleedsiddique70437 ай бұрын
👍
@Ramy-Remedy7 ай бұрын
Exactly I thought the same way but I kinda slap my mind and said "HEY!! .. THIS PERSON KNOWS WHAT HE IS TALKING ABOUT U PEACE OF SH*T .....!"
@muhammadmujtaba7856 ай бұрын
So basically we only get glucose monomer from the branches of glycogen structure not from the linear structure?
@alanharris78784 ай бұрын
Yes…straight up wrong
@254throwbackgaming5 жыл бұрын
You’re getting me through medical school man Thankyou 👏🏿
@HASSAN-ey2qf5 жыл бұрын
Billy Odhiambo Fitness which year u in
@studyturtle71592 жыл бұрын
Sir you are a lifesaver! Biochem never felt this easy.. thank u soo much!!
@jonasheiderking12532 жыл бұрын
thank you sooooo much you saved my pediatrics state exam!!!!!!
@aquadaughterofwater Жыл бұрын
im so happy i finally understand biochemistry, i could cry
@xxtimkimxx5 жыл бұрын
This is really high quality material man. Only a matter of time till your content blows up. Keep it up!
@Fahcina Жыл бұрын
God loves you most! Thank you for the clarity!!!
@mudasiral19563 жыл бұрын
Perfect, fabulous and excellent for ever. I need only one watch and it's fixed in my brain forever.
@Dina-cq9mw4 жыл бұрын
BRILLIANT!!! Thankk you so much you made that very easy to remember!
@CiciliaMercy-px7cv8 ай бұрын
You have saved me in biochemistry sir, thanks 😊💗✌️
@nonot911 Жыл бұрын
Somehow this was really enjoyable to watch despite my hate for biochem. Thank you for distilling the info!!!!!!!!
@RickyCoco1110 ай бұрын
- Andersong is long - Tus pompas make my heart hypertrophy (for spanish speakers)
@592Johno Жыл бұрын
Absolute banger of a video. Really informative and well explained.
@gilfunk_2 жыл бұрын
thank you good sir and thank you to the visiting student on my peds rotation who turned me on to you
@starrysky15885 жыл бұрын
Thankssss a lot.Its my exam tomorrow.You are a life saviour.I wish we had teachers like you in university.
@nystagmus5 жыл бұрын
Thanks This was excellent but debranching enzyme has multiple functions including adding part of the branches to the elongated glycogen chain for glycogen phosphorylase to chop down later on.
@manishayadav97153 жыл бұрын
Best video ever seen ...with best tricks to learn👍👍👍
@varunb2123 жыл бұрын
Who’s this savior of my life ily 😭❤️❤️
@sigmawalin57328 ай бұрын
Anytime i was struggling with a topic...& u always had something to help..thank u❤️❤️
@travelales21903 жыл бұрын
Thnx for making GSDs simple....I was literally going Mad with the topic
@abdullahnafea52795 жыл бұрын
Thank you a million times... may Allah help you just like you help us.
@BrownTingz Жыл бұрын
Also a big difference between Von Gierke disease and Cori Disease is that Cori disease involves muscle hypotonia and weakness; whereas Von Gierke disease only involves the liver. Additionally, Cori disease predisposes patients to hepatic fibrosis due to the short chain glycogen molecule
@MedKeys.9 ай бұрын
Thank you so much for making these videos man, i really appreciate your videos.
@il3354 Жыл бұрын
I can’t express how amazing this is! Thank you, thank you that k you!!!!!
@rayanisam60892 жыл бұрын
I think PUMPE disease is more related with lysosomes disfunction which can lead to (pump) enzymatic component of lysosomes to blood or nearby tissues then tissues damage occur . I don’t know why specifically cardiac muscle is the most effected but we can search about it .
@MedManiac13 жыл бұрын
Von gierke Von at last. last enzyme= glucose 6 phosphatase⬇️, inc glu-6-phos Anderson--branching enzyme⬇️ Cori -- alpha 1,6 glucosidase debranching enzyme⬇️ pompe =debranching (alpha 1-6 Glucosidase) in lysozyme⬇️ (acid alpha- glucosidase) Hers=hepatic gly phos, fasting hg Mcardle=myophosphorylase⬇️ (muscle) gly phos , hg on exersion0
@paulocastro55022 жыл бұрын
nice video, however... when you said coral reef is a branched sea plant was so cringe! It is not a plant! I am amazed no one commented this at least... cheers ;D
@summermorris1841 Жыл бұрын
I came to the comments to see if anyone pointed this out, thank you haha
@dyanan252 жыл бұрын
You're a genius and a lifesaver! Thank you so much!!! God bless you. :)
@DrSRana-xm9qk2 жыл бұрын
Excellent lectures on biochemistry. Thank you so much !
@shamshosho93067 ай бұрын
you're a lifesaver mister ❤❤
@labbad34212 жыл бұрын
12:38 :"A super branched plant that lives in the sea" Me: SPONGEBOB SQU.... wait no
@juanitaj52483 жыл бұрын
this is great info, but keeping up with what your saying is near impossible. .. PLEASE slow down so we have time to put it all together. ty
@Mishti2511 ай бұрын
Thankyouuuuu ❤❤
@sanahayyub51022 жыл бұрын
So easy !!! I struggled to remember these so much!! God bless u dirty
@drnawa93015 жыл бұрын
I love you man. thanks big time!
@Jpgolfmed Жыл бұрын
This was a fantastic video. Props to you good sir.
@josiahlartey87362 жыл бұрын
You killed it,man!!!! Mind blowing
@riichiota2683 Жыл бұрын
Thank you so much. You did a great job.
@TheRaju19694 жыл бұрын
thanks mate. you are god sent. appreciate it.
@shuvoislam81043 жыл бұрын
fantastic illustration bro...looking forward to more from u
@archangel803033 жыл бұрын
Thanks Dirty.God bless you.
@BhavyaKansal184 жыл бұрын
This went straight into the head ^_^ Thankyou so much for this!
@readerviewer86983 жыл бұрын
Great Presentation, Sir!
@GuardiansofEarth Жыл бұрын
That Anderson Cooper mnemonic was complete GOAT.
@ME-lc2zg2 жыл бұрын
The debranching enzyme generates glucose residues while glycogen phosphorylase will generate glucose 1 phosphate.
@myprettygirl913 жыл бұрын
amazing explanation! you are going to be a big reason i pass my boards
@issacgeorgy82899 ай бұрын
Life changing video❤❤❤
@doctor53663 жыл бұрын
Best best best video ever
@raniaramadan38193 жыл бұрын
You are a life saver 🙏🏻🙏🏻🙏🏻
@JujulianaFan2 жыл бұрын
the fact that I've been consistently getting biochem questions right over all other things when it is my god awful worst subject ever... i only have you to thank
@aleenaf.sheikh92553 жыл бұрын
i have an exam tomorrow ...............THANNNKKK YYOOUUUUUUU
@medmar9305 жыл бұрын
amazing video! easy to understand and review! Thank you 🙏🏻
@simoreta3 жыл бұрын
Very informative and easy to understand! Thank you
@shreetomadatta1804 Жыл бұрын
HUGE HUGE THANK YOU!!!
@pswpeng3 жыл бұрын
wonderful video, thank you so much!! i am preparing for Neurology broad exam, and I couldn't get through the metabolic disorders.
@juandiegoneuro3 жыл бұрын
thank you so much. best quality video
@glnerd4 жыл бұрын
In glycogenesis you have two more enzymes glycogenin and branching enzyme
@haderomar56362 жыл бұрын
I do thank you a lot, I'm from Egypt, and also do some mnemonics related to Arab culture , but in time of exams, yours save me really😂
@Veronicaixchel5 ай бұрын
Great lecture 😊
@nusratanwar6199Ай бұрын
excellant🎉🎉🎉
@Rotzfresh89 Жыл бұрын
Thank you so much. Much appreciated !!
@antontk25316 ай бұрын
A->B C-D is easier for me. Andersen- Branching, Cory - Debranching
@piyushsajnani38724 жыл бұрын
This is too good! Thank you
@Umniagh9 ай бұрын
THANK YOU SO MUCH ❤❤❤❤❤❤❤
@mohammedaltayeb62523 жыл бұрын
Very informative and easy... Thanx bro
@aayatprasad81114 жыл бұрын
WAT AN AMAZING EXPLAINATION!!👏🏻👏🏻👏🏻👏🏻👏🏻
@maryamnour88015 жыл бұрын
I love your videos and mnemonics. Thank you!!!!!!!!