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

@zxcvbnmasdfghjkl9960

4 жыл бұрын

Thnx alot 💐

@7ranj

3 жыл бұрын

U nailed it

@mercy1526

2 жыл бұрын

copied thanks a lot

@stephenharris-dr

7 ай бұрын

Great

@huzaifasiddiqui9703

Ай бұрын

good work highly appreciated

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

@debbie9962

3 жыл бұрын

Yooo thanks for this!

@amanali9912

3 жыл бұрын

This is so helpful

@memersharma1551

3 жыл бұрын

Ninja nerd 😄😄👍👍

@quratulainmuhammad3522

3 жыл бұрын

Ninga Nerds here✋✋

@kaouther3076

3 жыл бұрын

@Brycen Timothy yeah no one gives a damn

You are officially MVP of the internet. You deserve a streamy.

@robertobobby7646

Жыл бұрын

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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

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.

@sleepytraveler369

3 жыл бұрын

Thanks for that, I was kind of confused because I thought uric acid is from purine breakdown, not amino acids

@coolabe87

2 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..

@stephenharris-dr

7 ай бұрын

Great

@bronxbomma718

5 ай 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

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!💗

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!

No explanation can be more helpful than this!!! Seriously a great job. Thank you so much...

I know you’ve heard this a million times but you rock!!!!!

This was the most helpful and summarised med video I've ever watched. Thank you so much. The hard work really paid off. ❤❤❤

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"

Thank god you re-uploaded this! Just in time for my step 2. Thank you

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.

@issacgeorgy8289

3 ай бұрын

Yeahh

@waleedsiddique7043

16 күн бұрын

👍

@Ramy-Remedy

14 күн 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 .....!"

@muhammadmujtaba785

4 күн бұрын

So basically we only get glucose monomer from the branches of glycogen structure not from the linear structure?

I finally found the person who will get me to 260 in step 1. Thanks man. Not all heroes wear capes

@fromahappy5536

2 жыл бұрын

@@michigan1085 good luck bro. I'm still in prep. I'm going for pass and fail

This is really high quality material man. Only a matter of time till your content blows up. Keep it up!

Thnk you The only video on youtube regarding glycogen storage disease that you feel like listening

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 🥺💯

This is the best video about glycogen storage diseases. Thanks a lot!

BRILLIANT!!! Thankk you so much you made that very easy to remember!

Sir you are a lifesaver! Biochem never felt this easy.. thank u soo much!!

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

Excellent lectures on biochemistry. Thank you so much !

This guy is FANTASTIC!!!! I am impressed how he can transformer difficult things in very easy and understandable. Congratulations.

You’re getting me through medical school man Thankyou 👏🏿

@HASSAN-ey2qf

4 жыл бұрын

Billy Odhiambo Fitness which year u in

amazing video! easy to understand and review! Thank you 🙏🏻

Absolute banger of a video. Really informative and well explained.

Very informative and easy to understand! Thank you

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This is the best video on KZread. Nothing comes close to it

I love your videos and mnemonics. Thank you!!!!!!!!

Thank you so much for making these videos man, i really appreciate your videos.

Couldn’t get easier than this! Thank you 😊

Great crash course on the topic, thanks!

- Andersong is long - Tus pompas make my heart hypertrophy (for spanish speakers)

This really made the concepts so easy to understand. Thank you sir!

The best explanation i saw for glycogen storage diseases thank you very much

Best video ever seen ...with best tricks to learn👍👍👍

Perfect, fabulous and excellent for ever. I need only one watch and it's fixed in my brain forever.

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

@ailsaarthur1363

Жыл бұрын

Not always true though. Please check your comment

Somehow this was really enjoyable to watch despite my hate for biochem. Thank you for distilling the info!!!!!!!!

You just saved my ass from failing this topic. I love you man.

Thank you so much. You did a great job.

There are some important factual errors or omissions in this video. Debranching enzyme does not convert glucose-1-p to glucose-6-p. Only one branching enzyme (alpha-1,6-glucosidase) releases glucose directly from a debranched collaged. Some other points. Von Gierke's causes: * Lactic acidosis as a consequence of pyruvate accumulation in impaired gluconeogeneis * Gout secondary to high glucose 6 phosphate / HMP shunt Cori Disease findings: * No lactic acidosis (again important in differentiating GSD) * Presence of cardiomyopathy (secondary to abnormal glycogen accumulation) Pompe Disease: * No lactic acidosis * Cardiomyopathy (not cardiomegaly, secondary to accumulation of glycogen) * macroglossia (differentiated from Cori Disease) McArdle Disease: * Lactate in blood but flat venous curve (differentiated from Von Gierke) Debranching enzymes --> cardiomyopathy, differentiated by macroglossia

@evanhanoun

Жыл бұрын

first aid mentions cardiomyopathy and cardiomegaly for Pompe disease

I love you man. thanks big time!

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.

Thank you so much. Much appreciated !!

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

This is too good! Thank you

Thank you so much for your hard work!!!

God loves you most! Thank you for the clarity!!!

You killed it,man!!!! Mind blowing

Great Presentation, Sir!

im so happy i finally understand biochemistry, i could cry

fantastic illustration bro...looking forward to more from u

I can’t express how amazing this is! Thank you, thank you that k you!!!!!

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.

thanks mate. you are god sent. appreciate it.

thank you good sir and thank you to the visiting student on my peds rotation who turned me on to you

I needed this so much... thank you

So, so good, thank you so much!!

You have saved me in biochemistry sir, thanks 😊💗✌️

Thank you so much.

Just awesome.....Thanks a lot

WAT AN AMAZING EXPLAINATION!!👏🏻👏🏻👏🏻👏🏻👏🏻

This was a fantastic video. Props to you good sir.

This went straight into the head ^_^ Thankyou so much for this!

outstanding , thank you

Marvelous, thank you sir!!

THis was great, thank you!!!!

Amazing explanation !

Very informative and easy... Thanx bro

Awesome explaination

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.

finally I'm able to remember it! thanks!

Very helpful! Thank you!

Thanks Dirty.God bless you.

amazing explanation! you are going to be a big reason i pass my boards

Who’s this savior of my life ily 😭❤️❤️

amazing, God bless you

Thnx for making GSDs simple....I was literally going Mad with the topic

thank you so much. best quality video

So easy !!! I struggled to remember these so much!! God bless u dirty

What a great work

Thank you so much!

Just Incredible.❤

wonderful video, thank you so much!! i am preparing for Neurology broad exam, and I couldn't get through the metabolic disorders.

HUGE HUGE THANK YOU!!!

KEEP UP THE GOOD WORK...

"The Cori Tree can't lose it's branches" is another way to remember Cori Disease!

sir outstanding work🙏

THANK YOU , VERY VERY MUCH

12:38 :"A super branched plant that lives in the sea" Me: SPONGEBOB SQU.... wait no

That's helpful , THANK YOU

Thank you very very much!

Anytime i was struggling with a topic...& u always had something to help..thank u❤️❤️

Thank you!

Great work

Thank you for this. Really.

Thanks a lot.