Cell Biology | Tay-Sachs, Fabry, Gaucher, Niemann-Pick Disease
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- Опубликовано: 30 июл 2024
- Official Ninja Nerd Website: ninjanerd.org
Ninja Nerds!
In this lecture Professor Zach Murphy will be teaching you about the structure and function of lysosomes. We will begin by discussing the normal function of lysosomes and will conclude with lysosomal storage disorders in a USMLE step 1 review. These conditions include Tay-Sachs, Fabry, Gaucher, and Niemann-Pick Disease. We hope you enjoy this lecture and be sure to support us below!
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If everyone in the world were like Zach, the world would be heaven! Thank you for making 3 videos in 3 days!
But... We can't have good things
I agree 🧘🏻♀️🤍
I just started study medicine one week ago and already, I do nothing else.
These videos are probably going to be the reason, I pass my exams properly and going to be a knowledgeable doctor.
Thank you very much for all you've done and all the videos to come!
This channel is changing my life, no joke. 🙏🏻 I would be SO HAPPY if you did a lecture all about the Lymphatic system - especially how it works in conjunction with blood vessels - because I have lymphoedema and there is NO good information about this disorder out there. Thank you just a million thanks for what you guys do. So excellent 🏆
i can't believe this man is actually real!!! a genius indeed
Zach you are amazing! So close to 900K subs mate! Whenever I see your videos, I am so grateful. 3 Videos in 3 Days . In Soccer you would be Hat Trick Hero. In Basketball you would be MVP
Making STEP 1 studying so much easier. Thank you so much!
I love all of your videos, Zach! So helpful in all my courses I’ve taken thus far and I’m just 2 semesters away from graduating, then onto graduate school 😃 you’ve made learning an enjoyable experience. Do you think you could make a video series of ways that YOU study? Since you clearly have retained so much information 😃 thank you!
Thank you so much
You make my knowledge concrete!
I have been studying biology with you.
I hope you feel our happy 😊
Be safe from Covid19! Be always happy!
I clap after each video. Thank you so much for doing this, you're a blessing on this app! Virtual hugs
Such a simple, to the point, awesome video!!! You make learning fun. Keep going
This man is a Angel for medical students.
Thank you so much.
Keep it up.
I would have never made it through my first year of med school without your help. Thank you for all of the great videos.
Loved it! Thank you, Zach, appreciate your contribution!
This is the first video I've seen on RUclips without a dislike! Thanks Ninja!
The best lecture I've ever seen! Thank you so much!
Please make video on some tips for studying and How to make effective notes and how to keep maintain focus.. I know there is a lot on videos on RUclips but you are deep explainer so it will be helpful.. Thanks in advance 😇
Yessss please 🙏🙏
Best thing is to make notes form videos lecture or you can do another thing you can also make notes form the different books material and also from google and makes different diagram and writes in your own words as it will be easy for your revision.
@@drirfan72941 thanks you! 😊❤️
he already did a studying video on his channel 2-3 weeks ago
This is wonderful. Everything I wanted to know and understand about the lysosome, I found it here. Thank you very much😊
20 years after biology semester, recapitulation with Zach.Thank you Ninja Nerd🙏
Dude, you are a hero! We all love you!
Thanks to you and your team!
I’m currently taking a paramedic class thank you for your videos. Tremendous help!
For a future video topic can you do a video on med math . We were learning about IV piggybacks with dopamine drips of different Concentrations. Some of your clear explanation would really be helpful!
stunning!! Love all the lectures. Bless you all.
you are so awesome to watch!! Thank you for making and sharing your super personality with us!! love ur videos!!
Zach I want to say from the bottom of my heart you represent the best of humanity! You are giving so much and asking for so little. You are an angel mate! (Have tears in my eyes)
I’m a third year phd student at Notre dame working on npc disease. Loved the video, and it was really impressive.
can’t wait to watch this!
OMG you are simply amazing, i enjoyed the full lecture. Thank you very much for this!
U are just AMAZING Professor !
Thanks you ninja nerd for the best you have done.
Thank you for making it easy for me to understand 🙏🏾💞
great video! I like you explained end to end process
Thank you for making this video.. you make things simplier.
I will say only one sentence "you are amazing zach"❤️❤️❤️
Thank you so much for this knowledge🙂
Great as usual... good job !
Thank you so much for being just the way u r
Amazing explanation as always
you saved my many hours of reading
God Bless you Zach
Excellent Lecture! Please provide me with the links for all of your biochemistry and molecular biology lectures
Great video!! As always
Thanks a lot for your hard work👍🙏
Thank you zach bro!! 😊
Wow extraordinary it's makes me feel better understanding thankyou so much sir
it was an awesome lecture but I wished that you explained other sphingolipidoses. like krabbe farber etc. still was a nice roundup video. loved it.
Loves your teaching style...❤️❤️🙏🙏
You are a good teacher, thank you.
Thank you sir for making amazing lactures
can never thank you enough for this !
I memorized a chart for this section because I didn't understand the purpose of these enzymes in lysosomes. So basically what I think I'm getting from this video is that all the sphingolipidoses are due to defects in lipid metabolism. These lipids are all derivatives of sphingosine and are part of the cell membrane whether they are sphingomylelins, ceramides, cerebrosides, or gangliosides. They are not the same as TG that undergo beta oxidation, they are broken down in lysosomes and are either released in the cytoplasm or ECM via secondary lysosomes. They are used in the liver for LDL degradation, in all cells that undergo autophagocytoses, and in the WBC for pathogen degradation. Fabry is the only XR disease the rest is AR, Fabry and Gaucher usually present in adulthood whereas the other diseases present during childhood. Only gaucher disease may present with increased risk of infection due to pancytopenia, differentiating Tay Sach from Niemann Pick via hyperreflexia vs hyptonia and no hepatosplenomegaly vs hepatosplenomegaly.
You make the lesson very easy.
Thank you so much for this wonderful video❤
Don't often see anything on LSDs like this. Thanks for raising the profile. Woild like to see something on lysosomal membrane proteins especially NPC1 (Niemann-Pick 1 protein. Amazing small writing!
Many thanks. It was really helpful.
Thank you Zach
You are amazing...Love your videos...
Really,u r awesome n your videos r also awesome n very much helpful....thank you so much zach....lots of love (India)😍😍😍....please upload videos of all the cell organelles🙏
only 100k away from the million lets do this ninja nerds!
This helped a lot sir.... Thank you so much...
I love how you are repeating facts with having these little breaks, so one has a change to suggest frist
Amazing sir keep making videos on cell biology lots of love from Pakistan ❤❤
Very good explanation..
Thank you for this amazing video
Thanks again and again
I'm always waiting for your vedio guys 🌸
Thank you, Sensei
Congrats zach bro you are near to 1million
YOU ALL ROCK THANK YOU
Brilliant video
Wey neta que te amo, explicas super bien todo
thank you so much
Hi ... Thanks a lot for the videos
Sir also deliver lectures on remaining cytology such as details description on nucleus . Cell membrane. And so on👍👍👍👍👍👍👍
thank you so much !!
As an old engineer I had a brain freeze about 1:00 in. Then about 10:00 when you talk about Receptor Mediated Endocytosis a few brain cells came out of hibernation connecting stress analysis and vibrations and I nearly had a stroke. I couldn’t even relate to nonlinear optics. Holey-Moley I guess I better start with basic biology. However, after a third go of it some synapses are forming.
Amazing!
Kindly do a video series on how to read and interpret X-rays and other radiographs. Thanks
You r amazing.. plaease can explain the hormone and its role on immunity
Thank you so much
Wow❤️
Very nice
Thank you very much
Awesome 👌
thanks Zach
Make more video pls it inspires me to study well :>
Hi Zach,
Thank you so much for the amazing explanation, I have a question to share with you regarding the NPC disease (Niemann pick type c)!
Out of this explanation, what is the possible consequences of following a low carb & high fat diet and Intermittent fasting on NPC cells
and how consuming different kind of fat can increase or slow down the process of the disease in the cell level?
Sir pls post more videos on pathology , pharmacology and microbiology....
We r waiting....🙏🙏❤️
Very nice content.
Hello zach, will the white board image of this lecture be uploaded on your patreon?
Nice information
Thank you
Thx man ..!!
Wow Zach you are explaining stuff that we used to think that they are non-doable in medschool and never understand 😂 lysosomal storage diseases are 💀💀
god damn i love you guys so, so, so much
You are a blessing
Thank you so much for the video, such a clear concise explanation. However, does anyone know why Tay Sachs and Nieman Picks present so similarly besides the hepatomegaly while Gaucher and Fabry dont. Also why does gaucher seem to affect the bone (is it because the enzyme that its deficient in is only found in that bone?)? What is the reason behind Fabry causing Angiokeratomas while the others are less likely? These might all be moot points, but Im just curious to know if there is an actual answer or if its just by experience that these are the most common presentations?
His liiitttlle bit deeper is very deep😌
Thankyou sir
thank you
Excellent informative vedio
Danke!
We want more video on organells of cell I mean on ribosome on golgi aperatus etc
Very useful