Introduction
Hey there, ninja nerds! Today, we're diving deep into the fascinating world of lysosomes and lysosomal storage disorders. By understanding the function and structure of lysosomes, we can better appreciate how their dysfunction leads to disease. This article breaks down the life cycle of lysosomes, their essential functions within the cell, and the implications of their malfunction, particularly how this relates to various lysosomal storage disorders.
What are Lysosomes?
Lysosomes are membrane-bound organelles found in eukaryotic cells, often referred to as the "suicide bags" of the cell due to their capability to break down waste materials and cellular debris. They contain a variety of hydrolytic enzymes, which are critical for digesting all kinds of biological materials.
Structure and Function of Lysosomes
Lysosomes are spherical organelles that are rich in enzymes, capable of digesting a range of biomolecules. Their main functions include:
- Degradation of macromolecules: Lysosomes digest proteins, carbohydrates, lipids, and nucleic acids.
- Recycling cellular components: They help remove and recycle damaged organelles in a process called autophagy.
- Immune defense: Labeling pathogens for destruction by combining with phagosomes to facilitate the breakdown of engulfed materials.
Formation of Lysosomes
The creation of lysosomes involves several key steps, starting from the synthesis of enzymes in the rough endoplasmic reticulum (rough ER):
- Transcription and Translation: Instructions from DNA are transcribed into mRNA. The mRNA is then translated into enzymes (proteins) at the ribosomes.
- Synthesis in the Rough ER: The ribosome binds to the rough ER, translates the mRNA, and synthesizes proteins that get modified in the rough ER, where sugar residues like mannose are added through a process called N-linked glycosylation.
- Processing in the Golgi Apparatus: The proteins are packaged into vesicles and sent to the Golgi apparatus, where they undergo further modification and additions (like phosphorylation of mannose) to ensure they can function as enzymes.
- Formation of Lysosomes: This prepares the proteins to be transferred to emerging vesicles that will become lysosomes, equipped to digest materials.
Mechanisms of Lysosomal Function
Lysosomes engage in two main processes to bring materials into the cell for degradation:
Endocytosis
Endocytosis refers to the cellular process of taking in external substances. There are two main types:
- Phagocytosis: This process is key for immune cells that engulf pathogens like bacteria. Once engulfed, a phagosome (vesicle containing the pathogen) fuses with a lysosome, allowing hydrolytic enzymes to break down the pathogen.
- Receptor-Mediated Endocytosis: Specific molecules like LDL bind to cell surface receptors, forming vesicles that internalize the bound materials for processing by lysosomes.
Autophagy
Lysosomes also play a role in autophagy—the degradation of the cell’s own components. This process involves:
- Encapsulation of damaged organelles or proteins within a membrane to create an autophagosome.
- Fusion of the autophagosome with a lysosome to degrade the encapsulated material.
Lysosomal Storage Disorders
When lysosomes fail to perform their function properly, it can lead to a group of diseases known as lysosomal storage disorders (LSDs). These disorders typically result from genetic mutations affecting lysosomal enzymes.
Key Lysosomal Storage Disorders
-
Tay-Sachs Disease:
- Enzyme Deficiency: Hexosaminidase A.
- Accumulated Substrate: GM2 gangliosides.
- Symptoms: Neurodegeneration, hyperreflexia, and a characteristic cherry-red spot on the retina, without hepatomegaly.
-
Fabry Disease:
- Enzyme Deficiency: Alpha-galactosidase.
- Accumulated Substrate: Ceramide trihexoside.
- Symptoms: Pain in hands and feet, skin lesions (angiokeratomas), and kidney disease—this is X-linked recessive, affecting mainly males.
-
Gaucher’s Disease:
- Enzyme Deficiency: Beta-glucosidase (glucocerebrosidase).
- Accumulated Substrate: Glucosylceramide.
- Symptoms: Splenomegaly, bone pain, and the presence of Gaucher’s cells characterized by crumpled-tissue-paper appearance in macrophages.
-
Niemann-Pick Disease:
- Enzyme Deficiency: Sphingomyelinase.
- Accumulated Substrate: Sphingomyelin.
- Symptoms: Hepatosplenomegaly and neurologic symptoms with a cherry-red spot on the macula.
Conclusion
Understanding the function of lysosomes is crucial for grasping the pathophysiology of lysosomal storage disorders. By delving into how lysosomes are formed and their critical roles within the cell, we can better comprehend how their malfunction can lead to serious genetic conditions such as Tay-Sachs, Fabry's, Gaucher's, and Niemann-Pick diseases. Knowledge of these disorders is essential for anyone looking to master cell biology or prepare for board examinations, including the USMLE. So, ninja nerds, let’s keep learning and exploring the intricacies of human biology! For a deeper understanding of cell structure, check out our summary on Understanding the Structure and Function of the Cell: A Comprehensive Overview.
For related organelles, you might also find Understanding Lysosomes: Structure and Function Explained helpful. Additionally, explore Understanding Peroxisomes and Their Role in Paroxysmal Disorders to learn about another critical organelle involved in cellular metabolism.
hi ninja nerds in this video today we're going to be talking about the function of lysosomes and then the
lysosomal storage disorders before we get into this video please hit that like button comment down the
comment section and please subscribe also down in the description box with links to our
facebook instagram patreon account go check those out all right engineers let's get into
it all right ninja so when we talk about lysosomes we talked a little bit about
them their overall kind of design their structure their basic function in the video on the
structure and function of this of the cell but what we need to do is kind of dig in a little bit deeper into the
lysosomes and what they do the reason why is if we understand the function of lysosomes in a little bit
more of a greater detail it's much much easier to understand the lysosomal storage disorders
so let's start off talking about how lysosomes work well the first thing that you need
to know before how lysosomes work is how they're actually made we briefly talked about this in the structure and
function of the cell but if you remember we start off when we're making
proteins because that's what pretty much the components of lysosomes is these enzymes
and enzymes are proteins so we start off with remember the dna what does it call whenever you go from
dna to mrna it's called transcription so we take and we make
mrna from dna via the process of transcription in the nucleus then out of the nucleus what happens it
moves via the nuclear pores and binds with the particular structure out here which is called a
ribosome the ribosome will then begin to translate parts of that protein let's represent that as like this brown thing
coming out this portion of it so it's translating the mrna and making proteins
what happens is this ribosome will get then kind of like a magnet sucked onto the rough endoplasmic
reticulum so you see this structure here that's this maroon organelle here this is called our rough
endoplasmic reticulum the mrna will get translated into proteins taken up into the rough
endoplasmic reticulum you know what the rough yard does it pushes
this protein once this actual ribosome moves over to the rough er so look here's going to be our
mrna right there it's getting translated and it's translating this protein into the rough er
as it goes to the rough er let's imagine here is going to be our protein you know proteins are special
because on one end they have a amino end and on the other end they have a
carboxylate and all the little components that make them up are called amino acids what the rough er does is it
folds this protein in a particular way and then you know what else it's really important this is very relevant
it takes a particular enzyme and uses the enzyme in the rough er and adds on a sugar residue
to this amine group of the protein you know what this little sugar is called it's called mannose it's called a
mannose sugar but this process by which the rough er adds the mannose sugar onto the
in structure the nitrogen of the protein is called in linked glycosylation what is this
called in linked glycosylation this is a very special
activity that the rough endoplasmic reticulum performs then
after it does this process it then packages that actual protein with this mannose
sugar into a vesicle look at this vesicle here's that vesicle coming off of the rough endoplasmic
reticulum what's going to be inside of it you're going to have your protein
and what's going to be coming off the in the nitrogen of that actual protein a sugar residue right the mannose group
now from the rough er we have to send this to the golgi very important especially for us usm
release step one there's very important proteins that are found on these vesicles going
from the rough endoplasmic reticulum towards the golgi
what are these proteins they guide this like a magnet like zoo right to the golgi what are these called
these proteins that guide this vesicle to the golgi are called cop two proteins cop2
proteins they bind to the vesicle of the rough er and take it to the golgi now
in the golgi this protein with the amino sugar gets kind of budded into this so here we're going to
have that protein right there and then again what's coming off of that protein off the nitrogen
the mannose this actual golgi what is this organelle here this is called your golgi
it does something very special everything does something special i guess right
let's again take our protein here we have it we're going to the golgi as you go to the golgi the golgi does
something else it adds a phosphate group onto this mannose sugar so here let's actually
draw kind of our protein here here's our protein we have the carboxylate here we have the nitrogen in
here and then again what's coming off of this you're going to have your
mannose sugar and then off that manual sugar what does the golgi add on here let's actually do a
special color here let's use orange what the golgi does is is it actually adds in
a phosphate group you see this phosphate here we're gonna add this into this reaction
and then what we're gonna do is we're gonna add this phosphate onto the mannose sugar on the sixth carbon of it
this is a very important reaction this is called phosphorylation right this is called phosphorylation
the reason why this is important for your usmles step one is because there's a disease called eye cell
disease it's called eye cell disease and this disease is usually whenever the
golgi is unable to add a phosphate group onto the mannose of proteins
and so because you don't have that phosphate why is that important why am i stressing on that here's why
from the golgi guess what in order for these vesicles to butt off and become lysosomes so from the golgi
we're going to butt off a vesicle and we want those vesicles containing this protein to become a
lysosome we need this phosphate group onto it if that phosphate group is not
on that mannose of the protein we will not make functional lysosomes enzymes so because of that we need that actual
phosphate to be there if it is not we will not make lysosomes that is why it's important
okay so now to quickly recap we took mrna we translated into proteins stuffed it
into the rough er it added a sugar residue used the cop2 to send it to the golgi
golgi then added a phosphate onto the mannose that phosphate when it's added onto the mannose sugar that's bound to
the glue to the actual protein molecule it's then destined to become
lysosomes if that process of phosphorylation does not occur this is called
eye cell disease okay we've made our lysosomes once we've made our lysosomes we now
need to know what the heck these little buggers do there's very important processes called
endocytosis so what is this called endocytosis endocytosis is the process by which you bring
substances or macromolecules into the cell there's two main types that are
important with respect to the lysosomes the first one is called phagocytosis so it's called phagocytosis
and this is very very important within your white blood cells you know macrophages
neutrophils which are your white blood cells they love to engulf take in what types
of things pathogens what is a very very dangerous pathogen like one of them there's many
of them but one that we encounter is bacteria and what happens is these bacteria can
get engulfed by white blood cells like macrophages and neutrophils and brought
into the cell when they're brought into the cell via this process of phagocytosis guess what it does
it takes and actually creates a little vesicle what like an endosome if you will and
this endosome contains the bacteria in it now this actual endosome when it's with
respect to phagocytosis it's given a special name it's called a phagosome
it's called a phagosome what happens is that phagosome which contains the pathogen what type of
molecule in here it contains the bacteria it then gets combined with a lysosome what are these
molecules these are called your lysosomes once the lysosome combines with the
phagosome now look at this we're going to have here's the lysosome with all these nasty
little enzymes and then over here we're going to have the phagosome which combines
and it's going to have the bacteria here's what i want you to remember these lysosomes contain very dangerous enzymes
we already talked about how we synthesize these enzymes but we need to talk about what these enzymes
are they contain very nasty hydrolytic enzymes that just means it breaks down
substances by adding water into it and these hydrolytic enzymes they can be proteases
nucleases lipases glucosidases which means it breaks down proteins carbohydrates nucleic acids and
lipids you know what a bacteria is made up of all those macromolecules
so guess what the lysosome will do it gonna frick it up it's gonna come in there and start
breaking down the proteins the nucleic acids the lipids all of that stuff until it's all
digested that is what the lysosome will do so the lysosome will
actually do what it'll break down the bacteria and then in certain cells like
neutrophils guess what that'll do with that lateral lysosome which contains all the
broken down maybe remaining products it'll then take and spit it out of the cell
so that's one thing that you need to know with the lysosomes very important when it comes to these white blood cells
the next one is called receptor mediated endocytosis what the heck is that that's a big name right
receptor mediator so we need an example of a receptor and then a molecule that binds to that receptor
the most classically used examples is ldl which is a lipoprotein bad cholesterol
and then there's a particular molecule that that ldl binds to the ldl receptor pretty straightforward right
this happens on the liver when ldl binds onto this ldl receptor in the liver there's little proteins you know these
these special little proteins called clathrins what are they called here we're going to
put look at a c these are called clathrins and what the clathrin molecules do is they bind to the
membrane which has the ldl receptor and the ldl bound to it and creates a little invagination called
a clathrin-coated pit then it continues to pull pull pull pull until it invaginates and creates a
vesicle or an endosome so then look what will happen as a result of this through this process of what is it called
receptor mediated endocytosis we're going to take in this little structure here
so now we're going to have a vesicle and that vesicle is actually going to be called a
endosome that's what we like to call it an endosome just like a phagosome is an endosome
it's just a special name from the process of phagocytosis what does the endosome contain within it
it has the ldl receptors and then it also has the ldl molecules bound to it
guess what happens with this endosome the endosome has little like pumps on it little proton pumps
and these little proton pumps they start pushing in lots of protons making this environment very
acidic you want to know what that protons do whenever they get pumped into this
environment they make the bond between the ldl receptor
and the ldl very weak so that they disassociate from one another so then as a process of
this look what will happen as a result of this as the protons get pumped in look at
this let's kind of see what happens here you're going to get kind of like a
little bubbled vesicle here like a little bubbled one here right
and then what happens is as the process of these protons coming in it separates the ldl receptors
away from the ldl molecules then after that process guess what happens we pinch this off
and separate this into two separate vesicles so now we're gonna have that and we're gonna have this and then
you're going to have this vesicle here which contains the ldl receptors and this vesicle here which is going to
contain the ldl molecules guess where the ldl molecules will go
well they're a type of macromolecule they will get sent to this lysosome and when they get sent
to the lysosome guess what they're going to do the lysosomes contain specific types of
lipases and sphingomyelinases and all these different enzymes that'll break down the cholesterol the
phospholipids the proteins all of the stuff within the ldl molecule
so what else will break down not just the bacteria but it'll also break down in this
example since we're using this example the bacteria and the ldl particle the question is what the heck happens to
this little receptor here just to finish the story because i know you guys are begging to know what
happened right you know our cells are so cool you know what they do with these ldl molecules
they send them to get recycled so they get recycled and sent right back to the cell membrane to
rebind into the plasma membrane and express these ldl receptors so that the next ldl combined
bring it in and do the same process now again you need to understand what the fate is
of this lysosome we understand it breaks down macro molecules from
a particle matter via phagocytosis or breaks down macromolecules from receptor mediated
endocytosis once it breaks all these things down the important thing to remember is
what happens with this actual lysosome so let's say it's performed its function it's broken down all these
macromolecules and bacteria once it's done that it's called a secondary
lysosome there's two things that can happen with this secondary lysosome it can spit some of
these molecules out into the cytosol that maybe can be used for certain metabolic pathways
or you know what else it can do it can go to the cell membrane
bind with the cell membrane and release some of these molecules out of the cell via a process called exocytosis
so now you guys should understand with a quick quick recap lysosomes break down macromolecules
within a bunch of different ways right primarily via these hydrolytic enzymes it can break down macromolecules
for the process of phagocytosis break down molecules from the process of receptor immediate endocytosis
after it breaks it down it can spit some of these molecules out into the cytosol or it can spit it out of the cell via
exocytosis the last thing you need to know about the lysosomes sometimes our cells we have organelles
that they just reach their prime they're kaput they're old geezers they're done
like mitochondria and specific types of proteins within the cell when these things have been worn out
let's actually kind of let's tag them as that we're going to say that these are
worn out organelles okay or different you know protein elements whether it be mitochondria
cytoskeletal structures whatever when they're worn out they get tagged with like specific molecules
and you know what this tag does it says hey these these organelles are done it's time for
them to go so our cell creates a little membrane around them
it creates a little membrane around it so now i'm going to have a membrane around these organelles
so what will i have in here well my example was i had a mitochondria and then i also had a protein molecule
here what happens is once you do this you take and engulf your own organelles that
are done worn out this is called a auto phagosome
it's another type of endosome this autophagosome guess what it's going to do
it's going to get taken to the lysosomes and when it gets taken to the lysosomes
guess what the lysosomes are going to do they're going to break down the mitochondria what's the mitochondria
made up of it has lipids it has proteins it even has a little bit of nucleic acid in
there it's going to break all that down it's going to break down the protein elements
all of that stuff will get broken down so not only does it break down bacteria and ldls
but this process by which can break down worn out organelles what is this called this process is called we're going to
note it up here this process where we break down these elements here this is called auto
phagy very important process this is very important so that we understand the function of lysosomes now
that we understand the function of lysosomes how they carry out these activities
now let's talk about lysosomal storage disorders whenever these normal functional pathways
aren't occurring normally all right so we know that lysosomes have these very nasty hydrolytic enzymes
right so if we took a look at these lysosomes we understand that these lysosomes
release a lot of hydrolytic enzymes and again there's different kinds like lipases
there's what's called uh proteases nucleases and these are just like the broad category of them there's
glucosidases and there's many other different types of enzymes but these are your hydrolytic
enzymes and they're responsible for breaking down macromolecules so again these are just
the general understanding of your hydrolytic enzymes the reason why all of this is important is that there's
genetic conditions in which the lysosomes aren't being able to break down
those macromolecules whether it be a lipid a protein a nucleic acid or a sugar molecule
and that leads to these common diseases which are very high yield for u.s emily step one
what are some of these well one of them is tay sachs disease now taste accident has a particular enzyme a particular
hydrolytic enzyme that as you can see here i have the shape of him which is going to tell you
pretty much the actual enzyme this enzyme is called hex aminodace
a this hexaminodase a is responsible for breaking down a particular type of macromolecule
and this macromolecule is called a gm2 ganglioside ganglioside why is this important if there is a
deficiency or loss of this enzyme there is an accumulation of this gm2 ganglioside
if that gm2 ganglioside that substrate macromolecule builds up in particular tissues
it's going to cause damage to that tissue another important fact here is that this
disorder this tay sachs disease is autosomal recessive okay it's autosomal recessive
so you need two mutant copies in order to have this condition now what tissues does this gm2
ganglioside usually accumulate within the primary area that it accumulates in is in the central nervous system and as
these actual gm2 gangliosides accumulate within the central nervous system it leads to
progressive newer neurodegeneration so it's going to lead to neuro degeneration
and this neurodegeneration is the classic sign that you're going to see now what are some of the ways that we
can see this neurodegeneration well one of these ways is hyperreflexia so
increase deep tendon reflexes that's one big one another one is called hyperacusis where
they have very very uh like sensitivity to hearing very pain a very sensitive intense
sensitivity to hearing and the other one here is they have seizures
because of these deposits within the tissue and here's a very big fact on the retina they have an accumulation
of this tissue near the macula and this leads to what's called a cherry red spot on the
macula near the retina that's an important fact now lysosomes are very very abundant
and are actual what's uh what types of tissues well obviously white blood cells is a big one but what do we notice was
another one the liver here's one big thing that you need to remember tay sachs disease
is one of the few lysosomal disorders where there is no hepatomegaly as you can see most of
these have livers around them this one has no hepatomegaly
and the reason why i want to under help you guys to understand this is that usually taste accesses and neem
and pick disease can somewhat present similarly but one of the big differences between tay sachs
and neiman pick is that there is no hepatomegaly in taysac's disease
all right beautiful we know we understand taste axes hexadeficiency accumulation of gm2 ganglioside
autosomal recessive neurodegeneration no apatomegaly what about the next one
fabrics disease fabry's disease is actually a deficiency or a loss of alpha galactosidase so what is this
enzyme here called it is called alpha galactosidase alpha galactosidase breaks
down one heck of a sun of a gun of a substrate and this substrate is called
ceramide trihexacide trihexacide so in this condition which this condition is autosome sorry
x-linked recessive this is x-linked recessive so you're going to see this more commonly in what
sex male or females male so in this disease there is a mutation where there is a loss of alpha
galactositis which creates an accumulation of serum trihexacide within particular tissues
what are these particular tissues this one's a son of a gun so i tried to find a mnemonic for these
ones and the big thing that you can remember is fabry
c fabry c so f for foamy urine and that helps you to remember that this is usually due to
kidney failure okay so this will usually be due to the renal failure okay that's the first one the second one
is a and this is for angiokeratoma angio keratomas these like
little red spots that you can develop within the skin b is for burning pain
in the hands and the feet this is indicative of neuropathy r is for really dry
skin really dry skin and this is indicative of a condition where you don't make a lot of
sweat called hypohydrosis the next one is that this is only going
to be seen in males only males have a y chromosome and the last one here is that it's also
going to be accumulating within the heart tissue and this leads to cardiovascular
disease this is fabric disease deficiency or loss of alpha galactosidase accumulation of ceramide
triaxide excellent recessive foamy urine angiocarotoma burning pain really dry skin
only really seen in males and cardiovascular disease boom roasted let's move on to the most
common gaucher's disease all right the next one this is going to be the most
common lysosomal storage disorder so therefore that's a very important thing that you guys need to remember
this is the most common lysosomal storage disease now again there is an accumulation of a
particular substrate due to the loss of a particular hydrolytic enzyme that hydrolytic enzyme
you can remember beta glucosidase but the other name for it is called glucosere
the only reason i say this one is because guess what the substrate's name is
it's called gluco cerebro side so in this condition which is autosomal recessive an easy way to
remember these is that all of them are autosomal recessive except for fabries which is excellent recessive
this is autosomal recessive in this condition there is a loss of
glucocereprosidase or beta glucosidase and therefore an accumulation of glucose cerebroside within the particular
tissues what tissues does this glucose cerebroside like to accumulate in the bone tissue you know particular
portion of the bone tissue that's very important the red bone marrow guess what happens
if this kind of accumulates within the bread bone marrow and affects the activity of the red bone marrow
what does the red bone marrow make it makes red blood cells it makes white blood
cells and it also makes platelets if these accumulate it's going to damage the red
bone marrow what's going to happen to the number of platelets decrease red blood cells
decrease white blood cells decrease what is this called pancytopenia pan cyto
that's one thing the other thing is that can it can actually accumulate within the bone
and lead to bone infarctions which is called a bone crisis so it can lead to a vascular necrosis of the bone
and the other thing is if this is actually continuous over time it can lead to
early onset osteoporosis so it can also really damage the bone tissue and lead to
osteoporosis the next thing is if these little molecules these glucose cerebrosides
accumulate within the liver and accumulate within the spleen guess what this is called
this is called hepatosplenomegaly this is called apato and then for the spleen this is called
splenomegaly very very important for these the next thing
this is a very very high yield very commonly asked on the usmle step one you remember we said that macrophages
are very important because they contain lysosomes that break down a lot of bacteria and substances
if these glucose cerebroside molecules accumulate with inside of the macrophages it produces a
very classical type of cell we call these cells gaucher cells you can see this under microscopy and
it's basically where the glucose cerebroside which is a type of lipid these are all what's called
sphingolipidosis if this accumulates these lipids it leads to lots of lipid inclusions
accumulating within these macrophages and this is called gaucher cells and where they love to be
found the spleen the liver and in the bone tissue
okay the last one which is neem and pick disease now neiman pick disease is actually again an autosomal recessive
disorder so again what is this disease this is autosomal recessive again all of them are autosomal recessive
except for which one again fabrics which is excellent right and you can remember the mnemonic
because they only have a y chromosome in males now the particular enzyme hydrolytic
enzyme that is actually going to be lost in this look at this little dude this is shaped like an s this is called
sphingosphingo myelinase this enzyme is absent or super deficient and therefore
not able to break down the particular substrate it breaks down which is called oh thankful thank goodness it's a super
super simple one sphingo myelin so in this autosomal recessive disorder where you have no
sphingomyelinase you don't break down sphingomyelin and it accumulates within particular tissues what are the
particular tissues it accumulates in remember we said this is very similar to tay sachs disease
the only thing that's different with tay sachs is that it does not have hepatomegaly so in that case we already
can answer these two quick ones here it accumulates within the
liver and within the spleen what is this called collectively whenever they get enlarged because of that accumulation
it's called hepato splenomegaly the next thing that's important here it also
loves to accumulate within the brain tissue and when it accumulates within the brain
tissue it leads to neurodegeneration so again you're going to see that neurodegeneration
but here's the big thing here we remember in the tay sachs there was hyperacusis
hyperreflexia and there was even um some of that cherry red spot
this has some of the similar features it's still going to have like seizures and developmental delays but the big
thing here is it also has a cherry red spot that accumulates on the macula within the retina so which ones
both have cherry red spots name and pick disease and taste axe disease what's the big difference
between them the tay sachs has no hepatomegaly and neem and pick disease has
hepatosplenomegaly one more thing that you need to know phrenompic disease is that again
macrophages are important for you know they have lots of lysosomes to break down certain bacteria
if these sphingomyelin molecules accumulate it's going to produce again a lot of lipid inclusions that
accumulate within these macrophages so these are called whenever they have a lot of these lipids
they love to call these remember how these were called gaucher cells they love to call these foam cells
foam cells one other important high yield fact is if you have these when you're comparing these two because
you're like oh wow this has a lot of lipids this has a lot of lipids how do i know
the difference well one thing is the dependent upon the substrate but
here's another thing they got to try to make it a little easy when you look at this under a microscope
gaucher cells look like crumpled [Music] tissue paper and that is a very
important thing to remember for your u.s emily step ones so again crumpled tissue paper gaucher
cells lots of lipid accumulation in foam cells these are going to be the
name and pick disease types of macrophage accumulation with lipids so again that covers the lysosomal
storage diseases iron engineers in this video we talk about lysosomes and lysosomal storage
disorders i hope this made sense and i hope that it helped all right ninja nerds as always until
next time [Music] you
Heads up!
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