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The presentation my group made to explain the lysosome and a little on the Tay-Sachs disease. This was also uploaded to the maTrix.
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1 A Phagocytes production
TSD and the lysosome: maTrix edition
TSD and the lysosome: maTrix edition
Created by: Phagocytes, TJC CG 30/06
This presentation has been edited to reduce its file size.
A Quick Note
TSD and the lysosome: maTrix edition
Tay-Sachs Disease
and the lysosome
LEFT:Akari Yamada holds her son, Davis Jr., nicknamed DJ. He suffers from Tay-Sachs, a rare disease that has left him blind and taken away most of the skills he had as a baby.Daily Herald Photo/ Bill Zars
Tay-Sachs Disease Lysosome
3 A Phagocytes production
TSD and the lysosome: maTrix edition
In This Presentation
What is TSD?Genetically Speaking…
e- MicrographFunctions
About TSD (Tay-Sachs Disease)
StructureAbout the Lysosome
Structure-FunctionAmong OrganellesThe Lysosome’s Relationships
4 A Phagocytes production
TSD and the lysosome: maTrix edition
the lysosomes don’t contain a certain
enzyme to break down a certain fat…
a lysosomal storage disease!
Brain and nerve cells become damaged and gradually lose their ability to
function properly.
About TSD What is TSD?
Genetic disorder caused by a deficient enzyme which results in
the extreme accumulation of a fatty substance in the nervous
system.
No cure; death by about age 5
hex-Aganglioside GM2Fatty
materialsNormal brain cell
Tay-Sachs brain cell
Tay-Sachs Disease is…
1) Development of mental and
motor f(x) fails.
2) Nervous system is shut
down.
5 A Phagocytes production
TSD and the lysosome: maTrix edition
About TSD Genetically Speaking…
Autosomal recessive genetic condition
Inherit from carrier parents
Mutation on chr 15 in HEXA gene encoding hex-A
From carrier parents: 1 in 4 chance
6 A Phagocytes production
TSD and the lysosome: maTrix edition
About the LysosomeURL ASF
Structure
Generally… what the lysosome is about
TSD and the lysosome: maTrix edition
Source: United StreamingSaved As: lysosomes (general) (unitedstreaming).asf
The video clip has been removed to reduce file size.
7 A Phagocytes production
TSD and the lysosome: maTrix edition
About the Lysosome
URL
Structure
Lysosome
Lysosomes can be known as “suicide sacks”.
They are produced by the Golgi apparatus and consist of a single membrane surrounding powerful digestive enzymes.
This slide is derived from an animation:
Cutting the lysosome…•Outer membrane removed - easier to see contents
•Lumpy brown structures are hydrolytic (digestive) enzymes.
Dissolving the membrane…•Look at the enzymes.
Some important enzymes:
•Lipases
•Carbohydrases
•Proteases
•Nucleases
LYSOSOMEMembrane-bound vesicle containing hydrolytic enzymes
Partially permeabl
e membran
epH 5
medium Hydrolytic
enzymes
8 A Phagocytes production
TSD and the lysosome: maTrix edition
About the Lysosomee- Micrograph
9 A Phagocytes production
TSD and the lysosome: maTrix edition
About the Lysosome
1.Digestion of materials from phagocytosisLysosomes fuse with phagosomes to deliver hydrolytic enzymes for digestion.
2.Autophagy (Autophagocytosis)Old or damaged organelles are broken down and recycled.
3.AutolysisSelf-digestion of a damaged cell. Can also be part of normal development (eg tadpole)
Functions
1.Digestion of materials from phagocytosisLysosomes fuse with phagosomes to deliver hydrolytic enzymes for digestion.
2.Autophagy (Autophagocytosis)Old or damaged organelles are broken down and recycled.
1/2 3
10 A Phagocytes production
TSD and the lysosome: maTrix edition
About the LysosomeFunctions
1.Digestion of materials from phagocytosis2.Autophagy (Autophagocytosis)
URL ASF
TSD and the lysosome: maTrix edition
Source: United StreamingSaved As: lysosomes (phagocytosis) (unitedstreaming).asf
The video clip has been removed to reduce file size.
11 A Phagocytes production
TSD and the lysosome: maTrix edition
About the LysosomeFunctions
3.AutolysisExample with tadpole
URL ASF
TSD and the lysosome: maTrix edition
Source: United StreamingSaved As: lysosomes (tadpole) (unitedstreaming).asf
The video clip has been removed to reduce file size.
12 A Phagocytes production
TSD and the lysosome: maTrix edition
The Lysosome’s Relationships
1.pH 5 medium- For enzyme activity- Cell safety
2.Membrane: presence- Cell safety
3.Membrane: partial permeability- Ensures complete hydrolysis
Structure-Function
Partially permeabl
e membran
e
pH 5 mediu
m
Hydrolytic
enzymes
13 A Phagocytes production
TSD and the lysosome: maTrix edition
The Lysosome’s Relationships
Lysosome Formation
• Endoplasmic reticulum- Enzyme proteins made in rER- Transported to GA by vesicles
• Golgi apparatus- Create the hydrolytic enzymes- Produces lysosomes
Among Organelles
Next: Quick summary of lysosomes (Animation)
14 A Phagocytes production
TSD and the lysosome: maTrix edition
X Quick summary of lysosomes
TSD and the lysosome: maTrix edition
Source: McGraw-HillSaved As: lysosomes (mcgraw-hill).swf
The video clip has been removed to reduce file size.
TSD and the lysosome: maTrix edition
Tay-Sachs Disease
and the lysosome
Thank you for your kind attention.