TSD and the lysosome (maTrix edition)

1 A Phagocytes production

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


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



In This Presentation

What is TSD?Genetically Speaking…

e- MicrographFunctions

About TSD (Tay-Sachs Disease)

StructureAbout the Lysosome

Structure-FunctionAmong OrganellesThe Lysosome’s Relationships



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.



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



About the LysosomeURL ASF

Structure

Generally… what the lysosome is about


Source: United StreamingSaved As: lysosomes (general) (unitedstreaming).asf

The video clip has been removed to reduce file size.



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



About the Lysosomee- Micrograph



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



About the LysosomeFunctions

1.Digestion of materials from phagocytosis2.Autophagy (Autophagocytosis)

URL ASF


Source: United StreamingSaved As: lysosomes (phagocytosis) (unitedstreaming).asf




About the LysosomeFunctions

3.AutolysisExample with tadpole

URL ASF


Source: United StreamingSaved As: lysosomes (tadpole) (unitedstreaming).asf




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



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)



X Quick summary of lysosomes


Source: McGraw-HillSaved As: lysosomes (mcgraw-hill).swf



Tay-Sachs Disease

and the lysosome

Thank you for your kind attention.

TSD and the lysosome (maTrix edition)

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Transcript of TSD and the lysosome (maTrix edition)