TSD and the lysosome (maTrix edition)
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TSD and the lysosome (maTrix edition)

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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.

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. 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
  • 2. 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. In This Presentation What is TSD? Genetically Speaking… e - Micrograph Functions About TSD (Tay-Sachs Disease) Structure About the Lysosome Structure-Function Among Organelles The Lysosome’s Relationships
  • 4. 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-A ganglioside GM2 Tay-Sachs Disease is… the lysosomes don’t contain a certain enzyme to break down a certain fat… a lysosomal storage disease! Fatty materials Normal brain cell Tay-Sachs brain cell 1) Development of mental and motor f(x) fails. 2) Nervous system is shut down.
  • 5. 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. About the Lysosome URL ASF Structure Generally… what the lysosome is about TSD and the lysosome: maTrix edition Source: United Streaming Saved As: lysosomes (general) (unitedstreaming).asf The video clip has been removed to reduce file size.
  • 7. About the Lysosome URL Structure 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
    LYSOSOME Membrane-bound vesicle containing hydrolytic enzymes 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 . Partially permeable membrane pH 5 medium Hydrolytic enzymes
  • 8. About the Lysosome e - Micrograph
  • 9. About the Lysosome
    • Digestion of materials from phagocytosis Lysosomes fuse with phagosomes to deliver hydrolytic enzymes for digestion.
    • Autophagy (Autophagocytosis) Old or damaged organelles are broken down and recycled.
    • Autolysis Self-digestion of a damaged cell. Can also be part of normal development (eg tadpole)
    Functions
    • Digestion of materials from phagocytosis Lysosomes fuse with phagosomes to deliver hydrolytic enzymes for digestion.
    • Autophagy (Autophagocytosis) Old or damaged organelles are broken down and recycled.
    1/2 3
  • 10. About the Lysosome Functions 1.Digestion of materials from phagocytosis 2.Autophagy (Autophagocytosis) URL ASF TSD and the lysosome: maTrix edition Source: United Streaming Saved As: lysosomes (phagocytosis) (unitedstreaming).asf The video clip has been removed to reduce file size.
  • 11. About the Lysosome Functions 3.Autolysis Example with tadpole URL ASF TSD and the lysosome: maTrix edition Source: United Streaming Saved As: lysosomes (tadpole) (unitedstreaming).asf The video clip has been removed to reduce file size.
  • 12. The Lysosome’s Relationships
    • pH 5 medium - For enzyme activity - Cell safety
    • Membrane: presence - Cell safety
    • Membrane: partial permeability - Ensures complete hydrolysis
    Structure-Function Partially permeable membrane pH 5 medium Hydrolytic enzymes
  • 13. 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. X Quick summary of lysosomes TSD and the lysosome: maTrix edition Source: McGraw-Hill Saved As: lysosomes (mcgraw-hill).swf The video clip has been removed to reduce file size.
  • 15. Tay-Sachs Disease and the lysosome Thank you for your kind attention.