Lysosomes Flashcards
What is the structure of lysosomes?
- single membrane bound organelles
- contain ~ 60 different enzymes
- acidic pH of 5
- size ranges from 0.1 micrometers to 1.2 micrometers
How many lysosomes are found in a human cell ?
~300
What is the function of lysosomes ?
Lysosomes act as the digestive system of a cell and digest intracellular proteins and carbohydrates but also molecules and pathogens from outside the cell
Where do lysosomes originate from ?
They bud off of the trans face of the Golgi apparatus
Why are lysosomal membrane proteins glycosilated ?
Because the glycosilation protects the proteins from the proteases present in the lumen
What are the main lysosomal pathways ?
- Endocytosis
- Phagocytosis
- Pinocytosis
- Autophagy
What is heterophagy ?
The digestion of material of extracellular origin
What is autophogy ?
The digestion of material of intracellular origin
How is the internal acidic environment of a lysosome maintained ?
V-ATPase pumps protons from the cytoplasm to the lysosomal lumen which maintains the acidic environment within the lysosome
What is the role of mannose-6-phosphate ?
It targets proteins to lysosomes
It allows lysosomal hydrolases and membrane proteins to be delivered to lysosomes
What are lysosomal storage diseases ?
They are inherited metabolic diseases characterised by abnormal build up of materials in cells as a result of enzyme deficiencies
Give some examples of lysosomal storage diseases
- Hunter & Hurler syndrome
- Tay-Sachs disease
- Gaucher disease
- Inclusion cell disease
Summarise Hunter & Hurler syndrome
It develops as a result of large sugar molecules building up in body tissues
Symptoms include an enlarged head, thickening of the lips and a distended abdomen due to enlarged internal organs
Summarise Tay-Sachs disease
Develops as a result of deficiency of the hexosaminidase enzyme which means lipids accumulate in the brain and other organs
Symptoms include mental challenges, blindness and death within 3 years
Summarise Gaucher disease
Develops due to the accumulation of glycolipids
Symptoms include enlargement of the liver and spleen, anaemia and lung disease