Intracellular Vesicular Traffic 2 Flashcards
What is a lysosome?
membrane enclosed compartments filled with hydrolytic enzymes
What do lysosomes require for optimal activation?
acidic environment and proteolytic cleavage
What does a lysosome do?
intracellular digestion of macromolecules
What does a vacuolar ATPase do?
pumps H+ into lysosomes to maintain the acidic pH and drive transport of small metabolites
What is the pH inside of lysosomes?
5
What does the pathway that transports from the golgi network to the lysosome do?
delivers membrane proteins and hydrolyses to lysosomes
Lysosomal hydrolyses have sorting signal _______ attached to them in the ______
mannose-6-phosphate, cis golgi network
What receptors in the trans golgi network recognize mannose?
mannose-6-phosphate receptors
What type of vesicles are lysosomal proteins packaged into that bud from the TGN?
clathrin coated
Where are lysosomal proteins delivered to?
endosomes then lysosomes
What causes lysosomal storage diseases?
genetic defects in lysosomal hydrolases resulting in accumulation of undigested material in lysosomes
What is Hurler’s disease?
mutation in enzyme required to break down glycosaminoglycan chains
What is inclusion cell disease?
all of the lysosomal hydrolyses are missing in many cell types. Undigested substrates accumulate as inclusions
__________ adds M6P to lysosomal hydrolases
GlcNAc phosphotransferase
What happens when GlcNAc phosphotransferse does not add M6P to lysosomal hydrolyses?
enyzymes are not phosphorylated and not sorted into vesicles and not delivered to lysosomes instead careered to cell surface and secreted