Term
| 3 fates for "new" proteins |
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Definition
(1) Cytosolic
(2) Organelle specific
(3) PM and secretory |
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Term
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Definition
For organelle-specific and PM/secretory fates of proteins
Utilizes vesicular transport |
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Term
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Definition
(1) CGN-ER
(2) medial stacks
(3)TGN-periphery of cell
[image] |
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Term
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Definition
(1) Modify proteins
(2) Sort proteins via TGN (lysosome, secretion, PM) |
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Term
| Golgi modification of proteins |
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Definition
Proteolysis
Glycosylation
AA side chain alteration
Acyl group addition
Sulfate addition
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Term
| Proteins destined for organelles |
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Definition
(1) ER resident proteins
(2) Lysosomal proteins |
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Term
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Definition
ER retention signal binding sequence allows proteins to be retrieved from Golgi and sent to ER
Membrane receptor in CGN sends back via retrograde transport |
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Term
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Definition
Mannose-6-P
Tag on proteins destined for lysosome
Utilize M6P receptor at TGN which moves proteins to endosome |
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Term
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Definition
Lysosomal storage disease
Deficiency in enzyme that adds MGP to lysosomal proteins
If not marked, are secreted
Results in inclusion bodies in lysosome and increased number of hydrolases in plasma
Affect CT, die by age 5 |
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Term
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Definition
PM-->lysosome
(1) pinocytosis
(2) phagocytosis
(3) receptor-mediated |
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Term
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Definition
Constitutive
Non-regulated
Continuous in most cells
[image]
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Term
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Definition
Regulated
Neutrophils
Monocyte
Macrophage |
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Term
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Definition
Selective uptake via cell surface receptors
LDL
(1) LDL binds receptor
(2) form clathrin coated pit---vesicle buds off
(3) uncoate clathrin
(4) fuse vesicles
(5) vesicles bind to endosome--low pH causes receptor to release ligand
(6) ligand transported to lysosome; receptor goes back to PM
[image] |
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Term
| Lysosomal storage diseases |
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Definition
Family of diseases-undegraded material in lysosome
Lack hydrolases
(1) Mucopolysaccharidosis
(2) Mucolipodosis
(3) Sphingolipidosis (Niemann Pick)
Mental retardation and neuronal problems |
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Term
Mucopolysaccharidosis
Hurler's Syndrome |
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Definition
Lysosomal storage disease
Accumulation of GAG because of deficient ioduronidase |
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Term
Familial hypercholesterolemia
hyperlipidemia IIa |
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Definition
Defective receptor mediated endocytosis
Mutation in gene for LDL receptor
Increase in plasma LDL levels leads to artherosclerotic plaques |
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Term
| Proteins destined to PM or for exocytosis |
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Definition
Exocytotic pathways diverge in TGN
(1) regulated
(2) constitutive |
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Term
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Definition
Exocytotic
Special cells (hromone, neurotransmitters, digestive enzymes)
Always Ca2+ regulated
Proteins bud from TGN, combine and accumulate near PM
Proteolytic processing |
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