Cell Organelles Flashcards
3 pathways for cell degradation
1) Phagocytosis ( go to lysosome)
2) Autophagy ( damaged organelles degrade via lysosome)
3) Receptor Mediated Endocytosis- macromolecules -> early endosome(fusing vesicle from Golgi) ->late endosome-> lysosome
3 topological categories in cell
1) Nucleus and cytosol (communicate through complex
2) ER, Golgi apparatus, endosome, and lysosome (secretory and endocytic pathway) (communicate through vesicles).
3) Mitochondria
Familial hypercholesterolemia- how does it affect LDL?
- cholesterol uptake mechanism disrupted
- main transport protein in plasma
- Causes
1) mutation in gene encoding LDL receptor
- LDL receptors incaplable to binding LDL
- LDL receptors that bind at reduce capacity
- LDL bind normally but in capable of internatzation
*high LDL => atherosclerosis plaques
Function of Smooth ER
- Glycogen metabolism
- Lipid synthesis
- Phospholipid synthesis (other membranes)
- Detoxification
- Steroid genesis
- Calcium regulation
Golgi Apparatus function
- Post- transcriptional modification for proteins and lipids
- Carbohydrates attached to glycoproteins and proteoglycans
- Polysaccharide/oligosaccharide synthesis
- Secretory products- mannose -6- phosphate and packing in granules
How are lysosomes formed?
- Fusion of transport vesicle and late endosome ( exm cholesterol, taken up by endocytosis)
- Clathrin componenet recycle, early endosomes mature into late endosomes (Precursor to secondary lysosomes)
- to work= ph down to 5.5
Mannose- 6- Phosphate
Tag on lysosomal enzyme in ER to go into lysosome
- separates it from glycoproteins in ER
- binds to M6P receptors -> transport to clarithin coated receptor-> forms primary lysosome
Mitchondrial transfer RNA
- high variable
- have been linked to over 200 disease states
- mutations in:
- mtDNA -> affect mitochrondrial fxn
- nuclear DNA
Mitochondria in high energy cells
- less dynamic
- much more tightly pack between myofibrils (cardiac muscle) and around flagellum of sperm cells
Peroxisome function
- Catalase- enzyme broken down from h- bones
- degrade/synthesis of hydrogen peroxide
- B oxidation
- cholesterol/ bile synthesis
- detoxify alcohol
Primary Lysosomes
- storage of lysomal hydrolases
- no digestive event
- homogenous
- inactive enzymes
Secondary Lysosomes
- engages in catalytic process
- heterogenous
- digestive enzymes
- active enzyme
Signal Sequence
a few AAs in the beginning of polypeptide that directs ribosome to Endoplasmic Reticulm finish protein synthesis and spit into ER
- once protein is needed, will create vesicle to transport to Golgi
Two Types of Vesicle Transport
1) Clathrin-coated vesicles
- move things to break down
- transports products from Golgi or exterior of cells into LYSOSOMES (like cholesterol)
- endocytosis- dynamin pinch off of plasma membrane into cytosol
2)COP- coated proteins
COP I- move things between Golgi
COP II- move things from ER TO(2) Golgi
Zellweger Syndrome
- defect in assembly of peroxisome ( no cholesterol uptake)
- 12 genes to make peroxisomes (ph 6 before closeing)
Worst: any 12 gene will call Zellweger spectrum disorder -> none or reduced number of peroxisome cells - congenital at birth
no cure/treatment
First years: death at 1year old