PRIN 4 Organization of the Cell Flashcards
diastrase
enzyme that digests glycogen
*was used to clear the liver biopsy sample of glycogen. As a result, PAS allows visualization of the glycosylated granules of alpha1-proteinase inhibitor that was defective and trapped in the rER
Plasma vs Serum
PLASMA: contains all clotting factors
SERUM: after clotting factors have been removed
What is “Alpha-1 antitrypsin?”
- protein that is produced mostly in the liver
* protects the lungs from neutrophil elastase
What is “Neutrophil elastase?”
- enzyme that digests damaged or aging cells and bacteria in the lungs
- regulated by alpha 1 proteinase inhibitor
What is the result of alpha1-proteinase inhibitor def?
Elastase digests lung tissue in an uncontrolled fashion and leads to emphysema
***HIGH risk of lung disease
How is Kartageners Syndrome inherited?
Autosomal recessive mutation
What causes Kartageners Syndrome?
loss of dynein arms: cilia don’t work properly
Clinical Manifestation of Kartageners Syndrome:
(1) recurrent upper resp infections
(2) male infertility
(3) ectopic preg
(4) organs on wrong side
What causes CF?
Cystic Fibrosus Autosomal recessive (CFTR gene) *Defect in ABC transporter (ATP-binding cassette) results in faulty transmembrane protein that is an epithelial Cl- channel
What happens to the faulty CFTR gene in CF?
gets degraded by proteosome
Newly made proteins leave the rER and go where?
cis face of golgi via vesicle with COP2 coat
How do vesicles get BACK to the golgi?
via COP1 coat
What is the “H-antigen?”
*protein that is expressed on RBC membrane surfaces
What is glyco-syl-transferase?
- protein that modifies H-antigen on RBC surfaces
* exists in 3 forms depending on the 3 allele forms: ABO
What does the O allele result in?
encodes for an inactive glyco-syl-transferase leaving the H antigen unmodified
Example of Regulated Secretion
(1) Synaptic Transmission
(2) Mast Cell
(3) Salivary Glands
How is exocytosis regulated in Synaptic Transmission?
V-SNARE on vesicle interacts with T-SNARE on target
How do tetanus toxins disrupt synaptic transmission?
cleave SNARES preventing interaction and release of contents into synaptic cleft
How does hay fever result in allergies?
Hay fever toxins mimic IgE and bind & stimulate release of Mast Cell granules
What causes scurvy?
Lack of Vit C
Vit C is involved in recycling of enzyme proline hydroxylane which is needed to make collagen in rER
Proteosome vs Lysozome
PROTEOSOME: recycling of intracellular cytoplasmic materials
LYSOSOME: digestion of endocytized material