Cholera And Cystic Fibrosis Flashcards
Fxn of CFTR
- Membrane Channel
- Transports Cl- INTO and OUT of the cell
- Regulates the fxn of other Channels
(Na+)
Cystic Fibrosis
Genetic Disease
- Autosomal Recessive Disorder
How many CTR mutations are there?
- 1300+
- Categorized into 4 CLASSES
I, II, III, & IV
How do mutations in CFTR result in Clinical manifestations of CF
There is either a difficiency in either the SECRETION or REABSORPTION of Cl-
Mechanism of action for the Cholera toxin:
What happens after PKA is activated?
- Chlorine channels opens
- Chlorine Efflux
- Water moves outside into the lumen
- Results in Diarrhea
Treatment for Cholera
- Oral Rehydration
(Clean Drinking Water) - Antibiotic therapy
What organ does CF primarily effect?
The Lungs
CFTR is a member of what family?
ABC Transporter SUPER family
Mechanism of action for the Cholera toxin:
What happens after the the Cholera binds?
- Hijacks the Endo-membrane System
- Goes to the Golgi
T/F: 2 Homozygous CF parents are likely to pass along the disease to the offspring.
False
Very unlikely
Where do the Cholera toxins concentrate on?
Lipid Rafts
Where are CFTR’s found?
In cells that Produce
- Mucus
- Sweat
- Digestive enzymes
- Tears
What are Natural Reserviors for Cholera?
Shellfish
CFTR energy use?
Uses Energy from Nucleotide HYDROLYSIS to open the Channel
General symptom of Cholera
- RICE-WATER Stool
- Voluminous Diarrhea
Mechanism of action for the Cholera toxin:
What happens after the Cholera toxin reaches the ER?
- Ends up in the Transmembrane of the ER
- The Cholera toxin Escapes the proteosome
Mechanism of action for the Cholera toxin:
What happens after the toxin escapes the proteosome?
- The A subunits Activates ADENYLYL Cyclase
- Sets off a cascade
- Produces Cyclic AMP
- Activates PKA
Mechanism of action for the Cholera toxin:
What happens after the Cholera toxin reaches the Golgi?
- FUSESS with the Golgi
- Transported to the ER
Mechanism of action for the Cholera toxin:
Where does the Cholera toxin bind?
To the intestinal cells in the LUMEN
The Cholera Toxin is what type of Toxin?
Oligomeric A/B Toxin
- “A” Subunit is the ENZYMATIC part
- “B” Subunits (5) are the BINDING Receptors
What does CFTR stand for?
Cystic Fibrosis Transmembrane Conductance Regulator
Normally how in Adenylate Cyclase activated?
By a G-protein coupled receptor
Heterozygous Advantage of CF
Hypothesis
- Heterozygotes in the Population have a 50% Fxn CFTRs and Selective advantage in resisting death by Cholera
Symptoms of CF
- Salty Skin
- Persistent COUGHING
- Wheezing/ shortness of breath
- Excessive APPETITE
(poor weight gain) - Greasy, bulky stool
