Processing Control Flashcards
Alternative Splicing
Being able to remove certain things as if it was an intron
Hutchinson Gilford
-An error of alternative splicing
-Due to a single base pair change
-Lamin A gene GGC gets changed to a GGT
-RNA formed is a GGU
-GU creates an intron that would normal not be there
-So it get spliced out about 50 a.a get removed that should have been in Lamin A
-Lamin A is involved in cell stability
IPEX Syndrome
-Fox 3p gene is normally fine
- After the gene RNA poly II making RNA
-G-capping occurs
-But poly adenylation never occurs cause of a messed up sequence
-Due to not having a poly-A tail it degrades quickly
-Fox 3 proteins will not be created
What happens if Fox 3 protein is not created
-T-regulatory cells formation will not occur
-Macrophages or dendritic cells engulf viruses and bacteria in phagosome
-Lysosome and phagosome combine it becomes a phagolysozome
-gets chopped up presents it on MHC 1 and 2
-1 interacts with cytotoxic T-cells
-2 interacts with helper T-cells
-those cells are aggressive towards fighting infection
-After 2 weeks T-reg cells need to come in a shut the off
-Otherwise out own immune system will fight our own tissue causing autoimmune disease
MHC 1 interacted with
Cytotoxic T-cells
-stimulate to act and divide
What do cytotoxic cells do after being activated
Will find other MHC 1 were the bacteria is on our cells and release toxins into our cells killing them
MHC 2 interacted with
Helper T cells
-release hormones
What happenes after the helper t cells release hormones
-Hormones go into blood stream
-Interact with b-cells
Interaction of B-cells
-Each B-cells has a unique Antibody on its surface
-The hormone that matches with the antibody will stick
-Only b-cells that will divide must have that bacteria stuck to it
Happens once the B-cell get the hormone signal and bacteria
It will become a plasma cell and release it antibodies
-divided into many b-cells
Natural killer cells
-A bacteria that has a couple antibodies will bind to natural killer cells
-NKC release toxins, killing bacteria
How does the immune system impact IPEX
-If cytotoxic t-cells do not get turned off a lot of toxins will be created
-Need system to be turned off because it will become toxic to us
-T-regulatory cells can go and interact will T-cells that will shut down cytotoxic t cells and helper t cells
-Issue is FOX 3p gene codes for a protein that allows the t-reg cells to mature
-IPEX disease is a mutation in the poly A binding site so the gene rapidly degrades
-So do not get enough t-reg cells
-This causes are immune system to attack our own body
