jump into fire
Where is apolopoprotein A1 synthesized
liver and intestine
How is unwanted cholesterol accumulation prevented/ maintains cholesterol homeostasis
reverse cholesterol transport
What binds LDL receptors to mark them for degradation
PCSK9
What is short bowel syndrome
inability to absorb sufficient quantities of calories and nutrients
When you have low levels of selenium in water and food, causing congestive cardiomyopathy
Keshan
Circulating nascent HDL picks up cholesterol and phospholipids from hepatocytes, enterocytes, and other extra hepatic cells via
BONUS: what happens if you have ABCA1 mutation
ABCA1
BONUS: tangiers disease
What pathway do macrophages play a central role in?
reverse cholesterol
When LDL receptors migrate to opposite sides what do they form
CURL
I have blue sclerae. What is wrong with me
osteogenesis imperfecta
What amino acids are derived from glycolysis intermediates
serine (which produces glycine and cysteine): synthesized from 3PG
alanine: transamination of pyruvate
What enzyme esterifies cholesterol to be moved to the core of HDL?
BONUS for everyone besides Carsen: what activates it and what happens if you are deficient in it
LCAT
activated by apo-1 and deficiency causes decrease in serum HDL
What two transporter proteins does LXR up regulate?
ABCA1 and ABCG1
Name 2 non modifiable and 2 potentially controllable major risk factors for atherosclerosis
2 non modifiable: increasing age, male gender, fam history, genetics
2 controllable: dyslipidemia, htx, ciggarettes, diabetes
Four stages of urea cycle
transamination, oxidative deamination of glutamate, ammonia transport, reactions of urea cycle
What is a required cofactor for the urea cycle
pyridocial phosphate derived from B6
What does CETP do?
Which of the following best describes the role of cholesteryl ester transfer protein (CETP)?
A. It converts cholesterol from LDL to cholesterol esters.
B. It is a major protein of HDL.
C. It exchanges cholesterol/cholesterol esters from HDL to other
lipoproteins.
D.It activates LCAT.
E. It acts as a ligand for HDL receptors.
Answer is C
First pathophysiological event and first physical sign of atherosclerosis
1. oxidized LDL taken up by macorphages
2. formation of foam cell and fatty streak
What cycle is glutamate dehydrogenase in and what does it do?
urea cycle; deaminates glutamate to produce ammonium and a-KG
Most common urea cycle disorder. X-linked recessive (vs other urea cycle enzyme deficiencies, which are autosomal recessive). Interferes with the body’s ability to eliminate ammonia. Often evident in the first few days of life, but may present later. Excess carbamoyl phosphate is converted to orotic acid
ornithine transcarbomylase deficiency
How does cholesterol in HDL get absorbed
SR-B1
What organ influences LDL expression Carsen?
thyroid
What is important about Cys, Gln, Guy, Pro, and Try
they are conditionally essential
Rate limiting reaction for urea cycle and its enzyme
step 1, enzyme carbamoyl phosphate synthetase
The nitrogen in alanine comes out via
ALT alanine transaminase