Lipids 1 Flashcards
How much energy for fats vs glycogen?
9 vs 4
Nomenclature of FAs?
name 18:2 triangle 9, 12
name # of carbons; # of double bonds, position of double bonds counted from COOH end
What fat can be broken down? cis or trans?
cis
In humans, where can a double bond be introduced?
only up to carbon #9
What are FA’s that contain double bonds past the #9 carbon?
w-3 and w-6
FFA’s can be transfered across what?
cellular and mitochondrial membranes
Where does the degradation of FAs into acetyl Coa occur?
inside the mitochondria
How do you get a fatty acid into the mitochondria exactly?
you need to convert it into acyl-carnitine, which goes into the IMM; and then convert it back into acyl Coa
What disorder results from an inability to import FAs into the mito?
defect in carnitiine; hypoglycemia and ketone bodies in urine
Degradation of simple FAs (4 steps)
:)
What is the first enzyme used to degrade a simple FA?
acyl CoA DH
What is produced from the degradation of a simple fas?
acyl-Coa –> fatty acyl-cOa and acetyl coa
A FA with 16 carbons yields how many molecules of acetyl-Coa? # of NADH and # FADH? How many total ATP?
8 molecules of acetyl coA
7 NADH and FADH
106 ATP
How do you degrade unsaturated FAs?
- if double bond occurs between C3 and 4; enyol COA isomerase converts cis to trans bond
- If double bond occurs between C4 and C5; a reductase produces trans enyol
a-oxidation of fa’s occurs in what organelle?
peroxisome
In what 2 ways does B oxidation in the peroxisome differ from B-oxidation in the mitochondria?
- H202 is generated instead of FADH
2. peroxisomal B-oxidation degrads long acyl COa and ends with acyl chain shortened to 8 carbons
What are 4 acyl-CoA DH deficiencies?
you can’t break down FAs; VLCAD, LCAD, and MCAD, and SCAT; causes non-ketotic hypoglycemia and can be life-threatening
VLCAD, LCAD, MCAD, and SCAT are what type of disorder?
acyl-Coa DH deficiencies
What are 3 types of FA oxidation disorders?
- acyl-CoA DH deficiencies
- porblems wiht a-oxidation of branched FA phytanic acid (Refsum syndrome)
- Zellweger syndrome
What is a disorder that results from problems with a-oxidation of branched FA phytanc acid?
Refusm
What causes non-ketoic hypoglycemia?
VLCAD, LCAD< MCAD, SCAT: acyl-Coa DH defieicnes