Lipid Biochemistry II Flashcards
muscle weakness in arms and legs, elevated triglycerides with primary long chain FAs, lipid vacuoles in muscles
carnitine deficiency
glucagon
does not stimulate lipid release
insulin
stimulate lipid release
increased hormone sensitive lipase
TGL > glycerol + FAs
decreased insulin stimulates, as well as increased epi and increased cortisol
fatty acids
adipose tissue
- carried with albumin in blood
- to liver
-beta oxidation to acetyl CoA**
acetyl CoA
can go to ketone bodies
-brain and cardiac muscle
or to citric acid cycle
alpha oxidation
peroxisomes
-phytanic acid breakdown
omega oxidation
in some species of animals
alternate path to beta
beta-oxidation
long chain FA breakdown
in mito to produce acetyl CoA
to TCA cycle - to ETC > ATP
short chain FAs
2-4C
medium chain FAs
6-12C
short and medium chain FAs
diffuse freely into mitochondria
long chain FAs
14-20C
-activated first then transported into mito by carnitine shuttle to be oxidized
very long chain FAs
> 20C
- enter peroxisomes
- unknown mechanisms
- oxidation occurs
fatty acid beta oxidation pathway
first in cytosol
long chain FA - activated by ATp to fatty acyl CoA
- forms AMP (2 energy bonds)
- in cytosol
-cytosolic fatty acyl CoA reacts with carnitine - fatty acyl carnitine (CAT1 or CPT1)
passes across inner mitochondria membrane via carnitine acylcarnitine tranferase
CAT II, or CPT II, converts fatty acyl carnitine to fatty acyl CoA in matrix
to beta oxidation
CPT1
outer mito membrane
-fatty acyl CoA to fatty acylcarnitine
-then transported across inner mito membrane
aka CAT1
CPT II
inner mito membrane
fatty acylcarnitine converted back to fatty acyl CoA in mitochondrial matrix
aka CAT2
fatty acyl CoA synthetase
FA + CoA > fattyl acyl CoA
outer mito membrane
myopathic CAT/CPT deficiency
problem with long chain FA metabolism
muscle aches, weakness, myoglobinuria, provoked by exercise and fasting
elevated muscle triglyceride**
MCAD
medium chain acyl-CoA DH deficiency
fasting hypoglycemia
no ketone bodies
vomiting
coma, death
carnitine
can be measured in blood
if present in blood - either CPT1 or CPT2 deficiency
CPT1 and 2 deficiency treatment
avoid fasting
dietary restric long chain FAs
carnitine supplement
muscle weakness with exercise, hypoerammonemia, death
CPT II deficiency
fasting hypoglycemia, inability to use LCFAs as fuel by liver
CPT I defiency