Biochemistry week 2 Flashcards
How are fatty acids stored
- They are stored in WAT as TAG( triacylglycerol)
Explain the process how fatty acids are broken doen
ATGL (Adipose triglyceride lipase):
Breaks TAG into diacylglycerol.
HSL (Hormone-sensitive lipase):
Breaks diacylglycerol into monoacylglycerol.
MAG lipase:
Breaks monoacylglycerol into free fatty acids (FFA) and glycerol.
What does TAG stand for
triacylglycerol
Explain how fatty acids are transported
- Travel in the blood and bind to albumin
Free Fatty acids are activated intio fatty acylCoA using what?
- Fatty acyl - CoA synthetase ( thiokinase)
- Uses 2 ATP
Where does fatty acid oxidation happen
IN the mItochodnria
What is the purpose of the carnitine shuttle
- Helps the long chain fatty acids to get inside the mitochondria
Where does the actication of fatty acids tske place(2)
- Endoplasmic reticulum
- outer mitochondrial membrane
What does the beta oxidation step produce
- 1 FADH2
- 1 NADH
- 1 acetyl CoA
Name the 3 special cases during the beta oxidation step
-Oxidation of fatty acids with an odd number of carbons
- Oxidation of unsaturated fatty acids
-Peroxisome b oxidation for VLCFAs
What is the unique feature of the special case + what is the end product:
- The oxidation of fatty acids with odd chain FA
- Propionyl CoA
- Succinyl coA, which goes to the TCA cycle
What is the unique feature of the :oxidation of unsaturated fatty acids as a special case
- The double bonds interrupt the cycle
What is the unique feature of VLCFAs as a special case + end product
- Too long for mitochondria so its processed in the peroxisomes
-Shortened fatty acids
explain what regulates b oxidation
Increases:
-Glucagon, epinephrine
- Exercise which decreases malonyl coA , which therfore increases B oxidation
Decreases:
-Insulin
-HighATP /NADH
- Low ATP
-Fed state, which increases malonyl coA which decresses b oxidation
Where are ketone bodies made
- Liver mitochondria during fasting , diabetes
In ketone bodies/ketogensis what does the liver use FA to make
- acetycl coA
What happens in ketolysis
- Tissues convert acetoacetate into aceto acety coA via thiophrase
Name the 4 steps in b oxidation
- Oxidation (dehydration) by FAD Linked acyl dehydrogenase
- Hydration by hydratase
- Oxidation by NAD linked dehydrogenase
- Thiolytic cleavage by thiolase
What is Zellweger syndrome
- Defective peroxisome formation which leads to impaired fatty acid oxidation
- the buildup of VLCFA
What is symptoms of Zellweger syndrome
- Hypotonia
-Seizures
-Developmental delay
-Jaundice
What is Ketonuria
The presence of ketone bodies in urine
What causes Ketonuria
- Fasting, Diabetes type 1
