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50 Cards in this Set

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fatty acids are the major source of fuel when:
1.
2.
3.
1. in between meals
2. when fasting (e.g. overnight)
3. periods of physical exertion
fatty acids are a fuel source for what kind of tissue:
1.
2.
3.
skeletal muscle
cardiac muscle
liver
fatty acids generate (more/less) fuel compared to glucose
more
dietary fatty acids are transported from intestine in the form of lipoprotein particles ... and ...
chylomicrons
VLDL
when fatty acids reach their target cells, they are cleaved from VLDL and the free fatty acid diffuses across the cell membrane where 2 things can happen:
1.
2.
1. used immediately for energy
2. converted into fatty acyl CoA
fatty acyl CoA combines with ... to from ... so it can be stored for later use as fuel for the body
glycerol-3-phosphate
triglyceride
excess glucose can be converted into ... and ...

this is stimulated by the hormone ...
acetyl CoA
glycerol-3-phosphate
insulin
look at the figure and use a ↑ or ↓ to indicate what is going on with the below listed hormones and enzymes:

glucagon / (insulin)
cAMP levels in adipocytes
protein kinase A (PKA)
hormone-sensitive lipase
↑ glucagon (↓insulin)
↑ cAMP levels in adipocytes
↑ protein kinase A (PKA)
↑ hormone-sensitive lipase
put the following in the correct order:

↑ cAMP levels in adipocytes
↑ glucagon (↓insulin)
↑ hormone-sensitive lipase
↑ protein kinase A (PKA)
fasting
fasting
↑ glucagon (↓insulin)
↑ cAMP levels in adipocytes
↑ protein kinase A (PKA)
↑ hormone-sensitive lipase
what are the function of lipases:
to relase fatty acids from triglycerides
lipases are ... sensitive
hormone
free fatty acids diffuse out of adipose cell and into the bloodstream where they travel attached to ...
albumin
glycerol derived from lipolysis enters bloodstream and can be used by the liver for ...
gluconeogenesis
Fatty acids enter cells through two routes:
1.
2.
diffusion
transport via fatty acid binding protein (FaBP)
where is the fatty acid binding protein located:
plasma membrane
fatty acids are ultimately oxidized to ... and ...
CO2
H2O
acetyl-CoA produced through β-oxidation of fatty acids in the liver can be:
1.
2.
1. converted to ketone bodies
2. enter the TCA Cycle.
where does β-oxidation of fatty acids occur:
mitochondria
in β-oxidation of fatty acids, free fatty acids are converted into ... before they cross the outer membrane of the mitochondria
fatty acyl CoA
... shuttles fatty acyl CoA across the ... membrane
carnitine
mitochondrial inner
what is the enzyme the catalyzes the following reaction:

fatty acid --> fatty acyl CoA
acyl CoA synthetase + ATP
what can we use fatty acyl CoA for:

1.
2.
3.
1. energy
2. membrane lipids
3. storage
carnitine comes from ... or can be synthesized in the ... from ... and
red meats/dairy
liver
lysine
synthesis of carnitine requires ... and ...
vitamin C
S-adenosylmethionine (SAMe)
... is on the outer mitochondrial membrane and is the rate-limiting enzyme (inhibited by malonyl CoA)

transfers the fatty acyl group to carnitine, forming fatty acylcarnitine, which is then translocated into the mitochondrial matrix
Carnitine Palmitoyl Transferase I (CPT I)
... on the inner mitochondrial membrane

transfers the fatty acyl group back to CoASH (re-forming the fatty acyl CoA) and releases the carnitine for recycling
Carnitine Palmitoyl Transferase II (CPT II)
identify the 3 enzymes labeled a, b, and c
(see figure)
what are the 3 most common diseases associated with carnitine metabolism:
1.
2.
3.
1. Carnitine or carnitine uptake deficiency
2. CPT I deficiency
3. CPT II deficiency
what happens in persons with carnitine deficiencies:
inability to transport fatty acids into the mitochondria
who is afflicted with carnitine deficiences:
occurs in newborns (particularly pre-term infants), patients undergoing hemodialysis, patients with organic acidemia
what are the symptoms in carnitine deficiencies:
muscle weakness, muscle cramping, cardiomyopathy, hepatomegaly, encephalopathy, sudden death
CPT I Deficiency is a (sex-linked/autosomal) (dominant/recessive) defect in CPT I gene that primarily affects ... in the liver
autosomal
recessive
fatty acid oxidation
the symptoms of CPT I Deficiency usually present after a period of ... or after a ... illness:

the symptoms include:
1.
2.
3.
4.
fasting
gastrointestinal
1. hypoketosis and hypoglycemia
2. lethargy
3. seizures
4. coma
CPT II Deficiency is a (sex-linked/autosomal) (dominant/recessive) defect in CPT II gene and has ...(number) subtypes
autosomal
recessive
3
the 3 subtypes of CPT-II deficiency are:
1.
2.
3.
1. myopathic CPT-II deficiency (most common)
2. hepatic CPT-II deficiency (similar to CPT I)
3. multiorgan
what are the symptoms of myopathic CPT-II deficiency:
1.
2.
3.
1. adolescence or adulthood onset
2. recurrent muscle pain, fatigue, and myoglobinuria (particularly following exercise)
3. often misdiagnosed (fibromyalgia, chronic fatigue)
The β-Oxidation Spiral is where ... are sequentially cleaved from ...
2-carbon acetyl CoA units
fatty acyl group
what are the 4 repeating steps in fatty acid oxidation:
1.
2.
3.
4.
1. oxidation
2. hydration
3. oxidation
4. cleavage
what are the 4 repeating steps in fatty acid synthesis:
1.
2.
3.
4.
1. condensation
2. reduction
3. dehydration
4. reduction
what is the most important (rate-limiting) step in fatty acid oxidation and what is the enzyme that catalyzes the reaction:
1st oxidation
aceyl CoA dehydrogenase
in oxidation, a ... is inserted into the fatty acid
double bond
there are several acyl CoA dehydrogenases, each works on a specific chain length. the most important is ... because it is most involved in pathologies and it works on fatty acids that are ... carbons in length
MCAD - medium chain acyl CoA dehydrogenase
6-12
what are the most frequently diagnosed of the fatty acid oxidation disorders
MCAD Deficiency
what accounts for about 1 in 100 SIDS deaths
MCAD Deficiency
... usually presents clinically between second month and second year of life following a period of fasting (even overnight fasting) or illness
MCAD Deficiency
how do you prevent Deficiency symptoms:
frequent feedings, high-carb/low-fat diet
...% of MCAD Deficiency patients will die with their first episode of illness
20-25
how would you manage MCAD Deficiency:
1.
2.
3.
1. eat regularly, avoid fasting
2. diet high in carbs, low in medium and long chain fatty acids
3. oral carnitine
where is the energy coming from in β-oxidation
FADH2 and NADH from the 2 oxidation steps in the β-oxidation spiral
the most common fatty acids used in β-oxidation are:
1.
2.
3.
4.

and they are the most common becuase:
1. palmitate
2. stearate
3. oleate
4. linoleate

most common dietary fatty acids and also commonly synthesized in humans