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27 Cards in this Set

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when using unsaturated fatty acids in β-oxidation, there needs to be additional first steps and they are:
1.
2.
1. cis double bonds isomerized to trans (isomerase)
2. the double bond reduced (reductase) prior to β-oxidation
what hormones regulate β-oxidation:
1.
2.
3.
1. glucagon
2. epinephrine (sympathetic nervous system)
3. β-corticotropin (sympathetic nervous system)
what other 2 things besides hormones regulate β-oxidation:
1.
2.
1. malonyl CoA (from fatty acid synthesis)- inhibits
2. ATP - inhibits
dietary medium-chain-length fatty acids are more ... soluble than long chains and not stored in ...
water
adipose
following meal, dietary medium-chain-length fatty acids enter liver mitochondrial matrix by ...
monocarboxylate transporter
for odd number fatty acids, β-oxidation spiral continues until 5 carbons remain, 2 become ..., the last 3 become ...
acetyl CoA
3-carbon propionyl CoA
propionyl CoA converted to TCA intermediate ... and can be used for ...
succinyl CoA
gluconeogenesis
these are usually toxic metabolites that are removed from the cell via fatty acid oxidation
xenobiotic fatty acid-like compounds
by definition ... must contain catalase to breakdown hydrogen peroxide
peroxisomes
what are 2 important functions of peroxisomes:
1.
2.
1. β-oxidation of VLCFA (very long chain fatty acids)
2. detoxification of glycolate to glycine
what does excess glycolate lead to:
precipitation of calcium oxalate → kidney stones
peroxisomal β-oxidation is similar to mitochondrial β-oxidation with a few exceptions:
1. carnitine not required
2. other enzymes have similar actions to their mitochondrial counterparts, but encoded by separate genes
3. first step catalyzed by FAD-containing oxidase
4. oxidation stops when chain reaches 4-6 carbons, then chain transferred to mitochondria to complete oxidation
there are 2 major categories of peroxisomal disorders
1.
2.
1. failure to form intact normal peroxisomes
2. deficiencies in a single peroxisomal enzyme
there are ... known peroxisomal disorders
25
in Zellweger’s Syndrome, there is an ... and is the most severe type of peroxisomal disorder and fatal ...

there is no oxidation of ... and there is an accumulation of ... in various tissues
absence of peroxisomes
within first year of life
very long chain fatty acids
C26-C38 polyenolic acids
Zellweger’s Syndrome is also called ...
cerebro-hepato-renal syndrome
the key organs affected by Zellweger’s Syndrome are:
brain
liver
kidney
what does the following describe:

-most common of the peroxisomal disorders
-mutation in ABC transporter involved in uptake of VLCFA across peroxisomal membranes (no β-oxidation)
-results in accumulation of VLCFA in blood and cells
Neonatal and X-Linked Adrenoleukodystrophy (ALD)
Symptoms of X-Linked ALD:
1.
2.
3.
4.
5.
6.
7.
1. first neurological symptoms occur at 4-8 years of age
2. ADHD-like symptoms
3. visual and auditory disturbances
4. poor coordination
5. seizures
6. rapid progression to vegetative state
7. impaired adrenal function and deficiency of steroid hormones
Branched-chain fatty acids are degradation products of ...
found mostly in ...
chlorophyll
green vegetables
the 2 most common branch chain fatty acids are ... and ...
phytanic acid
pristanic acid
alpha-oxidation of long-chain branched-chain fatty acids occurs only in ...
peroxisomes
Refsum’s Disease is inherited and there is an enzyme defect in ... causing the accumulation of
phytanoyl-CoA hydroxylase
phytanic acid
Symptoms of Refsum’s Disease:
1.
2.
3.
4.
5.
6.
1. usually adult onset, but infantile forms exist
2. retinitis pigmentosa, progressive vision loss
3. anosmia (loss of smell)
4. hearing loss
5. sensory and peripheral neuropathy
6. cardiac abnormalities may be present
ω-Oxidation of Fatty Acids occurs in the ... and converts fatty acids to ..., which can then undergo ... in mitochondria
ER microsomes
dicarboxylic acids
β-oxidation
dicarboxylic acids have ... groups at both ends
carboxylic
what does the following describe:

when there is some deficiency in mitochondrial medium chain acyl-CoA dehydrogenase (MCAD deficiency), impairing β-oxidation, ω-oxidation is stimulated, resulting in excess accumulation of dicarboxylic acids in the urine
Dicarboxylic Aciduria