Xexam3 Mfm46

Front 1

when using unsaturated fatty acids in β-oxidation, there needs to be additional first steps and they are:
1.
2.

Back 1

1. cis double bonds isomerized to trans (isomerase)
2. the double bond reduced (reductase) prior to β-oxidation

Front 2

what hormones regulate β-oxidation:
1.
2.
3.

Back 2

1. glucagon
2. epinephrine (sympathetic nervous system)
3. β-corticotropin (sympathetic nervous system)

Front 3

what other 2 things besides hormones regulate β-oxidation:
1.
2.

Back 3

1. malonyl CoA (from fatty acid synthesis)- inhibits
2. ATP - inhibits

Front 4

dietary medium-chain-length fatty acids are more ... soluble than long chains and not stored in ...

Back 4

water
adipose

Front 5

following meal, dietary medium-chain-length fatty acids enter liver mitochondrial matrix by ...

Back 5

monocarboxylate transporter

Front 6

for odd number fatty acids, β-oxidation spiral continues until 5 carbons remain, 2 become ..., the last 3 become ...

Back 6

acetyl CoA
3-carbon propionyl CoA

Front 7

propionyl CoA converted to TCA intermediate ... and can be used for ...

Back 7

succinyl CoA
gluconeogenesis

Front 8

these are usually toxic metabolites that are removed from the cell via fatty acid oxidation

Back 8

xenobiotic fatty acid-like compounds

Front 9

by definition ... must contain catalase to breakdown hydrogen peroxide

Back 9

peroxisomes

Front 10

what are 2 important functions of peroxisomes:
1.
2.

Back 10

1. β-oxidation of VLCFA (very long chain fatty acids)
2. detoxification of glycolate to glycine

Front 11

what does excess glycolate lead to:

Back 11

precipitation of calcium oxalate → kidney stones

Front 12

peroxisomal β-oxidation is similar to mitochondrial β-oxidation with a few exceptions:

Back 12

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

Front 13

there are 2 major categories of peroxisomal disorders
1.
2.

Back 13

1. failure to form intact normal peroxisomes
2. deficiencies in a single peroxisomal enzyme

Front 14

there are ... known peroxisomal disorders

Back 14

25

Front 15

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

Back 15

absence of peroxisomes
within first year of life
very long chain fatty acids
C26-C38 polyenolic acids

Front 16

Zellweger’s Syndrome is also called ...

Back 16

cerebro-hepato-renal syndrome

Front 17

the key organs affected by Zellweger’s Syndrome are:

Back 17

brain
liver
kidney

Front 18

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

Back 18

Neonatal and X-Linked Adrenoleukodystrophy (ALD)

Front 19

Symptoms of X-Linked ALD:
1.
2.
3.
4.
5.
6.
7.

Back 19

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

Front 20

Branched-chain fatty acids are degradation products of ...
found mostly in ...

Back 20

chlorophyll
green vegetables

Front 21

the 2 most common branch chain fatty acids are ... and ...

Back 21

phytanic acid
pristanic acid

Front 22

alpha-oxidation of long-chain branched-chain fatty acids occurs only in ...

Back 22

peroxisomes

Front 23

Refsum’s Disease is inherited and there is an enzyme defect in ... causing the accumulation of

Back 23

phytanoyl-CoA hydroxylase
phytanic acid

Front 24

Symptoms of Refsum’s Disease:
1.
2.
3.
4.
5.
6.

Back 24

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

Front 25

ω-Oxidation of Fatty Acids occurs in the ... and converts fatty acids to ..., which can then undergo ... in mitochondria

Back 25

ER microsomes
dicarboxylic acids
β-oxidation

Front 26

dicarboxylic acids have ... groups at both ends

Back 26

carboxylic

Front 27

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

Back 27

Dicarboxylic Aciduria