Fatty Acid Oxidation

Question 1

• Under starvation conditions, fatty acids are released from TAG by _______

Answer 1

hormone sensitive lipases

Question 2

The products of β-oxidation are ________

Answer 2

FADH2, NADH and acetyl CoA

Question 3

Acyl-CoA molecules cannot enter the mitochondrial matrix, so the long-chain acyl (fatty acid) group is transferred temporarily to the carrier molecule ______via the action of the key rate limiting step catalyzed by _______

Answer 3

carnitine,

carnitine-palmitoyl transferase I or CPT-I

Question 4

Each cycle Beta-oxidation has 4 steps that result in the production of _______

Answer 4

one acetyl CoA, one FADH2 and one NADH

Question 5

Deficiency of _______ results in severe hypoglycemia provoked by fasting because the energy derived from fat oxidation is used to power gluconeogenesis

Answer 5

acyl CoA dehydrogenases

Question 6

_________deficiency has been identified as the cause of some cases of sudden infant death syndrome, likely because infants rely on milk for nutrition and milk contains mostly medium chain fatty acids.

Answer 6

Medium-chain fatty acyl CoA dehydrogenase (MCAD)

Question 7

In odd number carbon beta oxidation 3-carbon propionyl CoA is converted to succinyl CoA, an intermediate in the TCA cycle. what co enzymes are required for these steps

Answer 7

there is a biotin-requiring step and a

vitamin B12-requiring step

Question 8

low blood glucose levels are a sign of a defect in _____

Answer 8

Beta-oxidation.

Question 9

very long chain fatty acids and branched fatty acids are

preferentially oxidized to fatty acids in ______.

Answer 9

peroxisomes

Question 10

The genetic disorder of Zellweger syndrome is caused by a defect in _______ in all tissues

Answer 10

peroxisomal biogenesis

Question 11

X-linked adrenoleukodystrophy is caused by a defect in ________ activation of very long-chain fatty acid

Answer 11

peroxisomal

Question 12

How do zellweger syndrome and x-linked adrenoleukodystrophy present?

Answer 12

at infancy, hypotonia (weakness), feeding problems, vision and hearing loss, large fontanelles

Question 13

Phytanic acid, a branched-chain fatty acid.

It is not a substrate of _______

Answer 13

acyl CoA dehydrogenase.

Question 14

Deficiency of a-hydroxylase causes ______

Answer 14

Refsum disease

Question 15

what is the presentation of Refsum disease

Answer 15

vision loss from retinitis pigmentosa,
anosmia (inability to smell), may also develop
muscle weakness, ataxia, hearing loss and
dry skin (ichthyosis)

Question 16

________ are collectively called ketone bodies.

Answer 16

Acetoacetate, 3-hydroxybutyrate and acetone

Question 17

what is the rate limiting step for the synthesis of ketone bodies

Answer 17

HMG CoA synthase combines a third molecule of acetyl CoA with acetoacetyl CoA to produce HMG CoA.