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Biochemistry > Beta Oxidation > Flashcards

Beta Oxidation Flashcards

1
Q

Site of beta oxidation of FA

A

Mitochondria; Aerobic

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2
Q

Beta oxidation generates large quantities of ATP

A

106

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3
Q

At High rate of Fatty Acid oxidation

These are produced

A

Acetoacetate
3-Hydroxybutarate
Acetone

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4
Q

Most fats in the body are stored as

A

TAG

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5
Q

Glycerol + 3 FA

A

TAGS

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6
Q

The only step that requires ATP

Through the enzyme acyl coA synthetase (thiokinase)

A

Fatty acids are activated first before they can be metabolized

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7
Q

Acyl CoA undegoes beta oxidation to become

A

Acetyl CoA

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8
Q

Fatty acid is converted to Acyl CoA

Acyl CoA is converted into

by the enzyme

A

Acyl Carnitine

Carnitine Palmitoyl Transferase I

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9
Q

Acyl carnitine is shuttled from cytosol to

A

mitochondria

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10
Q

AcylCarnitine is converted to

by the enzyme

A

Acyl CoA

Carnitine Palmitoyl Transferase enzyme II

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11
Q

Acetyl CoA can be

A

Recycled to become
Acyl CoA
Proceed to Krebs

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12
Q

Carnitine transports

A

Long chain fatty acids through the inner mitochondrial membrane

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13
Q

Transports long chain fatty acid through inner mitochondrial membrane

Only the metabolic product is converted can be transferred into mitochondrial membrane

A

Carnitine

Acylcarnitine

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14
Q

Inside the inner mitochondrial membrane acylcarnitine reacts with CoA catalyzed by

A

CPTII

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15
Q

Step 1 of beta oxidation: long chain fatty acid is

via enzyme

A

Dehydrogenated

fatty acyl CoA dehydrogenase

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16
Q

Step 2 of Beta oxidation

Trans-delta2-enoyl CoA is

A

hydrated

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17
Q

Step 3 Beta Oxidation

L-B hydroxylacyl CoA is

by enzyme

A

dehydrogenated again

B hydroxyl CoA dehydrogenase

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18
Q

Step 4 of beta oxidation:

by the enzyme

A

Thiolysis

Thiolase enzyme

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19
Q

Steps of Beta Oxidation

A

Dehydrogenation
Hydration
Oxidation
Thiolysis

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20
Q

Beta oxidation invovles

A

FA oxidation

Shortening of B Acyl CoA Chain

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21
Q

Disorder of fatty acid synthesis involves this organelle

A

Peroxisome

22
Q

Very long chain FA are associated with this organelle

A

peroxisomes

23
Q

Catalyzes first oxidative step of pathway

A

Acyl coA catalase ??

24
Q

Beta oxidation is induced by

A 
High fat diets
Hypolipidemic drugs (Clofibrate)
25
Q

Newborn, preterm

A

Carnitine deficiency

Tx: oral supplementation with Carnitine

26
Q

Liver
Reduced FA oxidation
Ketogenesis
Hypoglycemia

A

CPT I deficiency

27
Q

Skeletal muscle

Liver

A

CPT II deficiency

28
Q

Inherited absence of peroxisomes

Loss of peroxisimal functions

Severe neurological disease

Die in first year of life

A

Zellweger’s cerebrohepatorenal syndrome

29
Q

Enzyme deficient in Zellweger’s

A

Pipicolate oxidase

30
Q

Rare neurologic disorder

Accumulation of phytanic acid
Pathologic effect on membrane function, protein prenylation and gene expression

A

Refsum’s disease

Phytanic acid storage disease

31
Q

Enzyme deficiency in Refsum’s disease

A

Phytanoyl CoA hydroxylase

Peroxin

32
Q

Gene mutation in Refsum disease

A

PEX7 Gene

33
Q

Gene mutation in Zellweger Syndrome

A

PEX1 gene

34
Q

Refsum presents with

A

Ataxia

Icthyosis

35
Q

Associated with ingestion of the fruit of akee tree

Toxin inactivates medium and short chain acting Acyl-CoA dehydrogenase inhibiting beta oxidation and causing hypoglycemia

A

Jamaican vomiting sickness

Hypoglycin toxin

36
Q

Undergoing hemodialysis uses up the body’s L carnitine

It is also used as replacement supplement in strict vegetarians, dieters, and low weight or premature infants

A

L-Carnitine

37
Q

Process by which Fatty acids are broken down into Acetyl Coa to form ketones and metabolic fuel

Forms majority of energy available to liver

Forms ketone bodies available to brain and muscle

Size of fatty acid determines the location of beta oxidation

A

Beta oxidation of fatty acid

38
Q

Short/Medium Chain

(2-12 carbons) are broken down in

A

Diffuse freely in mitochondria

39
Q

Long Chain
(14-20)

Beta oxidation

A

Must use carnitine transport into mitochondria

40
Q

Very long chain
>20 carbons

Beta oxidation

A

Must be oxidized in a peroxisome

41
Q

Step 1:

Fatty acids -> Fatty acyl coa by the enzyme

A

Fatty Acyl Coa Synthetase

42
Q

Step 2

Fatty acyl coa diffuses into mitochondrial membrane and is converted to Fatty acyl carnitine by the enzyme

A

Carnitine Acyltransferase I

43
Q

Fatty acyl coa -> Fatty acyl-carnitine by Carnitine Acyltransferase I is inhibited by

A

Malonyl CoA (Fatty acid synthesis)

44
Q

Step 3

Fatty acyl-carnitine is shuttled to the mitochondria and

Fatty acyl carnitine -> Fatty acyl coa + Carnitine by the enzyme

A

Carinitine Acyltransferase 2

in mitochondria

45
Q

Step 1 FATTY ACID OXIDATION

Fattyl Acyl Coa -> Acetyl coa by the enzyme

A

Fatty acyl coa dehydrogenase
or
Medium Chain Acyl Coa Dehydrogenase
Long Chain Acyl Coa Dehydrogenase

46
Q

Step 2 FA BETA OXIDATION

Acetyl coa ->

A 
Ketone bodies (liver)
Acetyl Coa to TCA
47
Q

Rate limiting enzyme of Beta Oxidation of Fatty acid

A

Carnitine Palmitoyl Transferase-1

CAT-1

48
Q

No transport of Fatty acyl coa to mitochondrial membrane

Hypoketotic
Hypoglycemia

A

Systemic Primary Carnitine Deficiency

49
Q

Myoglobinuria
Hypotonia, weak
Inc TAG in muscle (from build up of fatty acyl coa)

Fatty acyl-carnitine -> Fatty acyl coa + carnitine

Carnitine Palmitoyl Transferase 2

A

Myopathic CAT-2 Deficiency

50
Q

Deficiency of fatty acyl coa dehydrogenase (Non conversion of fatty acyl coa -> acetyl coa)

Non ketotic (unable to produce glucose) hypoglycemia
Hepatic dysfunction (inc accumulation of fatty acyl coa in liver) 
Hyperammonemia

Avoid fasting

A

Medium chain Fatty Acyl-CoA Dehydrogenase Deficiency

Biochemistry (32 decks)

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