Mbod block 4 week 4 Flashcards

3
Q

What is the goal of fatty acid synthesis?

A

To store excess glucose as fatty acids.

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4
Q

What are fatty acids stored as?

A

TAG

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5
Q

What are hepatic TAGs exported from the liver as?

A

VLDLs

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6
Q

What complex makes palmitate?

A

The fatty acid synthetase complex

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7
Q

Where does fatty acid synthesis occur?

A

In the cytosol

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8
Q

What does fatty acid synthesis require?

A

Pantothenic acid, NADPH, FADH2

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9
Q

What does fatty acid synthesis use?

A

Acetyl CoA and malonyl CoA.

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10
Q

Where are fatty acids found?

A

In TAGS, glycerolipids, sphingolipids, cholesterol esters, lipoproteins, membranes, etc.

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11
Q

What is the role of citrate from mitochondria?

A

To provide AcCoA and NADPH for fatty acid synthesis in cytosol.

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12
Q

How is glucose made into fatty acid?

A

By Lipogenesis

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13
Q

What are TG’s hydrolyzed by?

A

LPL (lipoprotein lipase)

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14
Q

What enzymes are upregulated by insulin in fatty acid synthesis?

A

Citrate lyase; malic enzyme

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15
Q

What does the malic enzyme make?

A

NADPH and pyruvate

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16
Q

What are the sources of NADPH for fatty acid synthesis?

A

Pentose-P pathway and malic enzyme

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17
Q

What is citrate cleaved by?

A

citrate lyase

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18
Q

What is the first committed step of fatty acid synthesis?

A

Acetyl CoA is converted to Malonyl CoA.

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19
Q

What is Acetyl CoA converted to Malonyl CoA by?

A

Acetyl CoA carboxylase; reqquires Biotin

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20
Q

Where are all of the enzymic activities of fatty acid synthase located?

A

On a single, large, mulifunctional fatty acid synthase enzyme.

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21
Q

What are the growing intermediates attached to proteins by during fatty acid synthesis?

A

Thioester bonds

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22
Q

What group does the Acyl carrier protein portion of synthase have?

A

Phospho-pantetheinyl prosthetic group.

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23
Q

What other groups are on the multifunctional fatty acid synthase enzyme that also attach to FA synthesis intermediates?

A

Two sulfhydryl groups

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24
Q

What is a component of the -SH group for a thioester bond with malonylCoA?

A

Panthothenic acid

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25
Q

What is the first step of FA synthesis?

A

Transfer of malonyl to FA synthase

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26
Q

What is the second step of FA synthesis?

A

Condensation of malonyl and acyl groups

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27
Q

What is the third step of FA synthesis?

A

Reduction of B-ketoacyl group with NADPH

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28
Q

What is the fourth step of FA synthesis?

A

Dehydration forms a double bond.

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29
Q

What is the fifth step in FA synthesis?

A

Reduction of double bond with NADPH.

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30
Q

How many carbons are added with each repeat of the repetitive reactions of FA synthesis?

A

2 C

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31
Q

What is required for a new FA chain to start?

A

An AcCoA is required to be the acceptor of the malonyl transfer. Must make malonyl CoA from AcCoA.

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32
Q

What is the intermediate that is formed from the condensation of malonyl and an acetyl group?

A

A B-keto intermediate

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33
Q

What do the reduction reactions of FA synthesis need?

A

NADPH

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34
Q

How many ATP are needed for Palmitate synthesis?

A

7 ATP

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35
Q

Where is elongase found?

A

In the ER

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36
Q

When does elongation occur?

A

While the fatty acid is bound to CoA

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37
Q

What is desaturase?

A

An oxidase located in the ER. It inserts a double bond at the 9th carbon from the carboxyl group. Makes palmitoleate from palmitate, and oleate from stearate.

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38
Q

What do Acyl CoA desaturases in the ER contain?

A

FAD, cytochrome B5, and an iron-sulfur center, froming a mini electron transport chain.

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39
Q

What is linoleate used for?

A

It is the major source of the essential fatty acids required in the human diet. They are used for the synthesis of arachidonic acid.

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41
Q

What do glycerolipids have as a backbone?

A

Glycerol

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42
Q

What are triacylglycerols made of?

A

A glycerol backbone + 3FA

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43
Q

What are glycerophospholipids made of?

A

A glycerol backbone + 2FA + head group

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44
Q

What are the functions of complex lipids?

A

Storage of fatty acids as TAG; Component of plasma membranes; Signal transduction; Recognition

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45
Q

What are some examples of glycerophospholipids?

A

Phosphatidylcholline; phosphatidylethanolamine; phosphatidylserine; phosphatidylinositol bisphosphate (PIP2); phosphatidylglycerol; cardolipin

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46
Q

What hydrolyzes triacylglycerol and diacylglycerol to form 2-monoacylglycerol?

A

Pancreatic lipase

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47
Q

What is TG incorporated into and released into the lymphatic system?

A

Chylomicrons

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48
Q

What are chylomicrons primarily composed of?

A

TG; also contain cholesterol and phospholipids

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49
Q

Where do chylomicrons deliver FA to?

A

Muscle and adipose tissue

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50
Q

What is an intermediate in TG synthesis?

A

A Glycerophospholipid

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51
Q

What is phosphatidic acid formed from?

A

Glycerol-3-P

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52
Q

What enzyme is used to form a triacylglycerol from diacylglycerol?

A

Diglyceride acyltransferase

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53
Q

What is LPL secreted in response to?

A

Insulin

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54
Q

What enzyme is activated to increase lipolysis?

A

Hormone sensitive lipase

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55
Q

What is the role of phospholipases?

A

To cleave bonds in the lysosomes, cytosol and cellular membranes.

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56
Q

What glycerophospholipids are produced by the 1st mechanism used to add the head group to phosphatidic acid?

A

Phosphatidylcholine; phosphatidylethanolamine; phosphatidylserine

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57
Q

What glycerophospholipids are produced by the 2nd mechanism used to add the head group to phosphatidic acid?

A

Phosphatidylinositol; Cardiolipin; Phosphatidylglycerol

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58
Q

What is the activation process in the 1st mechanism?

A

The head group is activated by CTP

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59
Q

What is the activation process in the 2nd mechanism?

A

DAG is activated by DAG

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60
Q

What is the alternate path to form phosphatidylcholine?

A

Methaylation of phosphatidylethanolamine

61
Q

Which glycerophospholipids are reversible through interconversion?

A

Phosphatidylethanolamine and Phosphatidylserine

62
Q

What is Cardiolipin?

A

Diphosphatidylglycerol made from phosphatidylglycerol and activated phosphatidic acid CDP-DAG

63
Q

How does Phosphatidylinositol made from CDP-DAG PI become PIP2?

A

After kinase phosphorylation

64
Q

What is 90% of surfactant?

A

Lipid

65
Q

What makes up 90% of the lipids of surfactant?

A

Phospholipid

66
Q

What makes up 75% of the phospholipid of surfactant?

A

Phosphatidylcholine

67
Q

What is required for normal lung function?

A

Surfactant

68
Q

What is the role of surfactant in normal lung function?

A

It reduces surface tension at the air-liquid interface and also contributes to the defense system

69
Q

In whom is neonatal respiratory distress syndrom seen in?

A

Premature infants with insufficient surfactant.

70
Q

What is the standard therapy for infants with Neonatal Respiratory Distress Syndrome?

A

Treating the lungs with replacement surfactant.

71
Q

Which phospholipid peaks right before birth?

A

Phosphatidylcholine

72
Q

What fatty acids are eicosanoids produced from?

A

Arachidonic acid

73
Q

What are glycerolphospholipids broken down by?

A

Phospholipases

75
Q

What are some head groups of glycerophospholipids?

A

Choline, ethanolamine, serine, inositol, glycerol

76
Q

What are some examples of ether glycerolipids?

A

Plasmalogens and Platelet Activating Factor

77
Q

How are complex lipids distributed in a plasma membrane?

A

Assymetricly

78
Q

What are plasmalogens?

A

A major class of mitochondrial lipids. Enriched in myelin and muscle.

79
Q

What is a platelet activating factor (PAF)?

A

It is released from phagocytic blood cells in response to stimuli; causes platelet aggregation, edema, and hypotension.

80
Q

What is the ether lipid structure of plasmalogen?

A

Glycerol with an ether bon to a hydrocarbon tail (long chain alcohol), FA, and head group.

81
Q

How is a PAF differend from a plasmalogen?

A

It has a 2-acetyl and 1-saturated fatty acid

82
Q

What is the 2-fatty acid replaced by in PAF?

A

An acetyl group

83
Q

What does the synthesis of ether glycerolipis require?

A

NADPH, AcylCoA, DHAP, and activated ethanolamine.

84
Q

What is an example of a sphingophospholipid?

A

Sphingomyelin

85
Q

What are some examples of glycolipids?

A

Cerebrosides, Sulfatides, Globosides, Gangliosides

86
Q

What head group do sphingophospholipids have?

A

A P-choline group

87
Q

What head group do glycolipids have?

A

A carbohydrate group

88
Q

What is a ceramide?

A

A Sphingolipid that is a sphingosine+FA which forms the backbone of all other classes of sphingolipids.

89
Q

What type of link does a ceramide have?

A

An amide link

90
Q

What does ceramide synthesis start with?

A

Palmitoyl CoA and Serine

91
Q

What is the reduced form of sphingosine?

A

dihydrosphingosine

92
Q

What are glycolipids?

A

A component of cell membranes; a ceraminde backbone with carbohydrate groups attached.

93
Q

What determines which glycolipid is formed?

A

The nature of the carbohydrate

94
Q

What makes a ceramide a sphingomyelin in membranes?

A

An attached phosphocholine

95
Q

What is mucolipidosis?

A

The inability to degrade glycolipids and sphingomyelin in membranes.

96
Q

Which diseases are associated with mucolipidosis?

A

Gaucher’s, Tay-Sachs, Fabray’s

97
Q

Where are glycolipids predominantly found?

A

In neural cell types

98
Q

What is a neutral glycolipid?

A

Contains one or more sugars.

99
Q

What are some examples of neutral glycolipids?

A

galactocerebrosides, glucocerebrosides; lactosyl-ceramide

100
Q

What is an acidic glycolipid?

A

It is negatively charged at physiological pH; contains N-acetylneuraminic acid (NANA) or sulfate

101
Q

What are some examples of acidic glycolipids?

A

Sulfatides, gangliosides

102
Q

Where does the synthesis of glycolipids occur?

A

In the ER and Golgi

103
Q

How does the synthesis of glycolipids occur?

A

Sequentially add activated glycosyl residues; enzymes recognize specific structures

104
Q

What is PAPS?

A

An activaed sulfate group that transferrs a sulfate for the formation of a sulfatide.

105
Q

What are the multiple kinds of gangliosides?

A

GM1, GM2, GM3, GQ1b

106
Q

What does ceramide synthesis require?

A

Serine, palmitoylCoA, FACoA, NADPH, FAD

107
Q

What additions to ceramindes make distinct sphingolipids like sphingomyelin or globoside?

A

The addition of phosphoryl choline or sugar, acidic sugar, sulfate

108
Q

What are sphingolipids degraded by?

A

Lysosomal enzymes (acid hydrolases-nucleases, proteases, glycosidases, lipases, phosphatases, sulfatases, phospholipases)

109
Q

Where does the degradation of sphingolipids occur?

A

In lysosomes

110
Q

What maintains the low pH in the lysosome?

A

A proton pump

111
Q

What is the enzyme deficiency in Tay-Sachs disease?

A

Hexosaminidase A

112
Q

What is the enzyme deficiency in Sandhoff disease?

A

Hexosaminidase A and B

113
Q

What is the enzyme deficiency in Fabry’s disease?

A

a-galactosidase

114
Q

What is the enzyme deficiency in Krabbe’s disease?

A

B-galactosidase

115
Q

What is the enzyme deficiency in Gaucher’s disease?

A

B-glucosidase

116
Q

What is the enzyme deficiency in Niemann-Pick Disease?

A

Sphingomyelinase

117
Q

What are the characteristics of Gaucher’s disease?

A

B-glucocerebrosidase enzyme deficiency; Accumulate glucocerebroside; autosomal recessive

118
Q

What are the characteristics of Tay-Sach’s disease?

A

Hexosaminidase A is absent; Autosomal recessive; GM2 ganglioside accumulates; rapid and progressive neurodegeneration; blindness, cherry-red macula; muscular weakness; seizures; common in Ashkenazi Jews

119
Q

What are the characteristics of Fabry disease?

A

Deficient in a-galactosidase A; X-linked recessive; accumulation of globosides; reddish-purple skin rash; kidney and heart failure; buring pain in lower extremities.

121
Q

What is a potent regulator of cellular function and is produced by almost all cells?

A

Eicosanoids

122
Q

What are some eicosanoids that are local hormones?

A

Prostaglandins, thromboxanes, leukotrienes

123
Q

What are the functions of Eicosanoids?

A

Participate in the inflammatory response; regulate smooth muscle contraction; increase water and sodium excretion; involved in blood pressure regulation; constrictors and dilators of blood vessels; bronchoconstriction and bronchodilation

124
Q

What is a fatty acid precursor to eicosanoids?

A

Arachidonic acid

125
Q

What are eicosanoids made in respnose to?

A

Cytokine, hormone signals.

126
Q

What are the three pathways for metabolism of arachidonic acid?

A

Cyclooxgenase, lipoxygenase, CYP450

127
Q

What do prostaglandins work through?

A

Adenyl cyclase, cAMP, Protein Kinase A

128
Q

What do PGF2, Thromboxane A2 and leukotrienes do in the cytosol?

A

Increase Ca2+

129
Q

What is the lifespan of eicosanoids?

A

Seconds to minutes

130
Q

What is the parent compound of prostaglandins?

A

prostanoic acid

131
Q

What is the level of oxidation of PGA compared to PGF?

A

PGA is most oxidized; PGF is most reduced

132
Q

What do PGI2, PGE2, and PGD2 increase?

A

Vasodilation and cAMP?

133
Q

What do PGI2, PGE2, and PGD2 decrease?

A

Platelet aggregation; leukocyte aggregation; IL-1; IL-2; T-cell proliferation; lymphocyte migration.

134
Q

What does PGF2a increase?

A

Vasoconstriction; bronchoconstriction; smooth muscle contraction

135
Q

What is prostacyclin produced by?

A

Vascular endothelial cells

136
Q

What does prostacyclin inhibit?

A

Platelet aggregation

137
Q

What does prostacyclin cause?

A

Vasodilation

138
Q

What is Thromboxane produced by?

A

Platelets

139
Q

What does thromboxane do?

A

Stimulates platelet aggregation and causes vasoconstriction

140
Q

What are prostaglandins inhibited by?

A

NSAIDs that block cyclooxygenase action

141
Q

What does the lipoxygenase pathway form?

A

HETE’s; Leukotrienes; and Lipoxins

142
Q

What are leukotrienes?

A

Synthesized in leukocytes and contain 3 double bonds.

143
Q

What does LTB4 increase?

A

Vascular permeability, T-cell proliferation, leukocyte aggregation, and IL-1 and IL-2.

144
Q

What does LTC4 and LTD4 increase?

A

Vascular permeability and bronchoconstriction.

145
Q

How many hydroxy groups do lipoxins contain?

A

3

146
Q

What are examples of compounds produced from arachidonic acid by cytochrome P450?

A

Epoxide, Dihydroxide

147
Q

In what areas are compounds produced from arachidonic acid by cytochrome P450 active?

A

Ocular, vascular, endocrine, and renal systems.