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Biochemistry > III - Carbohydrates > Flashcards

III - Carbohydrates Flashcards

1
Q

Most abundant organic molecules in nature

A

Carbohydrates

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2
Q

Empiric Formula: (CH2O)n - hydrates of carbon

A

Carbohydrates

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3
Q

Functions of Carbohydrates

A

energy source, storage form of energy, part of cell membranes, structural components

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4
Q

Polymers of repeating sugar units

A

Carbohydrates

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5
Q

One sugar unit

A

monosaccharide

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6
Q

Two sugar units

A

disaccharide

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7
Q

3-10 sugar units

A

oligosaccharide

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8
Q

> 10 sugar units

A

polysaccharide

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9
Q

How many sugar units do monosaccharides have?

A

One

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10
Q

How many sugar units do disaccharides have?

A

Two

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11
Q

How many sugar units do oligosaccharides have?

A

3-10

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12
Q

How many sugar units do polysaccharides have?

A

> 10

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13
Q

The simplest and most basic form of carbohydrate hence cannot be hydrolyzed further

A

monosaccharide

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14
Q

From fruit juices, hydrolysis of cane sugar, maltose and lactose

A

Glucose

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15
Q

The “sugar of the body”, carried by the blood, principal sugar used by the tissues

A

Glucose

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16
Q

Present in urine in DM owing to its high levels in the blood

A

Glucose

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17
Q

Found in fruit juices, honey, hydrolysis of cane sugar and inulin (from the Jerusalem artichoke)

A

Fructose

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18
Q

Can be changed to glucose in the liver and so used in the body

A

Fructose

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19
Q

Hereditary _____ intolerance leads to _____ accumulation and hypoglycemia

A

Fructose

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20
Q

From the hydrolysis of lactose

A

Galactose

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21
Q

Can be changed to glucose in the liver and metabolized, synthesized in the mammary gland to make the lactose of milk, a constituent of glycolipids and glycoproteins

A

Galactose

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22
Q

Failure to metabolize _____ leads to cataracts

A

Galactose

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23
Q

from the hydrolysis of plant mannans and gums

A

Mannose

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24
Q

A constituent of many glycoproteins

A

Mannose

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25
Q

Monosaccharide found in nucleic acids

A

Ribose

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26
Q

Monosaccharides found in glycoproteins

A

Xylose, Arabinose, Mannose

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27
Q

Monosaccharide found in proteoglycans

A

Neuraminic Acid

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28
Q

Monosaccharide found in cardiac tissue

A

Lyxose

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29
Q

Ribose is found in

A

nucleic acids

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30
Q

Xylose is found in

A

glycoproteins

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31
Q

Arabinose is found in

A

glycoproteins

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32
Q

Mannose is found in

A

glycoproteins

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33
Q

Neuraminic Acid is found in

A

proteoglycans

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34
Q

Lyxose is found in

A

cardiac tissue

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35
Q

Condensation product of two monosaccharide units, linked by glycosidic bonds

A

disaccharide

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36
Q

Glucose + Glucose

A

Maltose - α(1→4)

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37
Q

Glucose + Galactose

A

Lactose - β(1→4)

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38
Q

Glucose + Fructose

A

Sucrose - α1→β2

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39
Q

From germinating cereals, malt, digestion by amylase or hydrolysis of starch

A

Maltose

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40
Q

From milk, found in urine during pregnancy

A

Lactose

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41
Q

From sorghum, pineapples, carrots, cane and beet sugar

A

Sucrose

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42
Q

From fungi and yeasts, the major source of insect hemolymph

A

Trehalose

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43
Q

Condensation product of 3-10 monosaccharides, most are not digested by human enzymes, maltotriose

A

Oligosaccharide

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44
Q

Condensation product of >10 monosaccharides, may be linear or branched, easily digested

A

Polysaccharide

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45
Q

Homopolymer of glucose forming an α-glucosidic chain called glucosan or glucan

A

Starch

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46
Q

Most important dietary source of carbohydrate in cereals, potatoes, legumes and other vegetables

A

Starch

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47
Q

Storage polysaccharide in animals (“animal starch”)

A

Glycogen

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48
Q

More highly branched structure than amylopectin with chains of 12-14 α-D-glucopyranose residues with α(1→4) glycosidic linkage with branching via α(1→6) glycosidic bonds

A

Glycogen

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49
Q

Polysaccharide of fructose used to determine the GFR

A

Inulin

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50
Q

Chief constituent of plant cell walls, fiber, cannot be digested

A

Cellulose

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51
Q

Insoluble and consists of β-D-glucopyranose units linked by β(1→4) bonds to form long, straight chains strengthened by cross-linking H-bonds

A

Cellulose

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52
Q

Also known as mucopolysaccharides

A

Glycosaminoglycans

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53
Q

Complex carbohydrates containing amino sugars and uronic acids

A

Glycosaminoglycans

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54
Q

May be attached to a protein molecule to form a proteoglycan

A

Glycosaminoglycans

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55
Q

Also known as mucoproteins, found in cell membranes

A

Glycoproteins

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56
Q

Proteins containing branched or unbranched oligosaccharide chains

A

Glycoproteins

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57
Q

Compounds that have the same chemical formula but different structures

A

Isomers

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58
Q

Compounds that differ in configuration around only one specific carbon atom with the exception of the carbonyl atom

A

Epimers

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59
Q

Pairs of structures that are mirror images of each other

A

Enantiomers/Stereoisomers/Optical Isomers - Dextro- (R), Levo- (L)

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60
Q

More common configuration of sugars in the body (D vs. L)

A

Dextro- (R)

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61
Q

Compounds that differ in configuration (linear/ring)

A

Anomers

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62
Q

More common configuration of sugars in the body (linear vs. cyclic)

A

Cyclic

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63
Q

Linear form of sugars

A

Fischer Projection

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64
Q

Cyclic form of sugars

A

Haworth Projection

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65
Q

5C Ring

A

Furan

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66
Q

6C Ring

A

Pyran

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67
Q

α and β forms of sugar spontaneously interconvert through a process called

A

Mutarotation

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68
Q

Physical digestion in the mouth

A

Mastication

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69
Q

Amylase can only digest _____ glycosidic bonds

A

α(1→4) - glycogen

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70
Q

Facilitates diffusion for all sugars, found in the basement membrane

A

GLUT-2 Transporter

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71
Q

Facilitates diffusion for all sugars, found in the lumen of the SI

A

GLUT-5 Transporter

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72
Q

A secondary active transporter for glucose and galactose (needs Na-K-ATPase), Na/hexose symporter, for glucose and galactose

A

SGLT-1 Trasporter

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73
Q

Tells how fast a carbohydrate is absorbed compared to glucose and galactose

A

Glycemic Index

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74
Q

Fast Absorption: GI _ 1

A

GI > 1

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75
Q

Slow Absorption: GI _ 1

A

GI < 1

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76
Q

Food with ___ GI is beneficial for DM.

A

low GI

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77
Q

Disaccharidase deficiency found in Asians

A

Lactose Intolerance / Lactase Deficiency

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78
Q

Disaccharidase deficiency found in Greenland Eskimos

A

Isomaltase-Sucrase Deficiency

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79
Q

Acquired enzyme deficiency occurs during _____ where enzymes are removed in stool.

A

severe diarrhea

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80
Q

Sum of all the chemical reactions in a cell, tissue or the whole body

A

Metabolism

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81
Q

Synthesis of compounds from smaller raw materials

A

Anabolic Metabolism

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82
Q

An endergonic and divergent process

A

Anabolic Metabolism

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83
Q

Breakdown of larger molecules

A

Catabolic Metabolism

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84
Q

An exergonic and convergent process, usually oxidative

A

Catabolic Metabolism

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85
Q

Produces reducing equivalents and ATP mainly via the ETC

A

Catabolic Metabolism

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86
Q

Crossroads of metabolism, links anabolic and catabolic pathways

A

Amphibolic Metabolism

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87
Q

Regulators of Metabolism: signals from within the cell

A

substrate avaiability, product inhibition, allosteric activators/inhibitors

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88
Q

Regulators of Metabolism: communication between cells

A

gap junctions (direct contact), neurotransmitters (synaptic signaling), hormones (endocrine signaling)

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89
Q

Regulators of Metabolism: second messenger systems

A

calcium/inositol triphosphate (ITP), adenylyl cyclase system (cAMP), guanylate cyclase system (cGMP)

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90
Q

Inositol Triphosphate System: G Protein

A

Gq

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91
Q

Inositol Triphosphate System: Substrate

A

Phosphatidylinositol - found in the cell membrane, acted on by phospholipase C

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92
Q

Inositol Triphosphate System: 2nd Messengers

A

Diacyl glycerol (DAG) - activates protein kinase C, Inositol Triphosphate (ITP) - release intracellular Ca

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93
Q

Membrane-bound enzyme that converts ATP to cyclic AMP (cAMP) in response to hormones

A

Adenylyl cyclase

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94
Q

Hydrolyzes cAMP to 5’-AMP

A

cAMP phosphodiesterase

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95
Q

Adenylyl Cyclase System: G Protein

A

Gs - stimulates, increase cAMP, Gi - inhibits, decrease cAMP

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96
Q

Adenylyl Cyclase System: Substrate

A

ATP

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97
Q

Adenylyl Cyclase System: 2nd Messengers

A

cAMP - activates protein kinase A

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98
Q

GLUT-1 is found in

A

erythrocytes, brain, kidneys, colon, placenta

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99
Q

GLUT-2 is found in

A

liver, pancreatic β-cells, small intestines, kidneys

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100
Q

GLUT-3 is found in

A

brain, kidneys, placenta

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101
Q

GLUT-4 is found in

A

heart and skeletal muscle, adipose

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102
Q

GLUT-5 is found in

A

small intestines

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103
Q

GLUT Transporter in erythrocytes, brain, kidneys, colon, placenta

A

GLUT-1

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104
Q

GLUT Transporter in liver, pancreatic β-cells, small intestines, kidneys

A

GLUT-2

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105
Q

GLUT Transporter in brain, kidneys, placenta

A

GLUT-3

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106
Q

GLUT Transporter in heart and skeletal muscle, adipose

A

GLUT-4

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107
Q

GLUT Transporter in small intestines

A

GLUT-5

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108
Q

Function of GLUT-1

A

uptake of glucose

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109
Q

Function of GLUT-2

A

rapid uptake and release of glucose

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110
Q

Function of GLUT-3

A

uptake of glucose

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111
Q

Function of GLUT-4

A

insulin-stimulated uptake of glucose

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112
Q

Function of GLUT-5

A

absorption of glucose

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113
Q

Major pathway for glucose metabolism that converts glucose into 3C compounds to provide energy

A

Glycolysis

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114
Q

Glycolysis: Location

A

Cytoplasm, all cells

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115
Q

Glycolysis: Substrate

A

Glucose

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116
Q

Glycolysis: End-Product

A

Pyruvate or Lactate - depends on the availability of oxygen or mitochondria

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117
Q

Glycolysis: Rate-Limiting Step

A

fructose 6-phosphate → fructose 1,6-bisphosphate

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118
Q

Glycolysis: Rate-Limiting Enzyme

A

Phosphofructokinase 1

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119
Q

Occurs in cells with mitochondria in the presence of oxygen to produce Pyruvate

A

Aerobic Glycolysis

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120
Q

Occurs in cells without mitochondria without oxygen to produce Lactate

A

Anaerobic Glycolysis

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121
Q

3 Irreversible Steps in Glycolysis

A

Step 1: phosphorylation of glucose, Step 3: phosphorylation of fructose 6-phosphate, Step 10: formation of pyruvate

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122
Q

Glycolysis: Step 1

A

glucose → glucose 6-P

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123
Q

Phosphorylates glucose in the first step of glycolysis

A

Hexokinase or Glucokinase

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124
Q

Phosphorylates glucose in most tissues with low Km (high affinity) and low Vmax, inhibited by glucose 6-P

A

Hexokinase

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125
Q

Phosphorylates glucose in liver parenchyma and pancreatic islets with high Km (low affinity) and high Vmax, inhibited by fructose 6-P, induced by insulin

A

Glucokinase

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126
Q

Glucose phosphorylator that is saturated in the liver and acts in a constant rate to provide glucose 6-P to meet the cell’s need

A

Hexokinase

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127
Q

Glucose phosphorylator that removes glucose from the blood following a meal providing glucose 6-P in excess requirements for glycolysis which is used for glycogenesis and lipogenesis

A

Glucokinase

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128
Q

Glycolysis: Step 3

A

fructose 6-phosphate → fructose 1,6-bisphosphate

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129
Q

Phosphofructokinase 1 converts fructose 6-P to _____

A

fructose 1,6-BP

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130
Q

Converts fructose 6-P to fructose 1,6-BP

A

PFK1 - Phosphofructokinase 1

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131
Q

Phosphofructokinase 1 activators

A

fructose 2,6-BP, AMP

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132
Q

Phosphofructokinase 1 inhibitors

A

ATP, Citrate

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133
Q

Phosphofructokinase 2 converts fructose 6-P to _____

A

fructose 2,6-BP

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134
Q

Converts fructose 6-P to fructose 2,6-BP

A

Phosphofructokinase 2

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135
Q

Phosphofructokinase 2 activators

A

well-fed state - high insulin, low glucagon

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136
Q

Phosphofructokinase 2 inhibitors

A

fasting state - low insulin, high glucagon

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137
Q

Glycolysis: Step 10

A

phoshoenolpyruvate (PEP) → pyruvate

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138
Q

Forms pyruvate from PEP in glycolysis

A

Pyruvate kinase

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139
Q

Pyruvate kinase activator

A

fructose 1,6-BP - feedforward mechanism

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140
Q

Pyruvate kinase inhibitors

A

glucagon + cAMP = phosphorylation

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141
Q

ATP Consumption in Glycolysis

A

glucose → glucose 6-P (hoxokinase or glucokinase), fructose 6-phosphate → fructose 1,6-bisphosphate (phosphofructokinase 1)

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142
Q

ATP Production in Glycolysis

A

1,3-biphosphoglycerate → 3-phosphoglycerate (phosphoglycerate kinase), phosphoenolpyruvate → pyruvate (pyruvate kinase)

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143
Q

NADH Production in Glycolysis

A

glyceraldehyde 3-phosphate → 1,3-bisphosphoglycerate (glyceraldehyde 3-phosphate dehydrogenase)

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144
Q

Aerobic Glycolysis: Pyruvate

A

enters the Citric Acid Cycle

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145
Q

Aerobic Glycolysis: ATP Yield

A

6 or 8

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146
Q

Anaerobic Glycolysis: Pyruvate

A

reduced to lactate by NADH

147
Q

Anaerobic Glycolysis: ATP Yield

A

2

148
Q

NADH Shuttle found in liver, kidneys and brain

A

Malate-Aspartate Shuttle

149
Q

Malate-Aspartate Shuttle is found in

A

liver, kidneys and brain

150
Q

Malate-Aspartate Shuttle yields _ ATP

A

3 ATP

151
Q

NADH Shuttle found in skeletal muscle and brain

A

Glycerol Phosphate Shuttle

152
Q

Glycerol Phosphate Shuttle is found in

A

skeletal muscle and brain

153
Q

Glycerol Phosphate Shuttle yields _ ATP

A

2 ATP

154
Q

Strictly glycolytic organs

A

RBCs, testes, lens, cornea, kidney medulla, WBCs

155
Q

Used to reduce pyruvate to lactate

A

NADH

156
Q

Conversion to lactate is the major fate of pyruvate in

A

RBCs, testes, lens, cornea, kidney medulla, WBCs

157
Q

Found in RBCs where phosphoglycerate kinase is bypassed

A

2,3-bisphosphoglycerate (2,3-BPG)

158
Q

Catalyzes 1,3-BPG → 2,3-BPG

A

bisphosphoglycerate mutase

159
Q

Catalyzed by bisphosphoglycerate mutase

A

1,3-BPG → 2,3-BPG

160
Q

Inhibits pyruvate dehydrogenase by binding to lipoic acid, competes with inorganic phosphate as a substrate for glyceraldehyde 3-P dehydrogenase

A

Arsenic (Pentavalent)

161
Q

Most common enzyme deficiency in glycolysis, manifests as intravascular hemolytic anemia

A

Pyruvate Kinase Deficiency

162
Q

Glycolytic enzyme deficiency which manifests as low exercise capacity particularly on high carbohydrate diets

A

Muscle Phosphofructokinase Deficiency

163
Q

Fate of Pyruvate: Citric Acid Cycle

A

Acetyl CoA (pyruvate dehydrogenase)

164
Q

Fate of Pyruvate: Anaerobic Glycolysis

A

Lactate (lactate dehydrogenase)

165
Q

Fate of Pyruvate: Fermentation

A

Ethanol (pyruvate decarboxylase)

166
Q

Fate of Pyruvate: Gluconeogenesis

A

Oxaloacetate (pyruvate carboxylase)

167
Q

Pyruvate dehydrogenase coenzymes

A

Lipoic Acid, NAD, FAD, Thiamine pyrophosphate (B1 derivative), Coenzyme A

168
Q

Pyruvate dehydrogenase substrate

A

Pyruvate

169
Q

Pyruvate dehydrogenase products

A

Acetyl CoA, NADH, CO2

170
Q

Pyruvate dehydrogenase activators

A

NAD, CoA, Pyruvate

171
Q

Pyruvate dehydrogenase inhibitors

A

NADH, Acetyl CoA, ATP

172
Q

Most common cause of congenital lactic acidosis, x-linked dominant, dec. acetyl CoA deprives brain causing psychomotor retardation and death, treated with ketogenic diet

A

Pyruvate Dehydrogenase Deficiency

173
Q

An acquired pyruvte dehydrogenase deficiency aggravated by thiamine deficiency leading to fatal pyruvic and lactic acidosis

A

Chronic Alcoholism

174
Q

Final common pathway for the aerobic oxidation of all nutrients

A

Tricarboxylic Acid Pathway/Krebs Cycle/Citric Acid Cycle

175
Q

Provides majority of ATP for energy, gluconeogenesis from AA skeletons, building blocks for AA and heme (succinyl CoA)

A

TCA Pathway

176
Q

TCA Pathway: Functions

A

provides majority of ATP for energy, gluconeogenesis from AA skeletons, building blocks for AA and heme (succinyl CoA)

177
Q

TCA Pathway: Location

A

Mitochondrial matrix (except succinyl dehydrogenase - inner mitochondrial membrane), all cells with mitochondria

178
Q

TCA Pathway: Substrate

A

Acetyl CoA

179
Q

TCA Pathway: Products

A

3 CO2, GTP, 4 NADH, FADH

180
Q

TCA Pathway: Rate-Limiting Step

A

isocitrate → α-ketoglutarate

181
Q

TCA Pathway: Rate-Limiting Enzyme

A

isocitrate dehydrogenase

182
Q

TCA Pathway: ATP Yield from Acetyl CoA

A

12

183
Q

TCA Pathway: ATP Yield from Pyruvate

A

15

184
Q

TCA Pathway Sequence

A

Citrate 6C, Isocitrate 6C, α-Ketoglutarate 5C, Succinyl CoA 4C, Succinate 4C, Fumarate 4C, Malate 4C, Oxaloacetate 4C

185
Q

Acetyl CoA + Oxaloacetate → Citrate

A

Citrate Synthase

186
Q

Catalyzed by citrate synthase

A

Acetyl CoA + Oxaloacetate → Citrate

187
Q

Citrate → Isocitrate (isomerization)

A

Aconitase

188
Q

Catalyzed by aconitase

A

Citrate → Isocitrate (isomerization)

189
Q

Inhibits aconitase

A

Fluooroacetate (rat poison)

190
Q

Isocitrate → α-Ketoglutarate

A

Isocitrate dehydrogenase (rate-limiting enzyme)

191
Q

Catalyzed by isocitrate dehydrogenase

A

Isocitrate → α-Ketoglutarate (rate-limiting step)

192
Q

Products from Isocitrate → α-Ketoglutarate (isocitrate dehydrogenase)

A

CO2, NADH

193
Q

α-Ketoglutarate → Succinyl CoA

A

α-Ketoglutarate dehydrogenase

194
Q

Catalyzed by α-ketoglutarate dehydrogenase

A

α-Ketoglutarate → Succinyl CoA

195
Q

Coenzymes for α-ketoglutarate dehydrogenase

A

Thiamine Pyrophosphate (B1 derivative), Lipoic Acid, FAD

196
Q

Products from α-Ketoglutarate → Succinyl CoA (α-ketoglutarate dehydrogenase)

A

CO2, NADH

197
Q

Inhibits α-ketoglutarate dehydrogenase

A

Arsenite

198
Q

Succinyl CoA → Succinate

A

Succinate thiokinase

199
Q

Catalyzed by succinate thiokinase

A

Succinyl CoA → Succinate

200
Q

Product from Succinyl CoA → Succinate (succinate thiokinase)

A

GTP (ATP equivalent)

201
Q

Succinate → Fumarate

A

Succinate dehydrogenase

202
Q

Catalyzed by succinate dehydrogenase

A

Succinate → Fumarate

203
Q

Product from Succinate → Fumarate (succinate dehydrogenase)

A

FADH2

204
Q

Fumarate → Malate

A

Fumarase (fumarate hydratase)

205
Q

Catalyzed by fumarase (fumarate hydratase)

A

Fumarate → Malate

206
Q

Malate → Oxaloacetate

A

Malate dehydrogenase

207
Q

Catalyzed by malate dehydrogenase

A

Malate → Oxaloacetate

208
Q

Product of Malate → Oxaloacetate (malate dehydrogenase)

A

NADH

209
Q

TCA Intermediates: _____ delivers acetyl CoA to the cytoplasm for fatty acid synthesis via _____ shuttle.

A

Citrate

210
Q

TCA Intermediates: Used for heme synthesis and activation of ketone bodies in extrahepatic tissues

A

Succinyl CoA

211
Q

TCA Intermediates: May be used for gluconeogenesis

A

Malate

212
Q

Production of new glucose

A

Gluconeogenesis

213
Q

Gluconeogenesis can produce glucose from these intermediates

A

intermediates of glycolysis and the TCA cycle, glycerol from TGs, lactate through the Cori Cycle, carbon skeletons (α-ketoacids) of glucogenic AAs

214
Q

Gluconeogenesis: Location

A

liver (90%), kidney (10%, 40% during fasting), both in the mitochondria and cytoplasm

215
Q

Gluconeogenesis: Substrate

A

Pyruvate

216
Q

Gluconeogenesis: Product

A

Glucose

217
Q

Gluconeogenesis: Rate-Limiting Step

A

fructose 1,6-bisphosphate → fructose 6-phosphate

218
Q

Gluconeogenesis: Rate-Limiting Enzyme

A

Fructose 1,6-bisphosphatase

219
Q

Lactate generated during anaerobic metabolism is brought to the liver the be converted to glucose via hepatic gluconeogenesis.

A

Cori Cycle

220
Q

The Cori Cycle uses _ ATP to produce glucose.

A

4 ATP

221
Q

Important Steps in Gluconeogenesis

A

Step 10: pyruvate → OAA → PEP, Step 3: fructose 1,6-BP → fructose 6-P, Step 1: glucose 6-P → glucose

222
Q

Gluconeogenesis: Step 10

A

pyruvate → OAA (pyruvate carboxylase), OAA → PEP (PEP carboxykinase)

223
Q

Pyruvate → OAA

A

Pyruvate carboxylase

224
Q

Pyruvate carboxylase requires

A

Biotin

225
Q

Function of Carboxylases

A

Attaches a carbon atom using CO2 as a substrate

226
Q

3 Carboxylase Reactions

A

pyruvate → OAA (pyruvate carboxylase), acetyl CoA → malonyl CoA (acetyl CoA carboxylase), propionyl CoA → succinyl CoA (propionyl CoA carboxylase)

227
Q

Catalyzed by pyruvate carboxylase

A

Pyruvate → OAA

228
Q

OAA → PEP

A

PEP carboxykinase

229
Q

PEP carboxykinase requires

A

GTP

230
Q

Catalyzed by PEP carboxykinase

A

OAA → PEP

231
Q

Gluconeogenesis: Step 3

A

fructose 1,6-BP → fructose 6-P (fructose 1,6-bishosphatase) - rate-limiting step

232
Q

Fructose 1,6-BP → Fructose 6-P

A

Fructose 1,6-bishosphatase

233
Q

Catalyzed by fructose 1,6-bishosphatase

A

Fructose 1,6-BP → Fructose 6-P

234
Q

Fructose 1,6-bishosphatase activator

A

ATP

235
Q

Fructose 1,6-bishosphatase inhibitors

A

Fructose 2,6-BP, AMP

236
Q

Performs dual functions: promote glycolysis and inhibit gluconeogenesis

A

Fructose 2,6-BP

237
Q

Functions of Fructose 2,6-BP

A

activates phophofructokinase 1 (glycolysis), inhibits fructose 1,6-bishosphatase (gluconeogenesis

238
Q

Gluconeogenesis: Step 1

A

glucose 6-P → glucose (glucose 6-phosphatase) - final step, shared with glycogen degradation

239
Q

Glucose 6-P → Glucose occurs in

A

liver and kidneys only (muscle lacks glucose 6-phosphatase hence muscle glycogen can only be used by muscle itself)

240
Q

Gluconeogenesis: Regulation

A

circulating levels of glucagon, availability of glucognic substrates, allosteric activation by acetyl CoA, allosteric inhibition by AMP

241
Q

Gluconeogenesis: Energy Expenditure

A

4 ATP, 2 GTP, 2 NADH

242
Q

In hyperglycemia, the glomerular filtrate may contain more glucose that can be reabsorbed. This occurs when the venous blood glucose concentration exceeds 9.5-10 mmol/L (renal threshold).

A

Glucosuria

243
Q

In _____ high amounts of NADH is produced resulting in _____.

A

alcoholism, hypoglycemia

244
Q

When alcohol is consumed, high amounts of cytoplasmic NADH is produced by

A

alcohol dehydrogenase, acetaldehyde dehydrogenase

245
Q

High amounts of NADH favors

A

pyruvate → lactate, OAA → malate, DHAP → glycerol 3-phosphate

246
Q

NADH diverts pyruvate to lactate and OAA to malate resulting in

A

decreased gluconeogenesis → hypoglycemia

247
Q

High fetal glucose consumption results in

A

hypoglycemia in pregnancy

248
Q

Hyperinsulinemia in pregnancy is due to _____ and causes _____.

A

high estrogen, fasting hypoglycemia

249
Q

Insulin resistance in pregnancy is due to _____ and causes _____.

A

high human placental lactogen (HPL), post-prandial hyperglycemia

250
Q

Causes of Hypoglycemia in the Neonate

A

premature and LBW babies have little adipose, enzymes for gluconeogenesis are not yet completely functional

251
Q

Major storage carbohydrate in animals

A

Glycogen

252
Q

A branched polymer f D-glucose, uses α(1→4) glycosidic bonds for elongation and α(1→6) glycosidic bonds for branching

A

Glycogen

253
Q

Glycogen: Storage

A

liver - 100g, 6% of liver, muscle - 400g, < 1% of muscle

254
Q

Glycogen: Primary Bond

A

α(1→4) - 8-10 glucosyl residues

255
Q

Glycogen: Branching Bond

A

α(1→6)

256
Q

Synthesis of new glycogen molecules from α-D-glucose

A

Glycogenesis

257
Q

Glycogenesis: Location

A

Cytosol, liver and muscle

258
Q

Glycogenesis: Substrates

A

UDP-glucose, ATP, UTP, glycogenin (core, primer protein)

259
Q

Glycogenesis: Product

A

Glycogen

260
Q

Glycogenesis: Rate-Limiting Step

A

addition of α(1→4) bonds

261
Q

Glycogenesis: Rate-Limiting Enzyme

A

Glycogen synthase

262
Q

Important Steps in Glycogenesis

A

glucose 6-P → glucose 1-P, synthesis of UDP-glucose, elongation of glycogen chain, formation of branches

263
Q

Glucose 6-P → Glucose 1-P

A

Phosphoglucomutase

264
Q

Catalyzed by phosphoglucomutase

A

Glucose 6-P → Glucose 1-P

265
Q

Synthesis of UDP-glucose: Enzyme

A

UDP-glucose phosphorylase

266
Q

Catalyzed by UDP-glucose phosphorylase

A

Synthesis of UDP-glucose

267
Q

Synthesis of UDP-glucose: Substrates

A

glucose 1-P, UTP

268
Q

Elongation of glycogen chain

A

Glycogen synthase forms α(1→4) glycosidic bonds between glucose residues at the non-reducing end (carbon 4) - rate-limiting step

269
Q

Catalyzed by glycogen synthase

A

Elongation of glycogen chain - forms α(1→4) glycosidic bonds between glucose residues at the non-reducing end (carbon 4) - rate-limiting step

270
Q

Formation of branches in glycogen

A

Branching enzyme composed of amylo-α(1→4) → α(1→6) transglucosidase forms new α(1→6) bonds by transferring 5-8 glucosyl residues

271
Q

Catalyzed by branching enzyme - amylo-α(1→4) → α(1→6) transglucosidase

A

Formation of branches in glycogen - forms new α(1→6) bonds by transferring 5-8 glucosyl residues

272
Q

1-4 residues remaining on a branch after glycogen phosphorylase has already shortened it

A

Limit Dextrin

273
Q

Shortening of glycogen chains to produce molecules of α-D-glucose

A

Glycogenolysis

274
Q

Glycogenolysis: Location

A

Cytosol, liver and muscle

275
Q

Glycogenolysis: Substrate

A

Glycogen

276
Q

Glycogenolysis: Products

A

free glucose, glucose 1-P, glucose 6-P in muscle

277
Q

Glycogenolysis: Rate-Limiting Step

A

Removal of glucose - breaks α(1→4) bonds

278
Q

Glycogenolysis: Rate-Limiting Enzyme

A

Glycogen phosphorylase

279
Q

1-4 residues remaining on a branch after glycogen phosphorylase has already shortened it

A

Limit Dextrin

280
Q

Important Steps in Glycogenolysis

A

removal of glucose, removal of branches, glucose 1-P → glucose 6-P, lysosomal degradation of glycogen

281
Q

Removal of branches

A

Debranching enzyme composed of α(1→4) → α(1→4) glucantransferase (transfers limit dextrin) and amylo-α(1→6) glucosidase (removes free glucose)

282
Q

Catalyzed by α(1→4) → α(1→4) glucantransferase

A

Transfer of limit dextrin

283
Q

Catalyzed by amylo-α(1→6) glucosidase

A

Removal of free glucose - breaks α(1→6) bonds

284
Q

Glucose 1-P → Glucose 6-P

A

Phosphoglucomutase: liver - further converts glucose 6-P to glucose, muscles - glucose 6-P is the final product

285
Q

Catalyzed by phosphoglucomutase

A

Glucose 1-P → Glucose 6-P: liver - further converts glucose 6-P to glucose, muscles - glucose 6-P is the final product

286
Q

Lysosomal degradation of glycogen

A

α(1→4) glucosidase (acid maltase)

287
Q

Catalyzed by α(1→4) glucosidase (acid maltase)

A

Lysosomal degradation of glycogen

288
Q

Glycogen synthase activators

A

glucose 6-P, insulin, dephosphorylation, well-fed state

289
Q

Glycogen synthase inhibitors

A

glucagon, epinephrine, phosphorylation, fasting state

290
Q

Glycogen phosphorylase activators

A

Ca in muscle, glucagon, epinephrine, phosphorylation, fasting state

291
Q

Glycogen phosphorylase inhibitors

A

glucose 6-P, ATP, insulin, dephosphorylation, well-fed state

292
Q

Inherited disorders characterized by deposition of an abnormal type or quantity of glycogen in tissues, 12 types in total

A

Glycogen Storage Diseases

293
Q

Glycogen Storage Disease Type I

A

Von Gierke’s

294
Q

Von Gierke’s: Deficiency

A

glucose 6-phospatase

295
Q

Von Gierke’s: Findings

A

glycogen in liver and renal cells, hypoglycemia + lactic acidosis/ketosis

296
Q

Glycogen in liver and renal cells, hypoglycemia + lactic acidosis/ketosis

A

Von Gierke’s, GSD Type I

297
Q

Glycogen Storage Disease Type II

A

Pompe’s

298
Q

Pompe’s: Deficiency

A

acid maltase (α(1→4) glucosidase)

299
Q

Pompe’s: Findings

A

glycogen in lysosomes, cardiomegaly, heart failure

300
Q

Glycogen in lysosomes, cardiomegaly, heart failure

A

Pompe’s, GSD Type II

301
Q

Glycogen Storage Disease Type III

A

Cori’s

302
Q

Cori’s: Deficiency

A

debranching enzyme

303
Q

Cori’s: Findings

A

glycogen in liver and renal cells, MILD hypoglycemia + lactic acidosis/ketosis

304
Q

Glycogen in liver and renal cells, MILD hypoglycemia + lactic acidosis/ketosis

A

Cori’s, GSD Type III

305
Q

Glycogen Storage Disease Type V

A

McArdle’s

306
Q

McArdle’s: Deficiency

A

skeletal muscle glycogen phosphorylase

307
Q

McArdle’s: Findings

A

glycogen in muscle, muscle cramps + myoglobinuria without lactic acidosis

308
Q

Glycogen in muscle, muscle cramps + myoglobinuria without lactic acidosis

A

McArdle’s, GSD Type V

309
Q

Important source of galactose, found in milk

A

Lactose

310
Q

Lactose is hydrolyzed by lactase in the _____.

A

intestinal brush border

311
Q

All disaccharidases and trisaccharidases are found in the _____.

A

brush border of the intestinal epithelium

312
Q

Important Steps in Galactose Metabolism

A

phosphorylation of galactose, formation of UDP-galactose, use of galactose as a carbon source

313
Q

Phosphorylation of galactose

A

galactose → galactose 1-P (hexokinase or glucokinase)

314
Q

Formation of UDP-galactose (activated form of galactose)

A

galactose 1-P + UDP-glucose → UDP-galactose + glucose 1-P (galactose 1-P uridyltransferase)

315
Q

Use of galactose as a carbon source

A

UDP-galactose → UDP-glucose (UDP-hexose 4-epimerase)

316
Q

Galactokinase deficiency causes _____ and _____.

A

galactosemia, galactosuria

317
Q

Causes cataracts in early childhood

A

Galactokinase Deficiency

318
Q

Enzyme deficient in classic galactosemia

A

Galactose 1-P uridyltransferase

319
Q

Galactitol accumulates causing cataracts within a few days after birth, hepatosplenomegaly and mental retardation

A

Classic Galactosemia

320
Q

Vomiting and diarrhea after milk ingestion, hypoglycemia, liver disease and cirrhosis, lethargy and hypotonia, mental retardation

A

Classic Galactosemia

321
Q

Classic galactosemia is a _____ to breastfeeding.

A

absolute contraindication

322
Q

Important source of fructose, found in honey and fruits

A

Sucrose

323
Q

Sucrose is hydrolyzed by sucrase in the _____.

A

intestinal brush border

324
Q

Has the fastest metabolism and greatest yield of energy among sugars

A

Fructose

325
Q

Important Steps in Fructose Metabolism

A

phosphorylation of fructose, formation of DHAP and glyceraldehyde

326
Q

Phosphorylation of fructose

A

fructose → fructose 1-P (fructokinase or hexokinase)

327
Q

Formation of DHAP and glyceraldehyde

A

fructose 1-P → dihydroxyacetone phosphate (DHAP) + glyceraldehyde (aldolase B)

328
Q

Catalyzed by aldolase A

A

fructose 1,6-BP → DHAP + glycerol 3-P (glycolysis)

329
Q

Catalyzed by aldolase B

A

fructose 1-P → DHAP + glyceraldehyde (fructose metabolism

330
Q

Defective fructokinase, benugn and asymptomatic, only presents as fructosemia and fructosuria

A

Essential Fructosuria

331
Q

Deficiency of aldolase B, autosomal recessive, fructose 1-P accumulates decreasing phosphate, glycogenolysis and gluconeogenesis

A

Fructose Intolerance

332
Q

Severe hypoglycemia and lactic acidosis after fructose ingestion, vomiting, apathy, diarrhea, liver damage, jaundice, proximal renal tubule disorder resembling Fanconi syndrome

A

Fructose Intolerance

333
Q

Important component of glycoproteins, very little contribution to diet

A

Mannose

334
Q

Isomerization between mannose and fructose

A

mannose 6-P → fructose 6-P (phosphomannose isomerase)

335
Q

Sorbitol metabolism in lens, retina, Schwann cells, liver, kidney, placenta, RBCs, ovaries, seminal vesicles

A

glucose → sorbitol (aldose reductase)

336
Q

Sorbitol metabolism only found in the seminal vesicles

A

sorbitol → fructose (sorbitol dehydrogenase) - fructose is the fuel of sperm

337
Q

In DM, there is excess glucose which is converted into sorbitol.The lens and nerves lack sorbitol dehydrogenase. Sorbitol accumulates and causes

A

cataract formation and peripheral neuropathy

338
Q

Produces NADPH and ribose 5-P, metabolic use of 5-carbon sugars

A

Pentose Phosphate Pathway/Hexose Monophosphate Shunt

339
Q

NADPH provides electrons for

A

FA and steroid biosynthesis, reduction of glutathione, cytochrome P450, WBC respiratory burst, nitric oxide synthesis

340
Q

Pentose Phosphate Pathway: Location

A

Cytoplasm, liver, adipose, adrenals, thyroid, testes, RBC, lactating mammaries (high in tissue that produces lipids, low in skeletal muscle and non-lactating mammaries)

341
Q

Pentose Phosphate Pathway: Substrates

A

glucose 6-P

342
Q

Pentose Phosphate Pathway: Products

A

ribose 5-P, fructose 6-P, glyceraldehyde 3-P, NADPH

343
Q

Pentose Phosphate Pathway: Rate-Limiting Step

A

glucose 6-P → 6-phosphogluconate

344
Q

Pentose Phosphate Pathway: Rate-Limiting Enzyme

A

glucose 6-P dehydrogenase

345
Q

Catalyzed by glucose 6-P dehydrogenase

A

glucose 6-P → 6-phosphogluconate

346
Q

Pentose Phosphate Pathway: Phase 1

A

oxidative, irreversible

347
Q

Pentose Phosphate Pathway: Phase 2

A

non-oxidative, reversible

348
Q

Pentose Phosphate Pathway: Phase 1 Enzyme

A

glucose 6-P dehydrogenase

349
Q

Pentose Phosphate Pathway: Phase 2 Enzyme

A

transketolases (requires thiamine)

350
Q

Pentose Phosphate Pathway: Phase 1 Products

A

NADPH, ribulose 5-P

351
Q

Pentose Phosphate Pathway: Phase 2 Products

A

ribose 5-P, glyceraldehyde 3-P, fructose 6-P

352
Q

RBC transketolase activity can be used to diagnose

A

thiamine deficiency

353
Q

Reduced _____ removes H2O2 in a reaction catalyzed by _____.

A

reduced glutathione (G-SH), glutathione peroxidase

354
Q

Reacting with H2O2 oxidizes _____ but only reduced _____ can remove H2O2.

A

oxidized glutathione (G-S-S-G), glutathione

355
Q

Reduced glutathione sequesters harmful H2O2

A

glutathione peroxidase

356
Q

Glutathione peroxidase cofactor

A

Se - selenium

357
Q

Reduced glutathione is recreated using NADPH

A

glutathione reductase

358
Q

Most common disease producing enzyme abnormality in humans

A

Glucose 6-Phosphate Deficiency (G6PD)

359
Q

Decreased NADPH in RBCs and decreased activity of glutathione reductase causing hemolytic anemia due to poor RBC defense against free radicals and peroxides, neonatal jaundice 1-2 days after birth

A

Glucose 6-Phosphate Deficiency (G6PD)

360
Q

Precipitating factors for G6PD

A

Oxidative Stressors: infection (most common), drugs (AAA) - antibiotics (sulfonamides, chloramphenicol), antimalarials (primaquine), anti-pyretics (except ASA and paracetamol, fava beans

361
Q

Altered hemoglobin precipitates within RBCs found in G6PD

A

Heinz BOdies

362
Q

Altered RBcs due to phagocytic removal of Heinz bodies in the spleen

A

Bite cells

363
Q

Converts molecular oxygen into superoxide in leukocytes (especially neutrophils and macrophages) and used in the respiratory burst that kills bacteria

A

NADPH oxidase

364
Q

Deficiency in NADPH oxidase leading to severe, persistent and chronic pyogenic infections caused by catalase (+) bacteria

A

Chronic Granulomatous Disease

Biochemistry (14 decks)

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