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T2 - BioChem midterm 2 > unit 9 > Flashcards

unit 9 Flashcards

1
Q

which 2 essential amino acids can adults synthesize?

A

histidine

arginine

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2
Q

what is the one T amino acid that is non essential

A

tyrosine

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3
Q

oxaloacetate + NH4 –> _____

what is the enzyme needed?

A

aspartate

glutamate dehydrogenase

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4
Q

aKG + NH4 –>

what is the enzyme needed

A

glutamate

glutamate dehydrogenase

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5
Q

glutamate + NH4+ + ATP –>

enzyme needed?

A

glutamine + ADP + Pi

glutamine synthetase

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6
Q

aspartate + NH4+ + ATP –>

enzyme needed?

A

asparagine + ADP + Pi

asparagine synthetase

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7
Q

synthetase?

A

needs ATP

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8
Q

when you have ATP you need?

A

Mg2+

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9
Q

transamination needs 2 things

A

transaminases

B6PO4 (active form of B6)

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10
Q

____ is the primary source of -NH2 in transamination

A

glutamate

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11
Q

reactions of transamination are ____

A

reversible

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12
Q

transamination: the reactiosn use the coenzyme ___ ___ ( active form of B__)

A

pyridoxal phosphate

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13
Q

3 usual products of transamination

A

alanine
aspartate
glutamate

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14
Q

pyruvate + glutamate (NH2) –> ____ + ____
enzyme?
coenzyme?

A

alanine + aKG
alanine aminotransferase
B6PO4

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15
Q

OAA + glutamate (NH2) –> ____ + ____
enzyme?
coenzyme?

A

aspartate + aKG
aspartate aminotransferase
B6PO4

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16
Q

____ and ___ are essential for the synthesis of collagen

A

hydroxyproline and hydroxylysine

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17
Q

hydroxylation of one amino acid to form another requires 2 things

A

O2

aKG

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18
Q

proline + aKG + O2 –> ____ + ____ + ______
enzyme?
2 coenzymes?

A

4- hydroxyproline + succinate + CO2
prolyl hydroxylase
iron and vit C

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19
Q

lysine + aKG + O2 –> ____ + ____ + ______
enzyme?
2 coenzymes?

A

5- hydroxylysine + succinate + CO2
lysyl hydroxylase
iron and vit C

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20
Q

phenylalanine + H4 biopterin + O2 –> ___ + ___ + _____

enxyme?

A

tyrosine + H2 biopterin + H2O

phenylalanine hydroxylase

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21
Q

_____: deficiency of phenylalanine hydroxylase. affects mostly children

A

phenylketonura

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22
Q

serine + folate –> ___ + ___

enzyme?

A

glycine + methylene H4 folate

serine hydroxymethyltransferase

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23
Q

why do our bodies make ammonia?

A

soluble carrier of nitrogen waste

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24
Q

high concentrations of ammonia are highly _____

A

toxic especially to the CNS

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25
Q

ammonia can be recycled into amino acids or turned into ___ in the liver

A

UREA

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26
Q

urea has ___ toxicity

A

low

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27
Q

there are 4 major reactions classes to making ammonia

A

glutaminase and asparaginase
transamination
glutamate dehydrogenase reaction
l amino acid oxidase reaction

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28
Q

glutaminase and asparaginase hydroglyze ___ and ___ to form ammonia

A

glutamine

asparagine

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29
Q

glutaminase and asparaginase hydroglyze glutamine and asparagine to form ammonia nd ____ and ____

A

glutamate

aspertate

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30
Q

transamination to form ammonia occurs in ____ tissues

A

all

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31
Q

transaminations to form ammonia involve ____

A

aKG

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32
Q

transamination to form ammonia transfer amino groups from other amino acids to _____

A

glutamate

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33
Q

transamination to form ammonia requires vit B__

A

6

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34
Q

glutamate dehydrogenase reaction is the primary enzyme for elimination of ____

A

nitrogen

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35
Q

L amino acid oxidase reaction to form ammonia primarily occurs in ___ and ___

A

liver and kidney

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36
Q

L amino acid oxidase reaction to form ammonia acitvity is ____

A

low

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37
Q

L amino acid oxidase is a ____ that contains FMN and can auto oxidize

A

flavoprotein

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38
Q

____: an enzyme that is covalently bonded to riboflaven (B2)

A

flavoprotein

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39
Q

SLIDE 20….

A

AMAZING

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40
Q

biosynthesis of urea initially begins in the ++++ of cells, but remaining reactions occur in the ____ of hepatocytes

A

mitochondria

cytoplasm

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41
Q

5 reactions of the urea cycle: 2 ___ and 3 ____

A

mitochondrial

cytosolic

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42
Q

urea cycle converts 2 amino groups and a carbon from HCO3- to urea ( one from ___ and one from ____)

A

NH4+ (free ammonia

aspertate

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43
Q

urea cycle:

COSTS: ___ high energy phosphate bonds

A

4

3 ATPs hydrolyzed to 2ADP and 1 AMP

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44
Q

urea cycle:

GAIN: __ NADH

A

2 NADH = 4 ATP equivalents

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45
Q

urea cycle is the site of ___ synthesis

A

arginine

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46
Q

____: deficiency of urea cycle enzymes impairs urea formation… usually in children

A

hyperammonemia

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47
Q

signs and symptoms of hyperammonemia

A 
vomiting
avoidance of high protein foods
intermittent ataxia
irritability
lethargy
mental retardation
48
Q

____ ___: can produce ammonia intoxication and inability to excrete nitrogen

A

liver disease/surgery

49
Q

signs and symptoms o fliver disease/surgery

A 
asterixis (jerking_
slurring of speech
blurred visioin
coma
death
50
Q

most excess amino acids yield ____ OR ___/___ kreb cycle intermediates

A

pyruvate

4C/5C

51
Q

most proteins are ____ except 2

A

glycogenic
leucine
lysine

52
Q

amino acids that can generate acetyl CoA are ____

A

ketogenic

53
Q

2 ketogenic amino acids

A

leucine

lysine

54
Q

ketogenic amino acids used for 3 things

A

ketogenesis
lipogenesis
krebs

55
Q

glycogenic amino acids used for 2 things

A

gluconeogenesis

krebs

56
Q

keton bodies are generally from the break down of ___ ___ and produced from ____. mainly in the ___ of hepatocytes

A

fatty acids
acetyl coA
mitochondria

57
Q

3 ketone bodies

A

acetoacetate
beta hydroxybutyrate
acetone

58
Q

protein ____ a major source of energy

A

isnt

59
Q

protein is primaily metabolized during ___

A

exercise

60
Q

protein used comes from ___ ___ protein

A

skeletal muscle

61
Q

3 essential branch chain amino acids

A

valine
leucine
isoleucine

62
Q

during exercise the branch chain amino acids are needed to make glucose… they are ___ to get their C skeletons

A

deaminated

63
Q

SLIDE 37

A

chart

64
Q

victims with amino acid metabolism issues usually present with ____ ____

A

mental retardation

65
Q

____ ____: blockage of the recurrent step and step to make formate therefore, makes formate which leads to kidney stones (SLIDE 39)

A

primary hyperoxaluria

66
Q

____: tyrosine metabolism is interrupted and the intermediate is excreted in urine. later is can complicate arthritis

A

alkaptonuria

67
Q

_____: phenylalanine metabolism is interrupted.

A

PKU

68
Q

PKU 1 is due to the deficiency in _____ ____

A

phenylalanine hydroxylase

69
Q

signs and symptoms of PKU

A 
seizures
psychosis
eczema
mousy odor
mental retardation
70
Q

___ ____ urine disease: branch chained amino acid metabolism is interrupted

A

maple syrup

71
Q

maple syrup urine disease is due to a deficiency in ___ and ___

A

aKeto acid decarboxylase

72
Q

signs and symptoms of maple syrup urine disease

A 
anorexia
vomiting
lethargy
brain damage
death within 1 year if untreated
73
Q

_____ _____ dysfunction: cysteine amino acid metabolism is interrupted

A

sulfite oxidase

74
Q

cysteine amino acid metabolism is interrupted in sulfite oxidase dysfunction due to a defective ____ ___ ___

A

sulfite oxidase complex

75
Q

signs and symptoms of sulfite oxidase dysfunction

A

functionally decerebrate

76
Q

____ ____ functions as an energy storage molecule due to high energy phosphoester bond

A

creatine phosphate

77
Q

creatine phosphate functions as an energy storage molecule due to high energy ____ bond

A

phosphoester bond

78
Q

creatine phosphate: energy is transferred to ADP to form ATP by _____ ____

A

creatine (phospho)kinase

CP/CPK

79
Q

catecholamines are derived from ___

A

tyrosine

80
Q

3 catecholamines

A

dopamine
epinephrine
norepinephrine

81
Q

serotonin is derived from ____ by decarboxylation of 5-hydroxytryptophan

A

tryptophan

82
Q

enzymes that help make serotonin

A

5-hydroxytryptophan decarboxylase

aromatic L amino acid decarboxylase

83
Q

___ ___: responsible for metabolism of serotonin and catecholamines

A

monoamine oxidase

84
Q

Monoamine oxidase inhibitors allow longer ___ function

A

neurotransmitter

85
Q

natural MAOIs

A

tyramine in cheese and wine

86
Q

GABA is made by the decarboxylation of ____ by glutamate decarboxylase

A

glutamate

87
Q

histamine is made by the decarboxylation of ___ by aromatic L amino acid decarboxylate

A

histidine

88
Q

melanins are heterogenous macromolecules derived from ____

A

tyrosine

89
Q

melanins are resposible for ___ of eyes, skin, hair

A

color

90
Q

melanins are synthesized in ____

A

melanocytes

91
Q

melanocytes are found in the bottom layer of the ___ and in the middle layer of the ___

A

epidermis

eye

92
Q

tyrosine is converted to DOPA by ____

A

tyrosinase

93
Q

___ ___ dye to defect in tyrosinase

A

oculocutaneous albinism

94
Q

eumelanins

A

balck or brown eyes

95
Q

pheomelanins

A

yellow to reddish brown

96
Q

trichromes

A

color varies

97
Q

____ posses a ring structure called porphin

A

porphyrin

98
Q

____: complex of protoporphyrin + Mg2+ instead of Fe2+

A

chlorophyll

99
Q

protoporphyrin III + Fe2+ –> Heme

enzyme?

A

ferrochelatase

100
Q

4 side chains that might be attached to porphyrin

A

acetyl
propionyl
methyl
vinyl

101
Q

the biosynthesis of heme occurs in most cells except ___

A

RBCs

102
Q

____ + succinyl CoA –> protoporphyrin III

A

glycine

103
Q

2 precursors for heme synthesis

A

Fe2+

vitamin B6

104
Q

___: group of disorders due to defects in heme biosynthesis

A

porphyria

105
Q

accumulation of heme intermediates causes (4)

A

discoloration of urine and skin
photosensitivity
acute abdominal pain
neuropathy

106
Q

RBCs release most of the heme for degradation in reticuloendothelial cells of ____, ___ and ___ ___

A

liver
spleen
bone marrow

107
Q

initial step of degradation of the heme by ___ ___ __ ___

A

microsomal heme oxygenase system

108
Q

what happens in the catabolism of heme:

Fe2+ oxidized to ___

A

Fe3+

109
Q

what happens in the catabolism of heme:

porphyrin ring broken to ___ ___

A

biliverdin IX

110
Q

____ __: is insoluble and bound to serum albumin for transport

A

biliverdin IX

111
Q

biliverdin IX is absorbed by parenchymal cells of ____ for metabolism

A

liver

112
Q

bilirubin IX has to be converted to bilirubin - diglucuronide using the enzyme ___ ____ so that it can become soluble and excreted in bile

A

glucuronyl transferase

113
Q

____ ____ cycle recycles bilirubin

A

enterohepatic urobiliogen

114
Q

____: when blood concentration of bilirubin is greater than or equal to 1mg/dL

A

hyperbilirubinemia

115
Q

____ occurs when blood bilirubin concentration is greater than or equal to 20-25 mg/dL and penetrates the tissues

A

jaundice

116
Q

unconjugated hyperbilirubinemia is when there is no bilirubin detected in ____

A

urine

117
Q

conjugated hyperbilirubinemia is when bilirubin diglucuronate is found in ___

A

urine

T2 - BioChem midterm 2 (4 decks)

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