unit 9 Flashcards
1
Q
which 2 essential amino acids can adults synthesize?
A
histidine
arginine
2
Q
what is the one T amino acid that is non essential
A
tyrosine
3
Q
oxaloacetate + NH4 –> _____
what is the enzyme needed?
A
aspartate
glutamate dehydrogenase
4
Q
aKG + NH4 –>
what is the enzyme needed
A
glutamate
glutamate dehydrogenase
5
Q
glutamate + NH4+ + ATP –>
enzyme needed?
A
glutamine + ADP + Pi
glutamine synthetase
6
Q
aspartate + NH4+ + ATP –>
enzyme needed?
A
asparagine + ADP + Pi
asparagine synthetase
7
Q
synthetase?
A
needs ATP
8
Q
when you have ATP you need?
A
Mg2+
9
Q
transamination needs 2 things
A
transaminases
B6PO4 (active form of B6)
10
Q
____ is the primary source of -NH2 in transamination
A
glutamate
11
Q
reactions of transamination are ____
A
reversible
12
Q
transamination: the reactiosn use the coenzyme ___ ___ ( active form of B__)
A
pyridoxal phosphate
13
Q
3 usual products of transamination
A
alanine
aspartate
glutamate
14
Q
pyruvate + glutamate (NH2) –> ____ + ____
enzyme?
coenzyme?
A
alanine + aKG
alanine aminotransferase
B6PO4
15
Q
OAA + glutamate (NH2) –> ____ + ____
enzyme?
coenzyme?
A
aspartate + aKG
aspartate aminotransferase
B6PO4
16
Q
____ and ___ are essential for the synthesis of collagen
A
hydroxyproline and hydroxylysine
17
Q
hydroxylation of one amino acid to form another requires 2 things
A
O2
aKG
18
Q
proline + aKG + O2 –> ____ + ____ + ______
enzyme?
2 coenzymes?
A
4- hydroxyproline + succinate + CO2
prolyl hydroxylase
iron and vit C
19
Q
lysine + aKG + O2 –> ____ + ____ + ______
enzyme?
2 coenzymes?
A
5- hydroxylysine + succinate + CO2
lysyl hydroxylase
iron and vit C
20
Q
phenylalanine + H4 biopterin + O2 –> ___ + ___ + _____
enxyme?
A
tyrosine + H2 biopterin + H2O
phenylalanine hydroxylase
21
Q
_____: deficiency of phenylalanine hydroxylase. affects mostly children
A
phenylketonura
22
Q
serine + folate –> ___ + ___
enzyme?
A
glycine + methylene H4 folate
serine hydroxymethyltransferase
23
Q
why do our bodies make ammonia?
A
soluble carrier of nitrogen waste
24
Q
high concentrations of ammonia are highly _____
A
toxic especially to the CNS
25
ammonia can be recycled into amino acids or turned into ___ in the liver
UREA
26
urea has ___ toxicity
low
27
there are 4 major reactions classes to making ammonia
glutaminase and asparaginase
transamination
glutamate dehydrogenase reaction
l amino acid oxidase reaction
28
glutaminase and asparaginase hydroglyze ___ and ___ to form ammonia
glutamine
| asparagine
29
glutaminase and asparaginase hydroglyze glutamine and asparagine to form ammonia nd ____ and ____
glutamate
| aspertate
30
transamination to form ammonia occurs in ____ tissues
all
31
transaminations to form ammonia involve ____
aKG
32
transamination to form ammonia transfer amino groups from other amino acids to _____
glutamate
33
transamination to form ammonia requires vit B__
6
34
glutamate dehydrogenase reaction is the primary enzyme for elimination of ____
nitrogen
35
L amino acid oxidase reaction to form ammonia primarily occurs in ___ and ___
liver and kidney
36
L amino acid oxidase reaction to form ammonia acitvity is ____
low
37
L amino acid oxidase is a ____ that contains FMN and can auto oxidize
flavoprotein
38
____: an enzyme that is covalently bonded to riboflaven (B2)
flavoprotein
39
SLIDE 20....
AMAZING
40
biosynthesis of urea initially begins in the ++++ of cells, but remaining reactions occur in the ____ of hepatocytes
mitochondria
| cytoplasm
41
5 reactions of the urea cycle: 2 ___ and 3 ____
mitochondrial
| cytosolic
42
urea cycle converts 2 amino groups and a carbon from HCO3- to urea ( one from ___ and one from ____)
NH4+ (free ammonia
| aspertate
43
urea cycle:
| COSTS: ___ high energy phosphate bonds
4
| 3 ATPs hydrolyzed to 2ADP and 1 AMP
44
urea cycle:
| GAIN: __ NADH
2 NADH = 4 ATP equivalents
45
urea cycle is the site of ___ synthesis
arginine
46
____: deficiency of urea cycle enzymes impairs urea formation... usually in children
hyperammonemia
47
signs and symptoms of hyperammonemia
```
vomiting
avoidance of high protein foods
intermittent ataxia
irritability
lethargy
mental retardation
```
48
____ ___: can produce ammonia intoxication and inability to excrete nitrogen
liver disease/surgery
49
signs and symptoms o fliver disease/surgery
```
asterixis (jerking_
slurring of speech
blurred visioin
coma
death
```
50
most excess amino acids yield ____ OR ___/___ kreb cycle intermediates
pyruvate
| 4C/5C
51
most proteins are ____ except 2
glycogenic
leucine
lysine
52
amino acids that can generate acetyl CoA are ____
ketogenic
53
2 ketogenic amino acids
leucine
| lysine
54
ketogenic amino acids used for 3 things
ketogenesis
lipogenesis
krebs
55
glycogenic amino acids used for 2 things
gluconeogenesis
| krebs
56
keton bodies are generally from the break down of ___ ___ and produced from ____. mainly in the ___ of hepatocytes
fatty acids
acetyl coA
mitochondria
57
3 ketone bodies
acetoacetate
beta hydroxybutyrate
acetone
58
protein ____ a major source of energy
isnt
59
protein is primaily metabolized during ___
exercise
60
protein used comes from ___ ___ protein
skeletal muscle
61
3 essential branch chain amino acids
valine
leucine
isoleucine
62
during exercise the branch chain amino acids are needed to make glucose... they are ___ to get their C skeletons
deaminated
63
SLIDE 37
chart
64
victims with amino acid metabolism issues usually present with ____ ____
mental retardation
65
____ ____: blockage of the recurrent step and step to make formate therefore, makes formate which leads to kidney stones (SLIDE 39)
primary hyperoxaluria
66
____: tyrosine metabolism is interrupted and the intermediate is excreted in urine. later is can complicate arthritis
alkaptonuria
67
_____: phenylalanine metabolism is interrupted.
PKU
68
PKU 1 is due to the deficiency in _____ ____
phenylalanine hydroxylase
69
signs and symptoms of PKU
```
seizures
psychosis
eczema
mousy odor
mental retardation
```
70
___ ____ urine disease: branch chained amino acid metabolism is interrupted
maple syrup
71
maple syrup urine disease is due to a deficiency in ___ and ___
aKeto acid decarboxylase
72
signs and symptoms of maple syrup urine disease
```
anorexia
vomiting
lethargy
brain damage
death within 1 year if untreated
```
73
_____ _____ dysfunction: cysteine amino acid metabolism is interrupted
sulfite oxidase
74
cysteine amino acid metabolism is interrupted in sulfite oxidase dysfunction due to a defective ____ ___ ___
sulfite oxidase complex
75
signs and symptoms of sulfite oxidase dysfunction
functionally decerebrate
76
____ ____ functions as an energy storage molecule due to high energy phosphoester bond
creatine phosphate
77
creatine phosphate functions as an energy storage molecule due to high energy ____ bond
phosphoester bond
78
creatine phosphate: energy is transferred to ADP to form ATP by _____ ____
creatine (phospho)kinase
| CP/CPK
79
catecholamines are derived from ___
tyrosine
80
3 catecholamines
dopamine
epinephrine
norepinephrine
81
serotonin is derived from ____ by decarboxylation of 5-hydroxytryptophan
tryptophan
82
enzymes that help make serotonin
5-hydroxytryptophan decarboxylase
| aromatic L amino acid decarboxylase
83
___ ___: responsible for metabolism of serotonin and catecholamines
monoamine oxidase
84
Monoamine oxidase inhibitors allow longer ___ function
neurotransmitter
85
natural MAOIs
tyramine in cheese and wine
86
GABA is made by the decarboxylation of ____ by glutamate decarboxylase
glutamate
87
histamine is made by the decarboxylation of ___ by aromatic L amino acid decarboxylate
histidine
88
melanins are heterogenous macromolecules derived from ____
tyrosine
89
melanins are resposible for ___ of eyes, skin, hair
color
90
melanins are synthesized in ____
melanocytes
91
melanocytes are found in the bottom layer of the ___ and in the middle layer of the ___
epidermis
| eye
92
tyrosine is converted to DOPA by ____
tyrosinase
93
___ ___ dye to defect in tyrosinase
oculocutaneous albinism
94
eumelanins
balck or brown eyes
95
pheomelanins
yellow to reddish brown
96
trichromes
color varies
97
____ posses a ring structure called porphin
porphyrin
98
____: complex of protoporphyrin + Mg2+ instead of Fe2+
chlorophyll
99
protoporphyrin III + Fe2+ --> Heme
| enzyme?
ferrochelatase
100
4 side chains that might be attached to porphyrin
acetyl
propionyl
methyl
vinyl
101
the biosynthesis of heme occurs in most cells except ___
RBCs
102
____ + succinyl CoA --> protoporphyrin III
glycine
103
2 precursors for heme synthesis
Fe2+
| vitamin B6
104
___: group of disorders due to defects in heme biosynthesis
porphyria
105
accumulation of heme intermediates causes (4)
discoloration of urine and skin
photosensitivity
acute abdominal pain
neuropathy
106
RBCs release most of the heme for degradation in reticuloendothelial cells of ____, ___ and ___ ___
liver
spleen
bone marrow
107
initial step of degradation of the heme by ___ ___ __ ___
microsomal heme oxygenase system
108
what happens in the catabolism of heme:
| Fe2+ oxidized to ___
Fe3+
109
what happens in the catabolism of heme:
| porphyrin ring broken to ___ ___
biliverdin IX
110
____ __: is insoluble and bound to serum albumin for transport
biliverdin IX
111
biliverdin IX is absorbed by parenchymal cells of ____ for metabolism
liver
112
bilirubin IX has to be converted to bilirubin - diglucuronide using the enzyme ___ ____ so that it can become soluble and excreted in bile
glucuronyl transferase
113
____ ____ cycle recycles bilirubin
enterohepatic urobiliogen
114
____: when blood concentration of bilirubin is greater than or equal to 1mg/dL
hyperbilirubinemia
115
____ occurs when blood bilirubin concentration is greater than or equal to 20-25 mg/dL and penetrates the tissues
jaundice
116
unconjugated hyperbilirubinemia is when there is no bilirubin detected in ____
urine
117
conjugated hyperbilirubinemia is when bilirubin diglucuronate is found in ___
urine
