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MedBiochem > quiz 2.3 AA metab > Flashcards

quiz 2.3 AA metab Flashcards

1
Q

average daily protein turnover

A

250g/day

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2
Q

cases na may positive nitrogen balance

A

growing child
pregnant
convalescing pipz
refeeding after starvation

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3
Q

AA that does not undergo transamination

A

lysine

threonine

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4
Q

coenzyme in transamination

A

pyridoxal phosphate

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5
Q

product after trnsamination

A

alpha ketoacid

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6
Q

ATP and GTP are the positive allosteric effectors for the formation of ____ while ADP and GDP are for ____

A

glutarate

alpha-ketoglutarate

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7
Q

the only dehydrogenase which is non-specific for NAD and NADP

A

glutarate dehydrogenase

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8
Q

oxidase primarily involved in the deamination of lysine

A

L-amino acid oxidase

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9
Q

asparaginase converts asparagine to

A

ammonia and aspartate

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10
Q

important in kidney which helps excrete H in the form of NH4

A

glutaminase

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11
Q

direct deamination of histidine by ___ and ____ produces ___

A

histidase/histidine ammonia lyase

urocanic acid

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12
Q

dehydrative deamination of serine and threonine prodcues ____ and ___ respectively

A

pyruvate

alpha-ketobutyrate

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13
Q

3 ways ammonia is disposed

A

alanine cycle
glutamine cycle
urea cycle

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14
Q

urea cycle aka

A

Krebs-Henseleit cycle

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15
Q

where does urea cycle occur

A

liver

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16
Q

saan galing ung two nitrogens sa urea

A

from NH3 thru glutamate dehydrogenase

from aspartate by transamination

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17
Q

rate limiting enyzme fi the urea cycle

A

carbamoyl phosphate synthase 1

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18
Q

formation of carbamoyl phosphate by CPS1 is driven by

A

cleavage of 2 mols of ATP

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19
Q

ornithine plus carbamoyl phosphate by ornithine transcabamoylase forms

A

citrulline

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20
Q

in the urea cycle, citrulline condenses with aspartate to form ____ catalyzed by ____

A

argininosuccinate

argininosuccinate synthetase

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21
Q

arginine is cleaved by arginase to form

A

ornithine and urea

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22
Q

argininosuccinate is cleaved by ___ to form

A

argininosuccinate lyase

arginine and fumarate

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23
Q

after arginine is cleaved to ornithine and urea, what are the fates of these two

A

urea - excreted trhu urine

ornithine - enters mitcochondria

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24
Q

what component in the urea cycle enters the TCA

A

fumarate

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25
Q

where urea increased, starvation and high protein diet or low protein diet

A

starvation and high protein diet

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26
Q

the allosteric activator of CPS1

A

N-acetylglutamate

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27
Q

positive allosteric activator of NAG synthetase

A

arginine

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28
Q

amino acids which, in the final step of its emtab, can be converted into glucose pyruvate or a TCA cyle intermediate that can be converted to OXAA

A

glucogenic

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29
Q

amino acids which can be converted into fat

A

ketgenic

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30
Q

what are the purely ketogenic AAs

A

leu

lys

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31
Q

what AAs are both keto and glucogenic

A 
phe
ile
trp
tyr
thr
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32
Q

amino acids catabolized via pyruvate

A 
alanine
serine
glycine
cysteine & cystine
threonine
tryptophan
hydroxyproline
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33
Q

in the metab of alanine, the end product ____ whose formation is catalyzed by _____ ultimately results to the formation of ____

A

pyruvate
alanine aminotransferase
OXAA

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34
Q

alanine: keto or gluco?

A

glucogenic

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35
Q

in the first step, serine undergoes ____ by ___ to form ____

A

dehydrative deamination
serine dehydrogenase
pyruvate

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36
Q

pyruvate from serine in its metab is the converted to ___ which is then subsequently oxidize to __ and ___

A

glycine

CO2 and ammonia

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37
Q

catalyzes the 2nd step in the metab of serine

A

serine hydroxymethyltransferase

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38
Q

metab of glycine

A

glycine > (serine hydroxymethyltranferase) > serine > (serine dehydrogenase) > pyruvate

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39
Q

product when glycine is acted upon by D-amino acid oxidase

A

glyoxylate

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40
Q

glycine from serine or from diet can also be oxidized by ___ to yield a second equivalent of ______

A

glycine cleavage complex

N5,N10-methylene THF

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41
Q

universal sulfur donor

A

3’-phosphoadenosine-5’-phosphosulfate (PAPS)

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42
Q

simplest path of cystine

A

catalyzed by liver desulfurase to produce pyruvate

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43
Q

cysteine > (_______) > cysteinesulfinate

A

cysteine dioxygenase

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44
Q

cysteine > (cystein dioxygenase) > ______________

A

ccysteinesulfinate

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45
Q

cysteinesulfinate undergoing transaminattion produces what b

A

beta-sulfinylpyruvate

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46
Q

cysteinesulfinate undergoing decarboxylation and oxidation produces

A

taurine

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47
Q

beta-sulfinylpryuvate undergoing desulfuration yields

A

bisulfite

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48
Q

bisulfite acted upon by sulfite oxidase yields

A

sulfate and H2O2

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49
Q

the sulfate yielded from bisulfate will be used to

A

make PAPS

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50
Q

cystine > (__________) > cysteine

A

NADH linked-cystine reductase

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51
Q

how many moles of L-cysteine formed from a cystine acted upon by cysteine reductase

A

2 moles

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52
Q

the enzyme ___ can transfer the sulfur from one cysteine to another to form _____ and _____-

A

cystathionase

thiocysteine & pyruvate

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53
Q

transamination of cysteine yields __________ which can react with sulfite to form ____ and __

A

beta-mercaptopyruvate/3-mercaptopyruvate

thiosulfate & pyruvate

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54
Q

thiosulfate and thiocysteine can detoxify cyanide by the action of ___ incorporating the sulfur into the cyanide

A

rhodanase

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55
Q

pathway ng propionylCoA to succinylCoA

A

propionylCoA > (carboxylation) > D-methylmalonyl CoA > (racemase) L-methylmalonyl CoA > (mutase) > succinyl CoA

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56
Q

threonine > (serine/threonine dehydrogenase) > _____

A

a-ketobutyrate

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57
Q

a-ketobutyrte > (a-ketoacid dehydrogenase) > _____

A

propionyl CoA

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58
Q

threonine > (threonine dehydrogenase) > ______

A

a-amino-b-ketobutyrate

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59
Q

pathway ng a-amino-b-ketobutyrate hanggang pyruvate pakielaborate nang maging hero na si marcos

A

a-amino-b-ketobutyrate > aminoacetone > methylglyoxal > pyruvate

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60
Q

threonine acted upon by threonine aldolase produces __ and __-

A

acetylCoA and pyruvate

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61
Q

first enzyme of the trp metab is ___ than opens the indole ring

A

iron porphyrin oxygenase

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62
Q

first key branch point of trp metab

A

kynurenine

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63
Q

3 possible products/intermediate after the first branch point of trp metab

A

kynurenate
anthranilate
3-hydroxykynurenine

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64
Q

which of the products from kynurenine is an anticonvulsant

A

kynurenate

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65
Q

a urinary metabolite from trp seen during its metab

A

xanthurenate

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66
Q

xanthurenate came directly from or is a product of what

A

transaminated 3-hydroxykynurenine

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67
Q

2nd key branch point in trp metab

A

2-amino-3-carboxymuconic semialdehyde

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68
Q

the roduct from trp metab that is the precursor of NAD and NADP

A

nicotinate mononucleotide

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69
Q

from the 2nd branching point of trp metab, what are the ultimate products

A

picolinate
nicotinate mononucleotide
glutarylCoA > acetylCoA

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70
Q

in benign hyperhydroxyprolinemia, alin ung wala or blcoked

A

hydroxyproline dehydrogenase

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71
Q

in type 2 hyperprolinemia, alin ung wala or blocked

A

2nd dehydrogenase rxn

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72
Q

ulitmate products of hydroxyproline metab

A

glyoxylate

pyruvate

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73
Q

amino acids catabolized by OXAA

A

aspartate

asparagine

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74
Q

aspartate + oxoglutarate > _______ + glutamate

A

OXAA

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75
Q

amino acids catabolized via a-KG

A 
glutamine
glutamate
histidine
proline
arginine
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76
Q

convert glutamine to glutamate and NH3

A

glutaminase

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77
Q

where is glutaminase prominently found

A

kidney tuules

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78
Q

glutamate is converted to ____ by transamination or by oxidative deamination by glutamate dehyrdrogenase

A

a-KG

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79
Q

glutamate > (_______) > a-KG

A

glutamate drhydrogenase

80
Q

what component in the gutamate dehydrogenase pathway is a substrate for urea cycle

A

NH4

81
Q

ornithine in excess from the cleavage of arginine is transaminated to form

A

glutamate semialdehyde

82
Q

can serve as a precursor for proline biosynthesis

A

glutamate dehydrogenase

83
Q

common intermediate of proline and argnine

A

glutamate semialdehyde

84
Q

end peoduct of his metab

A

glutamate

85
Q

decarboxylation of histidine in the intest by nacte produces

A

histamine

86
Q

the 1-carbon THF intermediate of histi metab

A

N5 formimino THF

87
Q

intermediate in his metab tested in the skin

A

urocanate

88
Q

urinary metabolite in the his metab

A

N-formiminoglutamate

89
Q

what accumulates in histidinemia

A

imidazolepyruvate
imidazoleacetate
imidazolelactate

90
Q

histidinemia produces what result in the ferric chloride test?

A

false pos

91
Q

why false psoitive ang histidinemia sa ferric chloride

A

imidazolepyruvate

92
Q

2 fates of phe metab

A

incorporation into polypeps

tyrosine

93
Q

coenzyme required in the formation of tyrosine from phe

A

terrahydrobiopterin

94
Q

the enzyme requiring tetrehydrobiopterin

A

phenylalanine hydroxylase

95
Q

aside from the two main fates of phe in its metab, phe is also transaminated to form ____-, ______ or _____

A

phenyllactic acid
phenylacetic acid
glutamine

96
Q

main pathway of tyosine degradation involce conversion to __ and ____

A

fumarate

acetoacetate

97
Q

deficiency of phe hydroxylase

A

PKU

98
Q

dse leads to painful corneal eruptions and photophobia

A

hypertyrosinemia

99
Q

first genetic dse recognized

A

alkaptonuria

100
Q

alkaptonuria is the defiency of

A

homogentisic acid oxidase

101
Q

itsura ng urine sa alkaptonuria

A

black/darkening

102
Q

characterisitic of type 1 tyrosinemia

A

cabbage-odor urine

103
Q

deficiency of tyrosine transaminase

A

Richner-Hanhart syndrome (type2 tyrosinemia)

104
Q

dse when tyrosine is not converted to thyroxine

A

cretinism

105
Q

what is deificiecnt in type 1 tyrosinemia

A

fumarylacetoacetate hydrolase

106
Q

which of the products of tyr metab is keto and which is gluco

A

fumarate - gluco

acetoacetate - keto

107
Q

tyrpsine may serve as the precurosor of

A

catecholamines

108
Q

universal emthyl donor

A

S-adenosylmethionine

109
Q

SAM when devoid of methyl becomes

A

S-adenosylhomocysteine

110
Q

path ng tyrosine to epineph

A

L-tyr > dopa > dopamine > norepi > epineph

111
Q

what stimulates cystathione synthase

A

SAM

112
Q

block of the rxn that converts homocysteine back to methione is what dse

A

type2 homocysteinuria

113
Q

what conezyme does methionine synthase need

A

methylcobalamine

114
Q

what enzyme convert homocysteine back to meth

A

methionine synthase

115
Q

block of the rxn that uses cystathione synthase is what dse

A

type 1 homocysteinuria

116
Q

cystathione = _____ + ______

A

homocysteine + serine

117
Q

the intermediate comonly shared by meth and threo

A

a-ketobutyrate

118
Q

what are the BCAAs

A

val
ile
leu

119
Q

what is common in the metab of the the BCAAs

A

first 3 rxns

BCAA aminotansferase
BC a-ketoacid dehydrogenase
the next dehydrogenase

120
Q

block in the 2nd step of BCAA metab causes

A

increases ketoacids and BCAAs

121
Q

dse with burnt sugar odor, inc. BCAA, deficient BC a-ketoacid dehydrogenase

A

MSUD

122
Q

which of the BCAA is purely keto, purely gluco and both

A

leu - keto
val - gluco
ile - both

123
Q

intermediate from threonine before it ultimately becomes propionyl COA > succinyl CoA

A

a-ketobutyrate

124
Q

dse malabsorption of trp, acuumulation of indicansa

A

blue diaper syndrome

125
Q

amino acids cataolized into acetyl CoA and acetoacetate

A

trp
lys
leu

126
Q

amino acids catabolized via succinylCoA

A

met

BCAAs

127
Q

an intermediate in trp metab that antagonizes the effects of excfretory amino acids in teh brain

A

kynurenic acid

128
Q

epsilon amino group of lysine is transferred to the a-keto carbon of a-KG forming the metabolite

A

saccharopine

129
Q

the transmination of lysine is different from the others in a way that it

A

does not use PP

130
Q

saccharopine is acted upon by __ to form __ and ___

A

aminoadipic semialdehyde synthase
glutamate
a-aminoadipic semialdehyde

131
Q

ultimate end-product of lsine catab

A

acetoacetyl CoA

132
Q

lysine is the precurosr of ___ required for the transport o FA into mitochondria for oxidation

A

carnitine

133
Q

lysine + a-KG > (___) > saccharopine

A

lysine 2-oxoglutarate reductase-saccharopine dehydrogenase

134
Q

intermediate commonly shared by trp and lys

A

a-ketoadipate

135
Q

absence of the first enzyme in lys metab, ______, is the cause fo the dse _____-

A

lysine 2-oxoglutarate reductase-saccharopine dehydrogenase

hyperlysinemia

136
Q

a dse of transport failure of dibasic AAs

A

familial lysinuria protein something

137
Q

wat is decreased in familial lysinuria protein something

A

lys
arg
orni

138
Q

path ng leu to acetylCoA and acetoacetate

A

leu > HMGCoA > acetylCoA + acetoacetate

139
Q

end products of ile metab

A

succinylCoA

acetylCoA

140
Q

AAs canno be synthesized sufficiently by the body

A

essential

141
Q

the 3 essential AAs not related to lack of synthesis

A

arg
met
tyr

142
Q

essential AAs

A

PVT TIM HALL

proline
valine
trp

thr
ile
met

his
arg
lys
leu

143
Q

synthesis of nonessentials:

pyruvate > (ALT) > _____

A

alanine

144
Q

metab of nonessentials:

OXAA > (aminoatransferase) > ___

A

aspartate

145
Q

synth of noness AA

alanine + a-KG > (_____) > ____

A

glutamate pyruvate aminotransferase

glutamate

146
Q

synth of noness AA

glutamate > (____) > _____

A

glutamine synthase

glutamine

147
Q

synth of noness AA

aspartate > (___) > _____

A

asparagine synthase

asparagine

148
Q

synth of noness AA

serine > (____) > _____

A

serine hudroxymethyl transferase

glycine

149
Q

synth of noness AA

glycine aside from choline and serine can also be sunthesized from

A

gloxyli acid

150
Q

synth of noness AA

possible sources/orig of glycine

A

serine
glyoxylic acid
chiline

151
Q

synth of noness AA

glycine > (______) > ____ requiring the conezyme _____

A

hydroxymethyl transferase
serine

tetrahydrofolate

152
Q

synth of noness AA

aside from glucine, serine may also be sunthesized from

A

3 phosphogluceric acid > phosphohusroxyphruvate > phosphoserine > serine

153
Q

synth of noness AA

glutamate > ____ > delta1-pyrrolidine 5-carboxylic avid > ______

A

glutamate semialdehyde

proline

154
Q

carbon skeleton of custeine is provided by

A

serine

155
Q

sulfhydryl group in cysteine is provided by

A

homocyteine

156
Q

deficiency or absence in meth synthase what daw

A

type 2 3 4 cystinuria

157
Q

type 1 homocysteinurianis deficiency of

A

cystathione synthase

158
Q

symptoms or signs in type1 gomocysteinuria

A

osteoporosis

mental retardation

159
Q

another end peoduct from methionine during synth of cysteine

A

a-ketobutyrate

160
Q

synth of noness AA

phe > (____) > ____

A

phe hudroxylase

tyr

161
Q

converts dihydrobiopterin back to terrahydrobiopterin

A

dihydrobiopterin reductase

162
Q

absence of phe hudroxylase

A

classic Pku

163
Q

synth of noness AA

____ > (______) > arginine

A

argininosuccinate

argininosuccinase

164
Q

continuous highexcretio of endogenous of oxalate and caox deposits in the kidney and urinary tract

A

primary hyperoxaluria

165
Q

primary huperoxaluria is die to deficiency of

A

gloxylix acid oxidizig enzyme

glycine transaminase

166
Q

defective syntheisjs of melanin

A

albinism

167
Q

defiency in L methylmalonyl CoA mutase

A

methylmalonic aciduria

168
Q

biosynthesis of catecholamines explain para mapatunayang nandaya ang liberal party nung election

tyr > (___) > dopa > (__) > dopamine > (___) > norepi > (___) > epineph

A

turosine hydroxylase
dopa carboxylase
dopamine B oxidSe
PNMT phenylethanolamine A methyl transferase

169
Q

irinary metab in the catab of catechol and is the end product

A

vanillyl mandelate

170
Q

urinary metabolite in the synthesis of melatonin

A

5-hudroxyindole 3avetate (5-HIAA)

171
Q

sertoonin aka

A

5-huroxytryptamine

172
Q

serotonin undergoing avetylation then methylation produces

A

melatonin

173
Q

iodination of tyr produces

A

monoiodotyrosine

diiodotyrosine

174
Q

coupled mono and diiodotyrosines are released as

A

thyroxine T4

ttriiodothyronine T3

175
Q

decarboxylation of hisitidine

his > (____) > _____

A

hisitidine decarboxylase

histamine

176
Q

3 shit used in the synth of creatinine

A

argi
gly
SAM

177
Q

in creatinine synth

arg > (___) > ____

A

glycine cyclase transamidase

guanidoacetate

178
Q

guanidoacetate when methylated becomes

A

creatine

179
Q

creatine phosphate hs high every storage where

A

muscles

180
Q

____ gives rise to a different melanin intermed of barying color and the black pigment ____

A

dopaquinone

eumelanin

181
Q

derived from trp via anthranillic acid pathway

A

niacin

182
Q

niacin is derived from trp via

A

anthranillic acid apthway

183
Q

decarboxylation of glutamic acid forms

A

GABA

184
Q

transamination of GABA produces

A

succnic acid semialdehyde

185
Q

symth of polyamines

orni > (___) > _____ > (___)> _____ > ____

A 
ODC
pitrescine
spermidine synthase
spermidine
spermi e
186
Q

3 AAs used to synth gluta

A

glutamic acid
glycine
cysteine

187
Q

ano ug path ng paggawa ng gluta

A

glutamate > (y-glutamate cysteine synthetase) > y-glutamylcysteine > (gluta synthase) > gluta

188
Q

high levels of carbosine is found where

A

excitatroy tissues

189
Q

decreased urinary 3methylhistidine what dae

A

wilson’s

190
Q

devreased in wilson’s

A

3methylhisitidne

191
Q

funcction of carbosine

A

avtivate myosin ATPase

^ iron uptake

192
Q

structure of carnosine is ___ and answrine is _____

A

b-alamylhistidine

b-alamylmethylhistidine

193
Q

sunthesized from arg ny an enzyme which uses NADPH as conezyme

A

NO

194
Q

enzyem used to make NO

A

NOS

195
Q

the hormone for regulation that is the only anabolic

A

insilin

196
Q

purpose of T4 in hormone regulation

A

sensitization

197
Q

counteregulatory hormones of insilin

A 
glucagon
epine
cortisol
somatos
GH

MedBiochem (16 decks)

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