quiz 2.3 AA metab Flashcards by Zeth Renae Raquedan

average daily protein turnover

250g/day

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cases na may positive nitrogen balance

growing child
pregnant
convalescing pipz
refeeding after starvation

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AA that does not undergo transamination

lysine

threonine

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coenzyme in transamination

pyridoxal phosphate

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product after trnsamination

alpha ketoacid

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

glutarate

alpha-ketoglutarate

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

glutarate dehydrogenase

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oxidase primarily involved in the deamination of lysine

L-amino acid oxidase

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asparaginase converts asparagine to

ammonia and aspartate

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

glutaminase

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direct deamination of histidine by ___ and ____ produces ___

histidase/histidine ammonia lyase

urocanic acid

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dehydrative deamination of serine and threonine prodcues ____ and ___ respectively

pyruvate

alpha-ketobutyrate

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3 ways ammonia is disposed

alanine cycle
glutamine cycle
urea cycle

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urea cycle aka

Krebs-Henseleit cycle

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where does urea cycle occur

liver

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saan galing ung two nitrogens sa urea

from NH3 thru glutamate dehydrogenase

from aspartate by transamination

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rate limiting enyzme fi the urea cycle

carbamoyl phosphate synthase 1

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formation of carbamoyl phosphate by CPS1 is driven by

cleavage of 2 mols of ATP

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ornithine plus carbamoyl phosphate by ornithine transcabamoylase forms

citrulline

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

argininosuccinate

argininosuccinate synthetase

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arginine is cleaved by arginase to form

ornithine and urea

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argininosuccinate is cleaved by ___ to form

argininosuccinate lyase

arginine and fumarate

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

urea - excreted trhu urine

ornithine - enters mitcochondria

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what component in the urea cycle enters the TCA

fumarate

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

starvation and high protein diet

the allosteric activator of CPS1

N-acetylglutamate

positive allosteric activator of NAG synthetase

arginine

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

glucogenic

amino acids which can be converted into fat

ketgenic

what are the purely ketogenic AAs

leu | lys

what AAs are both keto and glucogenic

``` phe ile trp tyr thr ```

amino acids catabolized via pyruvate

``` alanine serine glycine cysteine & cystine threonine tryptophan hydroxyproline ```

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

pyruvate alanine aminotransferase OXAA

alanine: keto or gluco?

glucogenic

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

dehydrative deamination serine dehydrogenase pyruvate

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

glycine | CO2 and ammonia

catalyzes the 2nd step in the metab of serine

serine hydroxymethyltransferase

metab of glycine

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

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

glyoxylate

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

glycine cleavage complex | N5,N10-methylene THF

universal sulfur donor

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

simplest path of cystine

catalyzed by liver desulfurase to produce pyruvate

cysteine > (_______) > cysteinesulfinate

cysteine dioxygenase

cysteine > (cystein dioxygenase) > ______________

ccysteinesulfinate

cysteinesulfinate undergoing transaminattion produces what b

beta-sulfinylpyruvate

cysteinesulfinate undergoing decarboxylation and oxidation produces

taurine

beta-sulfinylpryuvate undergoing desulfuration yields

bisulfite

bisulfite acted upon by sulfite oxidase yields

sulfate and H2O2

the sulfate yielded from bisulfate will be used to

make PAPS

cystine > (__________) > cysteine

NADH linked-cystine reductase

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

2 moles

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

cystathionase | thiocysteine & pyruvate

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

beta-mercaptopyruvate/3-mercaptopyruvate | thiosulfate & pyruvate

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

rhodanase

pathway ng propionylCoA to succinylCoA

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

threonine > (serine/threonine dehydrogenase) > _____

a-ketobutyrate

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

propionyl CoA

threonine > (threonine dehydrogenase) > ______

a-amino-b-ketobutyrate

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

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

threonine acted upon by threonine aldolase produces __ and __-

acetylCoA and pyruvate

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

iron porphyrin oxygenase

first key branch point of trp metab

kynurenine

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

kynurenate anthranilate 3-hydroxykynurenine

which of the products from kynurenine is an anticonvulsant

kynurenate

a urinary metabolite from trp seen during its metab

xanthurenate

xanthurenate came directly from or is a product of what

transaminated 3-hydroxykynurenine

2nd key branch point in trp metab

2-amino-3-carboxymuconic semialdehyde

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

nicotinate mononucleotide

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

picolinate nicotinate mononucleotide glutarylCoA > acetylCoA

in benign hyperhydroxyprolinemia, alin ung wala or blcoked

hydroxyproline dehydrogenase

in type 2 hyperprolinemia, alin ung wala or blocked

2nd dehydrogenase rxn

ulitmate products of hydroxyproline metab

glyoxylate | pyruvate

amino acids catabolized by OXAA

aspartate | asparagine

aspartate + oxoglutarate > _______ + glutamate

OXAA

amino acids catabolized via a-KG

``` glutamine glutamate histidine proline arginine ```

convert glutamine to glutamate and NH3

glutaminase

where is glutaminase prominently found

kidney tuules

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

a-KG

glutamate > (_______) > a-KG

glutamate drhydrogenase

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

NH4

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

glutamate semialdehyde

can serve as a precursor for proline biosynthesis

glutamate dehydrogenase

common intermediate of proline and argnine

glutamate semialdehyde

end peoduct of his metab

glutamate

decarboxylation of histidine in the intest by nacte produces

histamine

the 1-carbon THF intermediate of histi metab

N5 formimino THF

intermediate in his metab tested in the skin

urocanate

urinary metabolite in the his metab

N-formiminoglutamate

what accumulates in histidinemia

imidazolepyruvate imidazoleacetate imidazolelactate

histidinemia produces what result in the ferric chloride test?

false pos

why false psoitive ang histidinemia sa ferric chloride

imidazolepyruvate

2 fates of phe metab

incorporation into polypeps | tyrosine

coenzyme required in the formation of tyrosine from phe

terrahydrobiopterin

the enzyme requiring tetrehydrobiopterin

phenylalanine hydroxylase

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

phenyllactic acid phenylacetic acid glutamine

main pathway of tyosine degradation involce conversion to __ and ____

fumarate | acetoacetate

deficiency of phe hydroxylase

PKU

dse leads to painful corneal eruptions and photophobia

hypertyrosinemia

first genetic dse recognized

alkaptonuria

alkaptonuria is the defiency of

homogentisic acid oxidase

itsura ng urine sa alkaptonuria

black/darkening

characterisitic of type 1 tyrosinemia

cabbage-odor urine

deficiency of tyrosine transaminase

Richner-Hanhart syndrome (type2 tyrosinemia)

dse when tyrosine is not converted to thyroxine

cretinism

what is deificiecnt in type 1 tyrosinemia

fumarylacetoacetate hydrolase

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

fumarate - gluco | acetoacetate - keto

tyrpsine may serve as the precurosor of

catecholamines

universal emthyl donor

S-adenosylmethionine

SAM when devoid of methyl becomes

S-adenosylhomocysteine

path ng tyrosine to epineph

L-tyr > dopa > dopamine > norepi > epineph

what stimulates cystathione synthase

SAM

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

type2 homocysteinuria

what conezyme does methionine synthase need

methylcobalamine

what enzyme convert homocysteine back to meth

methionine synthase

block of the rxn that uses cystathione synthase is what dse

type 1 homocysteinuria

cystathione = _____ + ______

homocysteine + serine

the intermediate comonly shared by meth and threo

a-ketobutyrate

what are the BCAAs

val ile leu

what is common in the metab of the the BCAAs

first 3 rxns BCAA aminotansferase BC a-ketoacid dehydrogenase the next dehydrogenase

block in the 2nd step of BCAA metab causes

increases ketoacids and BCAAs

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

MSUD

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

leu - keto val - gluco ile - both

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

a-ketobutyrate

dse malabsorption of trp, acuumulation of indicansa

blue diaper syndrome

amino acids cataolized into acetyl CoA and acetoacetate

trp lys leu

amino acids catabolized via succinylCoA

met | BCAAs

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

kynurenic acid

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

saccharopine

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

does not use PP

saccharopine is acted upon by __ to form __ and ___

aminoadipic semialdehyde synthase glutamate a-aminoadipic semialdehyde

ultimate end-product of lsine catab

acetoacetyl CoA

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

carnitine

lysine + a-KG > (___) > saccharopine

lysine 2-oxoglutarate reductase-saccharopine dehydrogenase

intermediate commonly shared by trp and lys

a-ketoadipate

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

lysine 2-oxoglutarate reductase-saccharopine dehydrogenase | hyperlysinemia

a dse of transport failure of dibasic AAs

familial lysinuria protein something

wat is decreased in familial lysinuria protein something

lys arg orni

path ng leu to acetylCoA and acetoacetate

leu > HMGCoA > acetylCoA + acetoacetate

end products of ile metab

succinylCoA | acetylCoA

AAs canno be synthesized sufficiently by the body

essential

the 3 essential AAs not related to lack of synthesis

arg met tyr

essential AAs

PVT TIM HALL proline valine trp thr ile met his arg lys leu

synthesis of nonessentials: pyruvate > (ALT) > _____

alanine

metab of nonessentials: OXAA > (aminoatransferase) > ___

aspartate

synth of noness AA alanine + a-KG > (_____) > ____

glutamate pyruvate aminotransferase | glutamate

synth of noness AA glutamate > (____) > _____

glutamine synthase | glutamine

synth of noness AA aspartate > (___) > _____

asparagine synthase | asparagine

synth of noness AA serine > (____) > _____

serine hudroxymethyl transferase | glycine

synth of noness AA glycine aside from choline and serine can also be sunthesized from

gloxyli acid

synth of noness AA possible sources/orig of glycine

serine glyoxylic acid chiline

synth of noness AA glycine > (______) > ____ requiring the conezyme _____

hydroxymethyl transferase serine tetrahydrofolate

synth of noness AA aside from glucine, serine may also be sunthesized from

3 phosphogluceric acid > phosphohusroxyphruvate > phosphoserine > serine

synth of noness AA glutamate > ____ > delta1-pyrrolidine 5-carboxylic avid > ______

glutamate semialdehyde | proline

carbon skeleton of custeine is provided by

serine

sulfhydryl group in cysteine is provided by

homocyteine

deficiency or absence in meth synthase what daw

type 2 3 4 cystinuria

type 1 homocysteinurianis deficiency of

cystathione synthase

symptoms or signs in type1 gomocysteinuria

osteoporosis | mental retardation

another end peoduct from methionine during synth of cysteine

a-ketobutyrate

synth of noness AA phe > (____) > ____

phe hudroxylase | tyr

converts dihydrobiopterin back to terrahydrobiopterin

dihydrobiopterin reductase

absence of phe hudroxylase

classic Pku

synth of noness AA ____ > (______) > arginine

argininosuccinate | argininosuccinase

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

primary hyperoxaluria

primary huperoxaluria is die to deficiency of

gloxylix acid oxidizig enzyme | glycine transaminase

defective syntheisjs of melanin

albinism

defiency in L methylmalonyl CoA mutase

methylmalonic aciduria

biosynthesis of catecholamines explain para mapatunayang nandaya ang liberal party nung election tyr > (___) > dopa > (__) > dopamine > (___) > norepi > (___) > epineph

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

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

vanillyl mandelate

urinary metabolite in the synthesis of melatonin

5-hudroxyindole 3avetate (5-HIAA)

sertoonin aka

5-huroxytryptamine

serotonin undergoing avetylation then methylation produces

melatonin

iodination of tyr produces

monoiodotyrosine | diiodotyrosine

coupled mono and diiodotyrosines are released as

thyroxine T4 | ttriiodothyronine T3

decarboxylation of hisitidine his > (____) > _____

hisitidine decarboxylase | histamine

3 shit used in the synth of creatinine

argi gly SAM

in creatinine synth arg > (___) > ____

glycine cyclase transamidase | guanidoacetate

guanidoacetate when methylated becomes

creatine

creatine phosphate hs high every storage where

muscles

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

dopaquinone | eumelanin

derived from trp via anthranillic acid pathway

niacin

niacin is derived from trp via

anthranillic acid apthway

decarboxylation of glutamic acid forms

GABA

transamination of GABA produces

succnic acid semialdehyde

symth of polyamines orni > (___) > _____ > (___)> _____ > ____

``` ODC pitrescine spermidine synthase spermidine spermi e ```

3 AAs used to synth gluta

glutamic acid glycine cysteine

ano ug path ng paggawa ng gluta

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

high levels of carbosine is found where

excitatroy tissues

decreased urinary 3methylhistidine what dae

wilson's

devreased in wilson's

3methylhisitidne

funcction of carbosine

avtivate myosin ATPase | ^ iron uptake

structure of carnosine is ___ and answrine is _____

b-alamylhistidine | b-alamylmethylhistidine

sunthesized from arg ny an enzyme which uses NADPH as conezyme

enzyem used to make NO

NOS

the hormone for regulation that is the only anabolic

insilin

purpose of T4 in hormone regulation

sensitization

counteregulatory hormones of insilin

``` glucagon epine cortisol somatos GH ```