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CPR Exam 2 > Nitrogen Metabolism - Kinde > Flashcards

Nitrogen Metabolism - Kinde Flashcards

1
Q

how to remove aa

A

becomes NH3 + carbon backbone partand oxidative deamination to wrea is the final step to N removal

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2
Q

carbon backbone part can be what

A
  1. Ketogenic : Acetyl CoA, Acetoacetate

2. Glucogenic : Pyruvate, TCA intermediates

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3
Q

the N giver and donor is what

A

Glutamate

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4
Q

glutamate + oxaloacetate —->

A

a-ketoglutoarate + Aspartate

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5
Q

glutamate + pyruvate —->

A

a-ketoglutoarate + Alanine

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6
Q

RLS in urea cycle

A

NH+4 —-> Carbamoyl Phosphate

* by carbamyl phosphate synthetase 1*

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7
Q

what happens after aa become glutamine

A

glu –> NH4 + Glutamine (glutamate dehydrogenase)
NH4 —-> Carbamoyl Phosphate
Carbamoyl Phosphate —-> UREA CYLCE

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8
Q

UREA CYCLE

A 
Carbamoyl Phosphate + ornithine 
Citrulline 
Argininosuccinate
Arginine
Urea + Ornithine
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9
Q

10 essential aa

A

PVT TIM HALL

Phe, Val, Trp, Thr, Iso, Met, His, Rg, Leu, Lys

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10
Q

where does glutamine come from and how is is broken down

A

from extrahepatic tissue and brain
removes N from brain
and becomes glu by glutaminase

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11
Q

co factor for transaminases

A

PLP Pyridoxal phosphate

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12
Q

special thing about diseases caused by transporters that are defective

A

causes both X absorption in GI and X reabsorption in PCT of kidneys

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13
Q

2 transporter deficiency defects

A
  1. Hartnup = no neutral AAs (TRP)

2. Cystinuria = no dibasic AAs (Cystine dimer buildup)

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14
Q

what transporter is on the PCT for reabsorption on AAs

A

SLC (solute carrier) AAs and glucose enter with NA+

* on apical side** (facing environment)

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15
Q

which SLC is defected in Hartnup

A

SLC6A19 gene (TRYPTOPHAN transporter and other neutral AAs)

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16
Q

which SLC is defected in Cystinuria

A

SLC7A9 + SLC3A1 genes (dibasic AAs transporter)

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17
Q

which AAs are effected by Cystinuria

A

Cystine, Lys, Arg, Ornithine

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18
Q

what happens in WHAT to cause amminoaciduria

A

in Hartnup disease , inability to absorb or reabsorb

Ala, Asn, Ser, Thr, Tyr, Trp, Val, Leu, Ile, Phe, Gln

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19
Q

what happens if body cant have Trp (precursor for what)

A

it is the precursor for:

  1. Serotonin —-> Metatonin (MOOD, like anxiety, depression)
  2. Niacin —-> NAD + NADP (nystagmus, ataxia, photosensitivity, pellergra, hyperpigmentation)
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20
Q

how to Tx harnup disease

A
  1. niacin repletion by NICOTINIC ACID
  2. high protein diet, to get some AAs in
  3. VIT B3
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21
Q

what does Trp need besides the SLC6A19 to get reabsorbed or absorbed

A

VIT B6

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22
Q

how does Nicotinic acid treat hartnup disease

A

NA –> Nicotinamide (which spike Trp to make NAD de novo)

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23
Q

what does NAD help with in DNA

A

repair by binding to PARP enzymes

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24
Q

what happens in cystinuria

A

cystine is found as a dimer and cant get reabsorbed by dysfunctional transporter
COAL (dibasic AAs cant get into body) = Cys, Orn, Arg, Lys

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25
Q

Sx :and Tx: cystinuria

A

renal colic, abd pain from kidney stones of cystine crystals

Tx: alkaline diet (alkaline urine dissolves the crystals)

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26
Q

PKU has defective what in
primary
secondary

A
  1. Primary : phenylalanine Hydroxylase ( PHE –> TYR)

2. Secondary : dihydrobiopterin reductase (NADPH–> NADP+, cofactor) * not as severe

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27
Q

what does Tyr make

A

GLU and Melanin

28
Q

another way of saying dihydrobiopterin reductase

A

BH4THB

29
Q

what happens in PKU

A

Phe –> phenylpyruvate –> phenyllacetate + phenylacetate which are both toxic
since PHE cant become TYR

30
Q

PKU Sx:

A

musty odor urine
—-I NTs to brain
severe brain dysfunction

31
Q

PKU Tx:

A
  1. X Phe in diet
  2. supplement Tyr in diet
  3. supplement tetrahydrobiopterin
32
Q

which disease do you always screen for in newborns

A

PKU (guthrie test)

33
Q

Tetrahydrobiopterin + dihydrobiopterin is essential what

which is active

A

hydroxylation of aromatic AAs
by dihydrobiopterin reductase
also helps Trp make NE, E, S

  • *** BH4 = THB is active
  • *** qBH2 = DHB s inactive
34
Q

Tyrosinemias all do what

A

have elevated Try (restricts TYR and PHE)

35
Q

Transient Tyrosinemia

A

newborns dont have developed enzymes (4-HPPD) yet to breakdown Tyr = HIGH TYR

36
Q

Tyrosinemia 1

A

cabbage like smell
X fumarylacetatoacetate hydrolase
= build up of succinylacetone = toxic for liver and kidney
= no heme + TCA + can need liver transplant

37
Q

Tyrosinemia Tx

A

Nitisinone = 4-HPPD inhibitor
*prevents buildup of succinylacetone so NO LIVER PROBLEMS
= you still have excessive Tyr and Phe

38
Q

Tyrosinemia 2

A

X tyrosine aminotransferase
= causes lesions on skin and cornea (tyr deposition)
mental problems
eye problems

39
Q

Tyrosinemia 3

A

X 4 -HPPD : neurological problems (very rare)

buildup of tyrosine

40
Q

Alkaptonuria

A

X homogenisate oxidase
= BLACK URINE, Bone, and sclera, arthritis
= accumulated homogentisic acid

41
Q

Ammonia Toxicity happens due to

A

excessive NH4 (due to one of urea cycle enzymes not working)
= CNS and brain toxicity
** increased NH4
1.. causes a-keto +NH4 —-> glutamate (*opposite then favored)
2. HIGH glu + NH4 —-> glutamine
= low GLU

42
Q

Ammonia Toxicity Sx

A 
pH imbalance, astrocyte swelling in brain, cerebral edema, intracrainial HTN
LOW GLU : 
1. X GABA 
2. low atp for brain 
* from high glutamine
43
Q

Gout
Primary
secondary

A

high uric acid from PURINE metabolism deposited in joints
Primary = high uric acid production
secondary = cant excrete uric acid enough

44
Q

what can increase chance of getting gout

A

beans, alcohol, meat, seafood, spinach, lentils

DM

45
Q

gout Tx:

A
  1. limit purine
  2. Allopurinol —-I xanthine oxidase and makes purines soluble
  3. colchicine = decreases uric acid movement to joints
46
Q

Acidemia

A

–> URAT1 transporter (reabsorbes uric acid)

47
Q

normal excretion if uric acid

A

60%-70%

48
Q

normal reabsorption of urate

A

90%

49
Q

NAG synthase deficiency causes what

A

glutamate + Acetyl CoA –X–> NAG
HIGH Nitrogen accumulation (NH4)
= toxic to CNS and, V, coma, lethargy, no eating

50
Q

what is the role of NAG

A

the cofactor to make NH4 —-> Carbomyl Phosphate

51
Q

Hyperammonemia is what

A

one of the 6 enzymes of the urea cycle is defected or any of 2 transporters (Asp/Glu + ornithine)

52
Q

Hyperammonemia by defected ornithine transcarboxylase (OTC)

A
  1. Carbomyl Phosphate cant leave mitochondria as Citrulline so it builds up and spills out into cytosol
  2. Carbomyl Phosphate in the cytosol is metabolized by pyrimidine synthase to OROTIC ACID —-CPS2—-> CMP in cytosol
  3. Orotic Acid becomes UMP eventully
    = low BUN + hyperammonemia + orotic aciduria can happen
53
Q

CPS2

A

first step of making pyrimidine de novo
stimulated by PRPP
inhibited by UTP

54
Q

UMP synthase deficiency causes what

A

accumulation of orotic acid = orotic aciduria (since it can not continue on with pyrimidine de novo, so it accumulates)

55
Q

OTC causes hyperammonemia because of what

A

the accumulation of CP leading to accumulated NH4+

56
Q

OTC deficiency Tx: and also type of condition

A

X- linked

Protein in diet

57
Q

UMP synthase deficiency Sx

A

orotic aciduria

NO low BUN or hyperammonemia

58
Q

CSP 1 is found where and activated by what

A

mitochondria for urea cycle

NAG

59
Q

CSP2 is found where and activated by what

A

Cytosol of Pyrimidine synthesis

PRPP

60
Q

what leads to increased unconjugated bilirubin in infants

A

UDP-glucuronyl transferase deficiency

61
Q

what can the UDP - glucuronyl transferase deficiency lead to besides jaundice

A

if mother takes Abs the baby cant metabolize drug and cant excrete unconjugated drug
= CHLORAMPHENICAL TOXICITY (Gray Baby Syndrome)
- circulatory collapse (low contractlity)

62
Q

what drug to use if mother has an infection if baby has a UDP - glucuronyl transferase deficiency

A

Cephalosporins

63
Q

conjugated vs inconjugated bilirubin

A 
conjugated = soluble (is pink mixed with Diazo *can by assayed directly)
unconjugated = insoluble (only with methanol is it soluble)
64
Q

what removed bilirubin before birth and after

A

before : placenta

after : liver when mature enough

65
Q

what can help jaundice in infants

A

blue fluorescent light makes unconjugated to conjugated bilirubin

66
Q

pathologic reasons infants have jaundice

A 
medication
infection
Hypoxia
Hepatitis
G6D X
trauma
67
Q

Physiologic reasons infants have jaundice

A

Low UGT enzyme
short RBC life
high bilirubin made
increased RBC mass circulation

CPR Exam 2 (22 decks)

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