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CPR Exam 2 > Nitrogen Metabolism - Kinde > Flashcards

Nitrogen Metabolism - Kinde Flashcards

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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
Sx :and Tx: cystinuria
renal colic, abd pain from kidney stones of cystine crystals | Tx: alkaline diet (alkaline urine dissolves the crystals)
26
PKU has defective what in primary secondary
1. Primary : phenylalanine Hydroxylase ( PHE --> TYR) | 2. Secondary : dihydrobiopterin reductase (NADPH--> NADP+, cofactor) * not as severe
27
what does Tyr make
GLU and Melanin
28
another way of saying dihydrobiopterin reductase
BH4THB
29
what happens in PKU
Phe --> phenylpyruvate --> phenyllacetate + phenylacetate which are both toxic *since PHE cant become TYR*
30
PKU Sx:
musty odor urine ----I NTs to brain severe brain dysfunction
31
PKU Tx:
1. X Phe in diet 2. supplement Tyr in diet 3. supplement tetrahydrobiopterin
32
which disease do you always screen for in newborns
PKU (guthrie test)
33
Tetrahydrobiopterin + dihydrobiopterin is essential what | which is active
hydroxylation of aromatic AAs by dihydrobiopterin reductase also helps Trp make NE, E, S * *** BH4 = THB is active * *** qBH2 = DHB s inactive
34
Tyrosinemias all do what
have elevated Try (restricts TYR and PHE)
35
Transient Tyrosinemia
newborns dont have developed enzymes (4-HPPD) yet to breakdown Tyr = HIGH TYR
36
Tyrosinemia 1
cabbage like smell X fumarylacetatoacetate hydrolase = build up of succinylacetone = toxic for liver and kidney = no heme + TCA + can need liver transplant
37
Tyrosinemia Tx
Nitisinone = 4-HPPD inhibitor *prevents buildup of succinylacetone so NO LIVER PROBLEMS = you still have excessive Tyr and Phe
38
Tyrosinemia 2
X tyrosine aminotransferase = causes lesions on skin and cornea (tyr deposition) mental problems eye problems
39
Tyrosinemia 3
X 4 -HPPD : neurological problems (very rare) | buildup of tyrosine
40
Alkaptonuria
X homogenisate oxidase = BLACK URINE, Bone, and sclera, arthritis = accumulated homogentisic acid
41
Ammonia Toxicity happens due to
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
Ammonia Toxicity Sx
``` pH imbalance, astrocyte swelling in brain, cerebral edema, intracrainial HTN LOW GLU : 1. X GABA 2. low atp for brain * from high glutamine ```
43
Gout Primary secondary
high uric acid from PURINE metabolism deposited in joints Primary = high uric acid production secondary = cant excrete uric acid enough
44
what can increase chance of getting gout
beans, alcohol, meat, seafood, spinach, lentils | DM
45
gout Tx:
1. limit purine 2. Allopurinol ----I xanthine oxidase and makes purines soluble 3. colchicine = decreases uric acid movement to joints
46
Acidemia
--> URAT1 transporter (reabsorbes uric acid)
47
normal excretion if uric acid
60%-70%
48
normal reabsorption of urate
90%
49
NAG synthase deficiency causes what
glutamate + Acetyl CoA --X--> NAG HIGH Nitrogen accumulation (NH4) = toxic to CNS and, V, coma, lethargy, no eating
50
what is the role of NAG
the cofactor to make NH4 ----> Carbomyl Phosphate
51
Hyperammonemia is what
one of the 6 enzymes of the urea cycle is defected or any of 2 transporters (Asp/Glu + ornithine)
52
Hyperammonemia by defected ornithine transcarboxylase (OTC)
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
CPS2
first step of making pyrimidine de novo stimulated by PRPP inhibited by UTP
54
UMP synthase deficiency causes what
accumulation of orotic acid = orotic aciduria (since it can not continue on with pyrimidine de novo, so it accumulates)
55
OTC causes hyperammonemia because of what
the accumulation of CP leading to accumulated NH4+
56
OTC deficiency Tx: and also type of condition
X- linked | Protein in diet
57
UMP synthase deficiency Sx
orotic aciduria | NO low BUN or hyperammonemia
58
CSP 1 is found where and activated by what
mitochondria for urea cycle | NAG
59
CSP2 is found where and activated by what
Cytosol of Pyrimidine synthesis | PRPP
60
what leads to increased unconjugated bilirubin in infants
UDP-glucuronyl transferase deficiency
61
what can the UDP - glucuronyl transferase deficiency lead to besides jaundice
if mother takes Abs the baby cant metabolize drug and cant excrete unconjugated drug = CHLORAMPHENICAL TOXICITY (Gray Baby Syndrome) - circulatory collapse (low contractlity)
62
what drug to use if mother has an infection if baby has a UDP - glucuronyl transferase deficiency
Cephalosporins
63
conjugated vs inconjugated bilirubin
``` conjugated = soluble (is pink mixed with Diazo *can by assayed directly) unconjugated = insoluble (only with methanol is it soluble) ```
64
what removed bilirubin before birth and after
before : placenta | after : liver when mature enough
65
what can help jaundice in infants
blue fluorescent light makes unconjugated to conjugated bilirubin
66
pathologic reasons infants have jaundice
``` medication infection Hypoxia Hepatitis G6D X trauma ```
67
Physiologic reasons infants have jaundice
Low UGT enzyme short RBC life high bilirubin made increased RBC mass circulation

CPR Exam 2 (22 decks)

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