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Urea cycle Flashcards

1
Q

Mousy odor

A

PKU

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2
Q

Increased branched chain amino acids/ketoacids

A

Maple syrup syndrome

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3
Q

Dark urine

A

Alkaptunoria

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4
Q

Defect in tyrosine

A

Albinism

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5
Q

Defective enzyme is Argininosuccinate synthetase

A

Citrullinemia

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6
Q

Tryptophan serves as a precursor of

A

Serotonin

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7
Q

Derived by Tyrosine

A

Catecholamines
Thyroid hormones
Melanin

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8
Q

Glutamate is a precursor of

A

GABA

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9
Q

Arginine is the precursor of

A

Nitric Oxide

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10
Q

AA that is essential for growth

A

Arginine

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11
Q

In human, lysine is broken down into

A

a-ketoglutarate

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12
Q

PKU is increased level of

A

Valine and Leucine

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13
Q

An amino group donor

A

Glutamate

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14
Q

Storage site for ammonium

A

Glutamine

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15
Q

Provide first N atom

A

Ammonium ion

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16
Q

Provide second N atom

A

Aspartate

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17
Q

Urea cycle rate limiting enzyme

A

Carbamoyl phosphate

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18
Q

An allosteric activator

A

N-acetylglutamate

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19
Q

A regenerating substrate

A

Ornithin

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20
Q

First rate limiting enzymes in the Porphyrin pathway

A

ALA synthase

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21
Q

Defective enzyme in PKU

A

Phenylalanine hydroxylase

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22
Q

Lethargy; convulsions ; early death

A

Carbamoyl phosphate synthase l deficiency

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23
Q

Faulty bone development. Elevated homocystein. Treatment is diet low in methionine

A

Homocystinuria

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24
Q

Vomiting; convulsion; mental retardation; early death

A

Methylmalonic acidemia

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25
Role of folate in AA metabolism
Carbon donor
26
Elevated levels of leucine, valine, and branched chain keto acids
Maple Syrup Urine Disease
27
Due to poor activity or poor suck of a newborn baby. Signs of severe dehydration and burnt sugar odor urine
Maple Syrup Urine disease
28
Homogentisate Oxidase deficiency
Alkaptonuria
29
Black stained diapers upon exposure to air
Alkaptonuria
30
Developmental delay and loss motor control and frequent vomiting. Mousy odor and eczematoid rash
PKU
31
A major end product of nitrogen catabolism
Urea
32
1mol of Urea requires
3 mol of ATP and 1mol of Ammonium ion and a-amino N of aspartate
33
An enzyme activator
N-acetylglutamate
34
Carriers of atoms
Urea
35
Carrier molecule in mitochondria
Ornithine
36
It reacts with aspartate in cytosol
Citrulline
37
It reacts with arginine in cytosol
Argininosuccinate
38
First substrate in Urea cycle
Carbamoyl phosphate
39
End product of purine and amino acid catabolism
Urate and Ammonium ions
40
Urea biosynthesis occurs in
Liver cells
41
These AA’s generated in tissues are transported to the liver chiefly as
Alanine and glutamine
42
The first two reactions leading ti the synthesis of urea occur in
Mitochondria
43
Remaining reactions in the synthesis of urea occur in
Cytosol
44
What reaction is this: Carbamoyl P Synthase l initiates Urea biosynthesis
Reaction 1
45
What reaction is this: Carbamoyl Phosphate + Ornithine forms Citrulline
Reaction ll
46
What reaction is this: Citrulline + Aspartate forms Argininosuccinate
Reaction lll
47
What reaction is this: Cleavage of Argininosuccinate forms Arginine and Fumarate
Reaction Type lV
48
What reaction is this: Cleavage of Arginine Release Urea and Re-forms Ornithine
Reaction Type V
49
Ammonia is generated in
Intestine
50
Where does ammonia metabolized
Liver
51
How skeletal muscle decrease blood ammonia
Metabolizing ammonia into glutamine
52
Role of kidney in determining blood ammonia by
Excretion of urea and Ammonium ion
53
Urea cycle defects where infants became lethargic
Type 1 Hyperammonemia
54
Urea cycle defects having a severe hyperammonemia, associated with coma, acidosis and diarrhea
N-acetylglutamate synthase deficiency
55
Urea cycle defects that is most commonly occuring UCD, only X-link UCD
Type 2 Hyperammonemia
56
Urea cycle defects that is rare UCD
Hyperargininemia
57
Urea cycle defects that is episodic hyperammonemia, coma
Classic Citrunellinemia
58
Urea cycle defects that is episodic symptoms similar to class citrullinemia
Argininosuccinic aciduria
59
Group of disorders due to abnormalities in the pathway of biosynthesis of heme. This can be genetic or acquired
Porphyrias
60
Daily formation if bilirubin in adults is
250-350 mg
61
Increased bilirubin cause
Jaundice
62
Important causes of conjugated hyperbilirubinemia
Hemolytic anemias
63
A rare autosomal recessive disorder or known as congenital non hemolytic jaundice. Severe congenital jaundice and fatal within the first 15mons. of life
Crigler-Najjar syndrome, Type 1
64
Mutations in the gene encoding bilirubin-UGT. Benign course
Crigler-Najjar syndrome, Type 2
65
Mutations in the gene encoding bilirubin UGT and 30% of the enzymes activity is preserved
Gilbert syndrome
66
Toxin induced liver dysfunction caused by chloroform etc.
Toxic hyperbilirubenemia
67
Commonly results from blockage of the hepatic. Most often due to gallstone or ti cancer of the head of the pancreas. Cholestatic jaundice
Obstructive biliary tree
68
A benign autosomal recessive disorder consist of conjugated hyperbilirubinemia in childhood or during adult life
Dubin-Johnson syndrome
69
A rare benign condition characterized by chronic conjugated hyperbilirubinemia and normal liver histology. Abnormality in hepatic storage
Rotor Syndrome
70
Bilirubin in urine without urobilinogen caused
Obstructive jaindice
71
Urobilinogen without bilirubin caused
Hemolytic jaundice
72
Biosynthesis of the heme ring occurs in
Mitochondria and cytosol
73
Elevation if the level of bilirubin in the blood caused
Jaundice
74
A breakdown product of bilirubin
Urobilinogen

Biochemistry (7 decks)

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