protein metabolism 2

Question 1

⮞ Melanin
⮞ Catecholamines
⮞ T3
⮞ T4
..are major derivatives of?

Answer 1

Phe & Tyr

Question 2

This inborn error manifests:
⮞ mental retardation
⮞ mousy odor of urine
⮞ loss of NADPH/DHB [dehydroxybenzoic acid] (or both)

Answer 2

PKU

Question 3

⮞ Ochronosis
⮞ arthritis
⮞ black urine
..is the manifestations of?

Answer 3

Alkaptonuria (triad of alkaptonuria) AKU

Question 4

Inborn error with increase in Homogentisic Acid

Answer 4

Alkaptonuria (AKU)

Question 5

The main reason why there is no melanin in Albinism

Answer 5

walang tyrosinase

Question 6

A more serious type of Tyrosinemia, that causes renal and liver failure.
What is the enzyme deficiency?

Answer 6

⮞ Type 1 (hepatorenal tyrosinemia)

⮞ Fumarylacetoacetate hydrolase deficiency (FAH)

Question 7

A less serious type of tyrosinemia, that causes eye and skin lesions together with mental retardation. name the deficiency din pls mwah

Answer 7

⮞Type 2. or (oculocutaneous tyrosenemia)

⮞ tyrosine aminotransferase deficiency (TyAT)

Question 8

melatonin, serotonin and niacin are derivatives of?

Answer 8

tryptophan

Question 9

What pathway converts Trp 🡆 niacin (B3)?

Answer 9

Kynerenine-anthranilate pathway (kyn-anth P)

Question 10

2 vitamins needed to convert Trp 🡆 niacin?

Answer 10

B2

B6

Question 11

This is due to B2 and B6 deficiency

Answer 11

pellagra

Question 12

what are the 3 D’s of pellagra?

Answer 12

➟ Dermatitis
➟ Diarrhea
➟ Dementia

Question 13

vitamin B6 deficiency would cause

Answer 13

xanthurenic acid formation

Question 14

This Trp inborn error disease causes ; pellagra, cerebellar ataxia, intellectual deterioration. also what is the deficiency

Answer 14

Hartnup disease:
➟ Tryp oxygenase/ pyrollase deficiency.
➟ where Trp cannot convert to Niacin

Question 15

⮞ accumulation of serotonin

⮞ 5-HIAA is found in the urine

Answer 15

Carcinoid syndrome

Question 16

⮞ Impaired intestinal renal absorption of Trp (shows blue urine or indicanuria)

Answer 16

Blue diaper syndrome

Question 17

What gets absorbed and gets converted to indican, that produces blue urine?

Answer 17

Indole

Question 18

uronic acid is a keto acid from of

Answer 18

Histidine

Question 19

For histidine 🡆 glutamic acid, it has to pass thru the formation of

Answer 19

FIGLU (N-formiminoglutamate)

Question 20

FIGLU 🡆Glutamic acid thru

Answer 20

THFA (tetrahydrofolic acid) [vit B9]

Question 21

what are the derivatives of Histidine?

Answer 21

⮞ Histidine
⮞ Carnosine
⮞ Anserine

Question 22

⮞ convulsive seizures

⮞ has histidase deficiency

Answer 22

Histidenmia (no conversion of histidine 🡆 uronic acid)

Question 23

a urocanase deficiency that has elevated excretion of uronic acid in the urine

Answer 23

Urocanic Aciduria

Question 24

Branched amino acids are metabolized where?

Answer 24

⮞ Brain

⮞ Muscles

Question 25

3 branched-chain amino acids

Answer 25

⮞ Valine ⮞ Leucine ⮞ Isoleucine

Question 26

1st step in branched chain metabolism that produces keto acids

Answer 26

Transamination

Question 27

Decarboxylation is the 2nd step in branched A.A metabolism, what is the product?

Answer 27

CoA

Question 28

Deficiency in Maple syrup disease

Answer 28

a-ketoacid decarboxylase deficiency

Question 29

In Maple Urine Disease there is mental retardation, whuay?

Answer 29

accumulations of Val, Leu, Ile in the brain (shii is toxic yo)

Question 30

A cheesy odor of breath and body fluids

Answer 30

Isovaleric acidemia

Question 31

Accumulation and deficiency in Isovaleric acidemia?

Answer 31

Accumulation: ⮞ Isovaleryl CoA ⮞ Deficiency: Isovaleryl CoA DH

Question 32

2 substances derived from Lysine and use

Answer 32

saccharopine, pipecolicacid, used in industrial purposes

Question 33

An accumulation of Lysine and deficiency in Saccharopine Dehydrogenase

Answer 33

HyperLYSinemia

Question 34

Too much of this would inhibit Arginase of the urea cycle

Answer 34

Lysine

Question 35

What non-essential amino acid can be acquired from threonine?

Answer 35

Glycine

Question 36

In threonine degradation, conversion of a-ketobutyric acid is by

Answer 36

Threonine dehydratase

Question 37

Major pathway for the catabolism of threonine proceeds through the

Answer 37

formation of glycine

Question 38

What is needed to be done to yield aminoacetone?

Answer 38

dehydrogenation and decarboxylation

Question 39

The catabolism of this protein proceed to the formation of cysteine

Answer 39

Methionine

Question 40

In the catabolism of Methionine you first pass thru

Answer 40

SAM (S-Adenosyl-Methionine)

Question 41

In the conversion of methionine to cysteine you pass thru the formation of

Answer 41

Homocysteine

Question 42

SAM is a donor in what reactions?

Answer 42

Creatine, Choline, Epinephrine synthesis

Question 43

⮞ Dangerous substance when it accumulates in the body ⮞ corrodes the lining of blood vessel wall ⮞ eventually develops to atherosclerosis

Answer 43

Homocysteine

Question 44

What lowers Homocysteine levels?

Answer 44

Vitamin B6, B9 (folic acid), B12

Question 45

What is the only B-complex stored in the liver, and too much of this can cause hepatic damage?

Answer 45

B12

Question 46

An asymptomatic inborn error, that has an accumulation of Homocysteine. pls tell me the deficiency <3

Answer 46

⮞ Homocystinuria | ⮞ deficient in Cystathionine Synthetase (CTS)

Question 47

In Cystathioninuria, there is a deficiency in

Answer 47

Cystathionase

Question 48

2 pathways for catabolism of Cysteine

Answer 48

⮞ Direct oxidative (Cysteine sulfinate pathway) | ⮞ Transamination (3-Mercaptopyruvate pathway)

Question 49

This can result in RENAL CALCULI, where there is defective transport of cysteine and basic amino acids

Answer 49

Cystinuria

Question 50

Disease where Cystine accumulates in lysosomes, with a deficiency of what?

Answer 50

⮞ Cystinosis | ⮞ Cystathionase deficiency

Question 51

simplest amino acid <3

Answer 51

Glycine

Question 52

In the degradation of glycine, what is the major route?

Answer 52

Glycine synthase (glycine cleavage system)

Question 53

Glycine can be converted to what 'in the major route'

Answer 53

⮞ NADH (reduced nicotinamide adenine dinucleotide) ⮞ CO2 ⮞ NH3 (ammonia)

Question 54

Glycine 🡆 serine and vice versa thru

Answer 54

Serine Hydroxymethyltransferase (SERHMT)

Question 55

Oxidative deamination by glycine oxidase yields?

Answer 55

Glyoxylic acid

Question 56

Uses of glycine

Answer 56

heme, purine, creatine synthesis. also conjugates with cholic acid to form bile salt, and benzoic acid to form hippuric acid

Question 57

This causes the ff: ⮞ Diarrhea ⮞ Abdominal pain ⮞ Vomiting

Answer 57

Benzoic acid

Question 58

5 AA that can be converted to KETOGLUTARATE:

Answer 58

``` 🡆 K-HERQP The King - ketoglutarate is H - histidine E - glutamate/glutamic acid R - Arginine Queen's - Glutamine (Qt amine) Priority - Proline ```

Question 59

Purely ketogenic means: ⮞ ONLY be converted to ketone bodies ⮞ NOT GLUCOSE

Answer 59

Leucine only.

Question 60

BOTH ketogenic and glucogenic?

Answer 60

Phe, Tyr, Iso, Trypto, Lys