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BIOCHEMISTRY > protein metabolism 2 > Flashcards

protein metabolism 2 Flashcards

1
Q 
⮞ Melanin
⮞ Catecholamines
⮞ T3
⮞ T4
..are major derivatives of?
A

Phe & Tyr

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2
Q

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

A

PKU

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3
Q

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

A

Alkaptonuria (triad of alkaptonuria) AKU

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4
Q

Inborn error with increase in Homogentisic Acid

A

Alkaptonuria (AKU)

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5
Q

The main reason why there is no melanin in Albinism

A

walang tyrosinase

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6
Q

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

A

⮞ Type 1 (hepatorenal tyrosinemia)

⮞ Fumarylacetoacetate hydrolase deficiency (FAH)

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7
Q

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

A

⮞Type 2. or (oculocutaneous tyrosenemia)

⮞ tyrosine aminotransferase deficiency (TyAT)

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8
Q

melatonin, serotonin and niacin are derivatives of?

A

tryptophan

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9
Q

What pathway converts Trp 🡆 niacin (B3)?

A

Kynerenine-anthranilate pathway (kyn-anth P)

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10
Q

2 vitamins needed to convert Trp 🡆 niacin?

A

B2

B6

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11
Q

This is due to B2 and B6 deficiency

A

pellagra

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12
Q

what are the 3 D’s of pellagra?

A

➟ Dermatitis
➟ Diarrhea
➟ Dementia

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13
Q

vitamin B6 deficiency would cause

A

xanthurenic acid formation

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14
Q

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

A

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

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15
Q

⮞ accumulation of serotonin

⮞ 5-HIAA is found in the urine

A

Carcinoid syndrome

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16
Q

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

A

Blue diaper syndrome

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17
Q

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

A

Indole

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18
Q

uronic acid is a keto acid from of

A

Histidine

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19
Q

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

A

FIGLU (N-formiminoglutamate)

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20
Q

FIGLU 🡆Glutamic acid thru

A

THFA (tetrahydrofolic acid) [vit B9]

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21
Q

what are the derivatives of Histidine?

A

⮞ Histidine
⮞ Carnosine
⮞ Anserine

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22
Q

⮞ convulsive seizures

⮞ has histidase deficiency

A

Histidenmia (no conversion of histidine 🡆 uronic acid)

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23
Q

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

A

Urocanic Aciduria

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24
Q

Branched amino acids are metabolized where?

A

⮞ Brain

⮞ Muscles

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25
Q

3 branched-chain amino acids

A

⮞ Valine
⮞ Leucine
⮞ Isoleucine

26
Q

1st step in branched chain metabolism that produces keto acids

A

Transamination

27
Q

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

A

CoA

28
Q

Deficiency in Maple syrup disease

A

a-ketoacid decarboxylase deficiency

29
Q

In Maple Urine Disease there is mental retardation, whuay?

A

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

30
Q

A cheesy odor of breath and body fluids

A

Isovaleric acidemia

31
Q

Accumulation and deficiency in Isovaleric acidemia?

A

Accumulation:
⮞ Isovaleryl CoA
⮞ Deficiency: Isovaleryl CoA DH

32
Q

2 substances derived from Lysine and use

A

saccharopine, pipecolicacid, used in industrial purposes

33
Q

An accumulation of Lysine and deficiency in Saccharopine Dehydrogenase

A

HyperLYSinemia

34
Q

Too much of this would inhibit Arginase of the urea cycle

A

Lysine

35
Q

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

A

Glycine

36
Q

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

A

Threonine dehydratase

37
Q

Major pathway for the catabolism of threonine proceeds through the

A

formation of glycine

38
Q

What is needed to be done to yield aminoacetone?

A

dehydrogenation and decarboxylation

39
Q

The catabolism of this protein proceed to the formation of cysteine

A

Methionine

40
Q

In the catabolism of Methionine you first pass thru

A

SAM (S-Adenosyl-Methionine)

41
Q

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

A

Homocysteine

42
Q

SAM is a donor in what reactions?

A

Creatine, Choline, Epinephrine synthesis

43
Q

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

A

Homocysteine

44
Q

What lowers Homocysteine levels?

A

Vitamin B6, B9 (folic acid), B12

45
Q

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

A

B12

46
Q

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

A

⮞ Homocystinuria

⮞ deficient in Cystathionine Synthetase (CTS)

47
Q

In Cystathioninuria, there is a deficiency in

A

Cystathionase

48
Q

2 pathways for catabolism of Cysteine

A

⮞ Direct oxidative (Cysteine sulfinate pathway)

⮞ Transamination (3-Mercaptopyruvate pathway)

49
Q

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

A

Cystinuria

50
Q

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

A

⮞ Cystinosis

⮞ Cystathionase deficiency

51
Q

simplest amino acid <3

A

Glycine

52
Q

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

A

Glycine synthase (glycine cleavage system)

53
Q

Glycine can be converted to what ‘in the major route’

A

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

54
Q

Glycine 🡆 serine and vice versa thru

A

Serine Hydroxymethyltransferase (SERHMT)

55
Q

Oxidative deamination by glycine oxidase yields?

A

Glyoxylic acid

56
Q

Uses of glycine

A

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

57
Q

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

A

Benzoic acid

58
Q

5 AA that can be converted to KETOGLUTARATE:

A 
🡆 K-HERQP 
The King - ketoglutarate 
is H - histidine 
E - glutamate/glutamic acid 
R - Arginine 
Queen's - Glutamine (Qt amine)
Priority - Proline
59
Q

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

A

Leucine only.

60
Q

BOTH ketogenic and glucogenic?

A

Phe, Tyr, Iso, Trypto, Lys

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