Exam 1 (AA's, Proteins, Non-Protein Nitrogens) Flashcards Preview

CC 17 > Exam 1 (AA's, Proteins, Non-Protein Nitrogens) > Flashcards

Flashcards in Exam 1 (AA's, Proteins, Non-Protein Nitrogens) Deck (165)
Loading flashcards...
1

20 amino acids are divided into two groups:

Essential
Non-essential

2

How are essential amino acid supplied:

through diet when we eat proteins

3

How are non-essential amino acids supplied:

They are made from the essential amino acids, converted by liver enzymes

4

This is the transfer of one amine group from 1 amino acid to another, done by liver enzymes to make new amino acids from recycled ones:

transamination

5

The removal of an amino group from an amino acid; produces ammonia which is converted to urea and excreted in urine:

Deamination

6

Amino acids consist of these parts:

Amino group
Carboxyl group
Hydrogen
R-group

7

Formation of a peptide involves the ___terminal end of one amino acid reacting with the ___terminal end of another through this type of reaction:

C-terminal, N-terminal
hydrolysis reaction (H2O is released)

8

Define polypeptide:

chain of amino acids linked via peptide bonds

9

Why are amino acids zwitterions:

Amino has + charge
Carboxyl group has - charge
*net charge = 0
*makes them good buffers

10

Describe secondary peptide structure:

coiling into helix by h bonds of neighboring peptides

11

Describe tertiary peptide stucture:

Folding of peptide chain by many forces

12

The interaction of the polypeptides results in a :

protein

13

Proteins have ___ main stages of assembly:

Primary, Secondary, Tertiary, Quaternary

14

T/F All proteins contain all 20 amino acids:

False

15

When errors in metabolism occur, these can build up in blood and urine and be detected by the lab:

amino acids

16

T/F When certain amino acids accumulate in blood, they can be toxic and result in death:

True

17

What are the two categories of aminoacidopathies:

Primary
Secondary

18

Describe primary vs secondary aminoacidopathy:

*Primary: inherited defect in enzymatic pathway
*Secondary: disease of a specific organ where amino acid is synthesized (liver, kidney)

19

List the 6 Primary (inherited) amioacidopathies:

Alkaptonuria
Cystinuria ???
Homocystinuria
Maple Syrup Urine
Phenylketonuria
Tyrosinemia

20

In alkaptonuria, what enzyme is inhbitied, and what builds up:

*enzyme: homogentisic acid oxidase
*builds up: homogentisic acid

21

Which aminoacidopathy creates a buildup of homogentisic acid, causing generalized pigmentation and arthritic-like degeneration:

Alkaptonuria

22

In alkaptonuria, this will happen to urine up standing, and is caused by a buildup of what:

*urine will darken
*buildup of homogentisic acid

23

Cystinuria is not an enzymatic defect, but a defect in _____ _____:

renal absorption

24

Buildup of cysteine causes:

renal calculi (cysteine stones)

25

This aminoacidopathy is caused by a defect in Branched Chain Ketoacid Dehydrogenase:

Maple Syrup Urine Disease
(MSUD)

26

What builds up in MSUD:

Ketoacids (leucine, isoleucine, valine)

27

What signs/symptoms result from MSUD:

*maple syrup smell in urine, skin, breath
*mental retardation
*convulsions
*acidosis
*death

28

What is the defect in MSUD:

the defect is in the Branched Chain Ketoacid Dehydrogenase
(ketoacids then buildup)

29

What is the defect in Type 1 Tyrosinemia:

FAA hydrolase (most severe)

30

Which type of Tyrosinema is most severe?

Type 1