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MCBM Exam 4 Kat > Nucleotide Metabolism > Flashcards

Flashcards in Nucleotide Metabolism Deck (41):
1

What are the 3 basic principle components of nucleotides?

Nitrogenous base
Pentose Sugar
One or more phosphate groups

2

What are nitrogenous bases?

Heterocyclic ring compounds of nitrogen and carbon

3

What are the two types of nitrogenous bases?

Pyrimidines (1 ring) and Purines (2 rings)

4

What are the Pyrimidines?

Pyramids are CUT

Cytosine
Uracil
Thymine

5

What are the Purines?

Pure As Gold

Adenine
Guanine

6

What are the types of pentose sugars used in nucleotides?

Ribose and Deoxyribose

7

Where do nucleosides attach?

C-1 on sugar
N-9 on purine
N-1 on pyrimidine

8

What are nucleotides formed by?

Attachment of phosphate to sugar in nucleoside

9

Are phosphates ever attached to the base?

No never

10

How many phosphates are attached to sugars and at which sites on the sugar?

At least one phosphate is attached to C-2, C-3 or C-5 of the sugar

11

Can phosphates be added together?

Yes they can!

12

What are the deoxyribonucleotides?

Adenine
Guanine
Thymine
Cytosine

13

Name the two pathways for biosynthesis of nucleotides?

1) de novo (from new)
2) salvage

14

What is de novo biosynthesis?

Synthesis from metabolic precursors (Energy expensive)

15

What is salvage biosynthesis?

Recycle free bases and nucleosides released from nucleic acid breakdown (Energy economical)

16

What compound is a key substrate in both purine and pyrimidine de novo synthesis?

PRPP

17

What can IMP convert?

AMP or GMP

18

In purine synthesis what is the five membered ring built on first?

PRPP

19

What is the committed step of de novo purine biosynthesis?

Step 1: formation of 5-phosphoribosyl-1-amine

20

What inhibits the first step of de novo purine biosynthesis?

Asaserine, acivicin

21

What is required to make one molecule of insinuate from ribose-5-phosphate?

1 ATP for PRPP
2 ATP for 2N10-THF
4 ATP for 4 steps

SOOOOOO 7 ATP total

22

What do you watch for in the "wobble rules"?

Inosinate

23

What are the four mechanisms of de novo purine biosynthesis

1) Feedback inhibition of PRPP synthetase by end products
2) Feedback inhibition of committed step by end products
3) Feedback inhibition of branches by each end product
4) Reciprocal use of GTP & ATP in AMP, GMP pathways

24

What does the first step of pyrimidine biosynthesis and where does it occur?

This is the formation of carbamoyl phosphate which occurs in the cytoplasm (urea cycle in mitochondria)

25

What is the committed step of de novo pyrimidine biosynthesis?

Step 2: N-carbamoylaspartate which is catalyzed by aspartate transcarbamoylase

26

In humans are purine salvaged or do they go through de novo synthesis?

Purines are salvaged

27

What are XO inhibitors used for?

Treatment of gout

28

What is ADA (Adenosine deaminase deficiency) called?

SCID (Severe combined immunodeficiency)

29

What is SCID also known as?

Boy-in-the-bubble disease

30

Is there a cure for SCID?

No

31

What is ADA?

Rare heritable disease leading to one form of SCID (20% of all SCID cases)

32

What happens in SCID?

T and B lymphocytes do not develop properly

33

What is gout?

A probably heritable disease (mostly males) leading to elevated [uric acid] in blood and tissues.

34

What does excess urate lead to?

Precipitation of sodium rate in tissues which can become inflamed, painful and arthritic. Deposition in kidney tubules leads to damage.

35

What two mechanisms lead to elevated serum urate?

Hyperactive PRPP
Partial lack of HGPRT

36

What do you use to treat gout?

Allopurinol
Probenecid (up urate excretion)
Febuxostat (Uloric)

37

What is the first FDA approved medication to lower gout in over 40 years?

Febuxostat (Uloric)

38

For what individuals is Uloric good for?

Allergy to allopurinol
Kidney damage

39

What is Lesch-Nyhan Syndrome?

A very rare X-linked recessive disorder whose symptoms manifest by 2 years of age

40

What are the symptoms of Lesch-Nyhan Syndrome?

Mental retardation
Poor coordination
Extreme hostility and compulsive self-destructive tendencies (must be protected from themselves)
Gout

41

What is Lesch-Nyhan Syndrome caused by?

A complete lack of HGPRT which leads to brain damage.