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Flashcards in Exam I Deck (42):
1

L designation

The stereoisomer of amino acids that are incorporated into proteins in vivi

In Fischer projection, amine group points LEFT

2

Zwitterion

Occurs around physiological pH. Carboxyl group is deprotonated and amino group is protonated

3

pKa amino acid terminals

C -> 2.2

N -> 9.4

4

L Dopa

A Tyrosine derivative drug with an extra hydroxyl on the ring. Used to treat Parkinson's

5

Branched chain AA

Valine
Leucine
Isoleucine

6

Histidine (His) (H)

Aromatic; pKa of 6; co-ordinates metal ions in hemoglobin

7

Basic AA

Lysine, Arginine

Helps stabilize the negative phosphate backbones of DNA

8

Phenylalanine (Phe) (F)

Aromatic; Promotes efficient stacking of protein (especially in cellular membranes)

9

Glycine (Gly) (G)

Achiral;
Most flexible AA; usually found in tight places and helps avoid steric hindrance

10

Sulfur Containing AA

Met -> Start codon

Cys --> Tertiary structure ( disulfide bridges); can be disrupted by urea/ethanomercaptol

11

Alcohol Containing

Involved in phosphorylation reactions; forms phosphate esters

Involved in protein signaling (glycoproteins)

Ser, Thr, Tyr

12

Proline (Pro) (P)

A pyrollidine heterocyclic;

Forms kinks and tight turns

Found in Elastin (nonhydroxylated)

Found in Fibrous proteins as Hydroxyproline (usually as "x" in Gly-X-Y motif); stabilized triple helix

Found in hairpin loops at the sharp turn (pos 2)

13

Modified AA

4-hydroxyproline and 5-hydroxyglycine help stabilize protein

14

Vitamin K

Essential in coagulation cascade; activated by gamma-carboxyglutamate (modified AA)

Deficiency leads to hemmorhaging

15

Monoasaccharides

Glucose (diabetes/hyperglycemia)

Fructose (fructose accumulation/hypoglycemia)

Galactose (hydrolysis of lactose; mammalian mill synthesis)

16

Disaccharides

Sucrose (1a, 2b linkage); Glucose + Fructose

Lactose (b 1,4 linkage); Glucose + Galactose

Maltose (a 1,4 linkage); Glucose + Glucose

17

Other saccharine Containing compounds

Glycoproteins: Oligo + proteins

Glycolipids: Oligo + lipids

Proteoglycan: Glycosaminoglycan + proteins

18

Sphingolipids

Serine and Palmitic acid derivative

Forms ceramides by attaching sphingosine backbone to amino group

19

Gaucher Disease

Glucocerebrosidase deficiency

Lysosomal (cellular) accumulation of glucocerebroside in bones, lungs, liver, spleen

20

Tay-Sachs Disease

Hexominodase deficiency

Build up of GM2 ganglioside

21

Niaman-Pick Disease

Sphingomyelinase deficiency; cherry spots visible in eye

Build up of sphingomyelin

22

Alpha Helices

Stabilized by H bonding parallel to helix axis; 3.6 aa/turn

Amphipathic aa are at protein surface; hydrophilic project outwards towards solvent; hydrophobic project onward towards protein core

23

Beta Sheets

Parallel (same direction wrt terminals); Antiparallel (opposite directions, stronger interactions) ; Mixed (both)

Stabilized by H bonding

Provide bulk of hydrophobic core due to optimal dense packaging

24

Reverse Turn; Hairpin Loop; Beta Turn

Comprised of 4 amino acids; Proline at pos 2 and Glycine at pos 3

Stabilized by H bonding (carbonyl of aa 1 and amide proton of aa 4)

25

Rossman Fold (Super-secondary structure)

Several Beta & Beta motifs; A helix connects two parallel Beta sheets

Usually found in glycolysis enzymes

26

Prions

Contains only proteins; lacks DNA & RNA

Propagates misfolding of proteins

27

Prion derivative diseases

Infection: Mad Cow Diseass

Genetic Mutation: Creutzfeldt-Jakob disease (CJD)

28

Tertiary structure

Hydrogen (acid/base)
ionic (salt)
Disulfide (urea/mercaptoethanol)
Hydrophobic interactions (detergent)

29

Actin fold

Structural domain in G-actin

Region of protein that folds independently; super-secondary structures

30

Triple Helix

Common for fibrous proteins

Repeat Gly-X-Y motif; X being Proline and Y being lysine (both hydroxylated)

31

Vitamin C

Required for Proline and Lysine hydroxylation; deficiency leads to inadequate X-Y and leads to scurvy

32

Ehlers Danlos Syndrome

Mutations in formation of Collagen type I and Collagen type III

33

Osteogenesis Imperfecta *

Also known as Brittle Bone Disease

Type I Collagen mutation

34

Elastin

Elastic connective tissue in lungs, arterial walls, etc

Composed of small nonpolar AA (G, V, A) as well as P and K

35

Alzheimer's

Accumulation of B Amyloid

36

Cystic Fibrosis

Deletion of 3 base pairs (phenylalanine at pos 508)

Thick mucus accumulation in lungs

37

Amyloidosis

Accumulation of amyloid proteins

Primary amyloidosis --> multiple myeloma

Secondary amyloidosis --> Tuberculosis, Chron's

Diabetes T2 --> Amyllin in pancreas

Dialysis associated amyloidosis --> Accumulation in blood seen in long term
Dialysis patients

38

Sickle Cell Anemia

B globin

39

Huntington's

Poly-Glutamine Repeat

Neurodegenerative

40

Parkinson's

Alpha Synuclein

41

Heat Shock Proteins

Cellular levels are increased during elevated temp in order to reduce protein unfolding

HSP60, HSP70, Chaperones

Utilize ATP

42

Histones

Alkaline proteins; package DNA into nucleosides and then into dhromatin

Interact with negatively charged phosphate backbones with Lysine + Arginine residues