quiz 4.2

Question 1

dietary def in vitC , leading to abnorml ollagen

Answer 1

scurvy

Question 2

deficient type3 collagen

progressive deterioration of collagens

Answer 2

Ehlers-Danlos

Question 3

dse of type3 collagen

leads to hematuria, end stage renal disease

Answer 3

Alport syndrome

Question 4

keratin defects

skin blistering

Answer 4

epidermolysis bullosa

Question 5

deficiency in copper

leads to defective crosslinking of collagen and elastin by lysyl oxidase

Answer 5

menkes dse

Question 6

overload ot copper

Answer 6

wilsons dse

Question 7

structural CT proteins

Answer 7

collagen
elastin
fibrillin
keratin
fibroins

Question 8

3 types of structural tissue

Answer 8

collagen
elastin
reticular

Question 9

the collagen molecule composed of three lefthanded polypeptide helices coiled around each other to form a righthanded superhelix

Answer 9

tropocollagen

Question 10

distinctive amino acid conposition of collagen

Answer 10

1/3 glycine

1/4 proline

Question 11

occurence of glycin ein collage is

Answer 11

every third psoition of the alpha chain

Question 12

100 AAs of the X and Y in gly-X-Y are

Answer 12

proline and hydroxyproline respectively

Question 13

hydroxylTion of hydroxyproline partciipates in ____ while hydroxylation serves to form ______

Answer 13

interchain hydrogen bonding

attchments for polysacchs

Question 14

proline hydroxylase requires what in the conversion of proline to hyp

Answer 14

ascorbic acid

Question 15

sugars are added to collagen by the successive action of what enzymes

Answer 15

gaalctosyl transferase

glucosyl transferase

Question 16

the carbohydrate with the collage is for what function

Answer 16

directing fibril assembly

Question 17

glycin is criticla in collagen bcoz

Answer 17

it is small

Question 18

what part of the proline repel one another by steric hindrance forcing the chain into a thin stretched out helix

Answer 18

pyrrolidine

Question 19

lysine oxidase converts the epsilon amino group of lysin or hyl to the aldehyde derivated _____

Answer 19

allysine

Question 20

allysin react blabla to produce ____ to form stable cross link

Answer 20

schiff base

Question 21

sse of animLs due to ingestion of a certain seed

Answer 21

lathyrism

Question 22

most seriousnkind of ehlees danlos

Answer 22

type 4

spontaneous rupture of bld bessels

Question 23

dystrophic form in epidermolysis bullosa is die to

Answer 23

mutation of COL7A1

Question 24

due to deletion sof alpha1 gene but a single amino acid change is sufficient to cause certain forms of this dse

Answer 24

osteogensis inperfecta

Question 25

CR protein responsible for extensivility and elastic recoil

Answer 25

elastin

Question 26

elastic has an _____ made of approximately 830 akino acid

Answer 26

irregular or random coil confirmation

Question 27

in contrast to colllagen, elastin has no ____ and vey little ____

Answer 27

hydroxylysine | hydroxyproline

Question 28

a molecular knot that ties 4 chains of elastin net together

Answer 28

desmonsine

Question 29

cindensation if 3 allysine residues with the epsi-amino acid of a lysine residue gives a product _____

Answer 29

desmosine

Question 30

disorder of elastin degardation

Answer 30

alpha1-antitrypsin deficiency

Question 31

tx for AAT deficiency

Answer 31

weekly IV

Question 32

what causes the emphysema and cirrhosis in AAT deficiency

Answer 32

neutrophils release elastase

Question 33

kind of intramolecular cross links in collagen

Answer 33

aldol

Question 34

intramolecular cross link in elastin

Answer 34

desmosine

Question 35

mutations in the gene for fibrillin-1 leads to ___

Answer 35

marfan syndrome

Question 36

characterized by ectopia lentis long leg bons and spiderlike fingers enlarged aorta

Answer 36

marfan syndrome

Question 37

major proteins of hair fingernails and a major fraction of animal skin

Answer 37

alpha keratin

Question 38

basic atructural unit og keratin

Answer 38

protofibril

Question 39

basic structural unit of hair

Answer 39

microfibeil

Question 40

alpha keratin is hardened by introduction of

Answer 40

disulfide croslinks

Question 41

how is microfibrilformed

Answer 41

8 protofibrils combine either ciruclar or square arrangement

Question 42

is alpha keratin mono di or polymer?

Answer 42

dimer

Question 43

structure ng fibroins

Answer 43

gly every other AA tas either ser or ala in between

Question 44

improrant glycoprotein fir cell adhesion and migration

Answer 44

fibronectin

Question 45

extracellular glycoprotein that enables eptihelial cells to attach underlying connective tissue

Answer 45

laminin

Question 46

laminin is a alrge compelx of ___ polypep chains arracged in the shape of a criss and held together by disulfide bonds

Answer 46

3