SPECIAL TISSUES I

Question 1

collective term for materials outside the cell

functions as support for cells

Answer 1

extracellular matrix

Question 2

major component of most connective tissue

most abundant structural protein in the body

there are 28 distinct types

Answer 2

collagen

Question 3

intracellular steps in collagen synthesis

1. H
2. G
3. DB
4. THF

Answer 3

hydroxylation
glycation
disulfide bonding
triple helix formation

Question 4

Keloid has an increase in the enzyme __________

Answer 4

prolyl hydroxylase

Question 4

osteogenesis imperfecta causes a decrease in collagen type _____

Answer 4

type 1

Question 5

in EDS type 4 there is problem in collagen type ___ and ___

Answer 5

type 3 and 4

Question 6

EDS type 6 has a decrease in the enzyme: ___________

Answer 6

EDS type 6 lysl hydroxylase

Question 7

EDS type 7
enzyme: ______

Answer 7

procollagen peptidase

Question 8

in achondrodysplasia there is a decrease in type ____ collagen

Answer 8

Type 2

Question 9

in epidemolysis bullosa there is a decrease of ________

Answer 9

anchoring fibrils

Question 10

in Allport syndrome there is an antibody attacking your type ___ collagen

Answer 10

type 4 collagen

Question 11

in menke’s diseases there is a defect in ____ transport

Answer 11

copper

Question 12

is found in the skin and tissue of the body

protein in connective tissue that is elastic and allows many tissues in the body to resume their shape after stretching or contracting

protein that helps to keep our skin flexible but tight

Answer 12

elastin

Question 13

deletion of elastin gene

manifestations:
looks old for their age
sagging skin

Answer 13

cutis laxa

Question 14

excessive production of elastin

manifestations:
tightening of the skin
inability to move the skin
joints are tightened

Answer 14

scleroderma

Question 15

large glycoprotein
secreted into the EM by fibroblast
found in lens, periosteum and aorta

Answer 15

fibrillin

Question 16

diagnosis of marfan syndrome
diagnosis includes ____ and ____ signs

Answer 16

steinberg’s sign
walker murdoch sign

Question 17

in glycosaminoglycans

GENERAL RULE:
All GAGs contain

sulfate except _____
sugar acid except _______

Answer 17

hyaluronic acid
keratan sulfate

Question 18

the majority of GAGs in the body are linked to core proteins forming _______

Answer 18

proteoglycans

Question 19

mutations in a gene encoding such as ___________ in the degradation of one or more GAGS

Answer 19

lysosomal hydrolase

Question 20

in Hurler’s syndrome there is an enzymatic defect of alpha ________-

Answer 20

alpha-L-Iduronidase

Question 20

in hunters syndrome there is a enzymatic defect: ___________

it is also ________ (only in males)

Answer 20

iduronate sulfatase
x-linked

Question 21

in morquio syndrome
enzyme defect: __________

Answer 21

galactosamine-6-sulfatase or Beta Galactosidase

Question 22

in Maroteaux Lamy
enzyme defect: _____________

Answer 22

N-acetylgalatosamine 4-sulfatase