proteins

Question 1

what is connective tissue

Answer 1

complex extracellular matrix that fills the space between cell and binds cells and tissue together

Question 2

what are the functions of connective tissue

Answer 2

provides support and connecting frame work for all other tissue

Question 3

name the three major classes of biomolecules in extracellular matrix

Answer 3

1. the structural proteins: collagen, elastin, & fibrillin
2. specialized protein: fibronectin & laminin
3. proteoglycans

Question 4

what are the functions of ECM

Answer 4

1. protection: protect brain from skull
2. structural support: bone
3. connection & binding: ligament, tendon
4. storage: bone stores ca & p
5. transportation: blood
6. immune function: WBC

Question 5

name the elastic protein found in tissue where stretch is needed

Answer 5

elastin

Question 6

elastin is a ___________ polypeptide that contain _________ fiber as major component
a) beta plates, , collagen
b) alpha chain, fibronectin
c) linear, elastic

Answer 6

c) linear, elastic

Question 7

what are the properties of elastin

Answer 7

stretch and resume their original length
high tensile structure

Question 8

where can we find elastin

Answer 8

Elastic ligaments, walls of arteries, lungs and vocal cord, epiglottis, ligamenta flava
Smaller quantities found in skin, ear cartilage etc.

Question 9

elastin is rich in ______________amino acids

Answer 9

nonhydroxylated proline, lysine, glycine

Question 10

unlike collagen elastin can exist on _______________ forms

Answer 10

no glycosylated

Question 11

tropoelastin with fibrillin forms

Answer 11

scaffolding

Question 12

cross linking occurs extracellularly via

Answer 12

lysyl oxidase

Question 13

lysyl oxidase gives elastin what property

Answer 13

elastic property

Question 14

describe the structure of elastin

Answer 14

highly cross linked insoluble, amorphous structure

Question 15

true or false
hydroxyproline (1%) present, but no hydroxylysine.

Answer 15

true

Question 16

which protein bind to tropoelastin to form elastic fiber

Answer 16

fibrillin

Question 17

name the enzymes that can degrade elastin protein

Answer 17

elastase enzyme such as neutrophil-elastase

Question 18

what inhibits a no of protease including neutrophil-elastase

Answer 18

α1- antitrypsin

Question 19

what is α1- antitrypsin

Answer 19

is a class of protease inhibitors that normally inhibits elastase

Question 20

α1-antitrypsin is synthesized by

Answer 20

liver

Question 21

α1-Antitrypsin deficiency results in

Answer 21

unopposed elastase activity, which can cause COPD

Question 22

PANACINAR Emphysema caused by

Answer 22

deficiency of α1- antitrypsin.

Question 23

deficiency of α1- antitrypsin. can cause

Answer 23

loss of inhibitory action on neutrophil elastase
excessive destruction of elastin in lung alveolus
excess elastase activity

Question 24

name the disease
-severe in the lower lobes
-associated with 𝜶1-antitrypsin deficiency but can also be found in patients in whom no genetic abnormality has been identified.

Answer 24

panacinar emphysema

Question 25

which type of emphysema is usually predominates in the upper lobes

Answer 25

centriacinar emphysema

Question 26

glycoprotein that cell adhesion & migrate

Answer 26

fibronectin

Question 27

insoluble monomers involved in linking in

Answer 27

cell surface integrins to ECM

Question 28

state two characteristics of fibronectin

Answer 28

soluble protein assists in blood clotting

Question 29

what is sequence of aa that binds to the receptor of fibronectin

Answer 29

Arg-Gly-Asp

Question 30

what are the functions of fibronectin

Answer 30

-binding heparin fibrilin and collagen -cell migration -cell morphology -embryogenesis -cytoskeletal organization -attach cells to various components of the ECM -can circulate in plasma to assist with clotting -biofilm formation via cell adhesion to basement membrane

Question 31

name the abnormality that cause early onset of scoliosis and skeletal abnormalities

Answer 31

spondylometaphyseal dysplasia

Question 32

spondylometaphyseal dysplasia is associated with

Answer 32

mutation in fibronectin

Question 33

which protein is the only one that exists as an insoluble dimer as well as a soluble clotting factor

Answer 33

fibronectin

Question 34

which is the major protein component of renal glomerular and other basal laminas

Answer 34

laminin

Question 35

how many elongated polypeptide chains in laminin

Answer 35

3 α, β1, β 2

Question 36

shape of laminin protein

Answer 36

cruciform shape

Question 37

what are the binding sites of laminin

Answer 37

type 4 collagen, heparin & integrins on cell surface and entactin (nidogen)

Question 38

what is fibrillin

Answer 38

a glycoprotein found in many tissue

Question 39

fibrillin is secreted into ____________ by _________

Answer 39

ECM, Fibroblast

Question 40

which protein provides a scaffold for deposition of elastin

Answer 40

fibrillin

Question 41

name the disease caused by mutation in the gene coding for fibrillin on chromosome 15

Answer 41

marfans disease

Question 42

Marfans disease is inherited as an ____________ trait

Answer 42

autosomal dominant trait

Question 43

name the disease that affect eyes and cause ectopia lentis

Answer 43

marfans disease

Question 44

name the disease has following presentations long extremities including fingers scoliosis myopia and lens dislocation (upward dislocation)

Answer 44

marfans disease

Question 45

what is scoliosis

Answer 45

sideways curvature of the spine (often diagnosed in adolescent)

Question 46

how marfan's syndrome affect skeletal system

Answer 46

hyper extensibility of joints, tall sature

Question 47

how marfan's syndrome affect CVS

Answer 47

dilatation of aorta( aortic aneurysm and dissection)

Question 48

deficiency of which enzyme cause homocystinuria

Answer 48

cystathionine synthase deficiency

Question 49

symptomsw

Question 50

difference

Question 51

what is proteoglycans

Answer 51

they are protein that contain covalently linked glycosaminoglycans major component of ECM

Question 52

proteoglycans are formed by polysaccharide called

Answer 52

glycosaminoglycans( GAH, 95%), protein (1%)