SPECIAL TISSUES I Flashcards

1
Q

collective term for materials outside the cell

functions as support for cells

A

extracellular matrix

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2
Q

major component of most connective tissue

most abundant structural protein in the body

there are 28 distinct types

A

collagen

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3
Q

intracellular steps in collagen synthesis

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

hydroxylation
glycation
disulfide bonding
triple helix formation

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4
Q

Keloid has an increase in the enzyme __________

A

prolyl hydroxylase

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4
Q

osteogenesis imperfecta causes a decrease in collagen type _____

A

type 1

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5
Q

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

A

type 3 and 4

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6
Q

EDS type 6 has a decrease in the enzyme: ___________

A

EDS type 6 lysl hydroxylase

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7
Q

EDS type 7
enzyme: ______

A

procollagen peptidase

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8
Q

in achondrodysplasia there is a decrease in type ____ collagen

A

Type 2

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9
Q

in epidemolysis bullosa there is a decrease of ________

A

anchoring fibrils

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10
Q

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

A

type 4 collagen

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11
Q

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

A

copper

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12
Q

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

A

elastin

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13
Q

deletion of elastin gene

manifestations:
looks old for their age
sagging skin

A

cutis laxa

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14
Q

excessive production of elastin

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

A

scleroderma

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15
Q

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

A

fibrillin

16
Q

diagnosis of marfan syndrome
diagnosis includes ____ and ____ signs

A

steinberg’s sign
walker murdoch sign

17
Q

in glycosaminoglycans

GENERAL RULE:
All GAGs contain

sulfate except _____
sugar acid except _______

A

hyaluronic acid
keratan sulfate

18
Q

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

A

proteoglycans

19
Q

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

A

lysosomal hydrolase

20
Q

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

A

alpha-L-Iduronidase

20
Q

in hunters syndrome there is a enzymatic defect: ___________

it is also ________ (only in males)

A

iduronate sulfatase
x-linked

21
Q

in morquio syndrome
enzyme defect: __________

A

galactosamine-6-sulfatase or Beta Galactosidase

22
Q

in Maroteaux Lamy
enzyme defect: _____________

A

N-acetylgalatosamine 4-sulfatase