ECM Flashcards

1
Q

what is the function of the epithelium

A

its a barrier, it protects

limits what can pass through

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2
Q

What collagen is present in the basal lamina

A

Type IV collagen

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3
Q

How many amino acids are there

A

20

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4
Q

what tissues are not elastic made of collagen

A

Cartilage, tendons, ligaments

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5
Q

What are the functions of the ECM

A
Structural scaffolding- fibrillar collagen
Selective barrier- type IV collagen
Guide cell migration
Elasticity- elastin
Holding water- proteoglycans
Regulation of cell survival 
Regulation of cell proliferation 
Regulator of GF activities
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6
Q

what systems stimulate cells

A

Endocrine
Paracrine
Autocrine

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7
Q

What is endocrine

A

cells secrete a hormone into the vascular system (systemically) which then reaches its target cells
Ex; Steroid hormones

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8
Q

what is paracrine

A

cells secrete a hormone which diffuses to a nearby target cell.
Ex. wound healing, GF
Immune system; cytokine

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9
Q

What is autocrine

A

same cell both produces and responds to a given hormonal signal.
Ex. Immune system; IL-2 produced by T lymphocytes stimulates proliferation of T lymphocytes.

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10
Q

What are common features in GF and cytokines

A

they are protein hormones that affect cell behavior by binding to high affinity plasma membrane receptors.

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11
Q

what are the three GF receptor domains

A

Extracellular
Transmembrane
Intracellular

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12
Q

what are the two types of kinase receptors

A

Tyrosine kinase receptor

Serine/Threonine Kinase receptor

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13
Q

What does Phospho relay do in Tyrosine Kinase receptor activation

A

Signal transduction

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14
Q

where are the steroid hormone receptor located

A

Cytoplasm

Nucleus

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15
Q

What are common GFs

A
BMPs
TGF-betas 
Int
FGF
IGF
PDGF
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16
Q

Ways to inactivate GF activity

A
Inhibition by:
pro domain 
ECM binding protein
ECM antagonists 
ECM
17
Q

What does TGF-beta do

A

controls proliferation, differentiation.

Negative autocrine GF

18
Q

Disregulation of TGF-beta activation/signaling is associated with

A
Marfan syndrome
Camurati-Engelmann disease 
cancer
fibrosis
Scleroderma
19
Q

What type of cells are in bone

A

osteoprogenitor
osteoblasts
Osteoclasts
Osteocytes

20
Q

What is the function of BMP

A

Increase osteoblast differentiation

Ectopic bone formation

21
Q

What is the function of TGF

A

inhibition of osteoblast differentiation

osteoclasts?

22
Q

Fibrillin-1

A

The most common elastic protein in nature
binds to elastin
found in all dynamic connective tissues: arteries, lung, skin, elastic cartilage.

23
Q

two overlapping characteristics of LDS and MFS

A

Micrognathia

Retrognathia

24
Q

Unique characteristics to LDS

A

Bifid uvula
Cleft palate
hypertelorism

25
Q

LDS mutations

A

TGF-beta R1

26
Q

Achondroplasia

A
Autosomal dominant
dwarfism
malocclusion
crowding 
FGFR 3
27
Q

Crouzon Syndrome

A

CRANIOSYNOSTOSIS
Exophtalmos
narrow high arch
FGFR 2 or 3 mutation

28
Q

Apert syndrome

A

Autosomal dominant
Acro-cephalo-syndactyly
syndactyly; suggest no apoptosis bn fingers/toes
FGFR 2 mutation

29
Q

Dental sx in Apert syndrome

A

High arched palate
pseudo mandibular prognathism due to under developed mid facial region.
Narrow palate
Crowding