Special tissues Flashcards

0
Q

Structural proteins

A

Collagen
Elastin
Fibrillin

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1
Q

Major classes of extracellular matrix

A

Structural proteins
Specialized proteins
Proteoglycans

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2
Q

Specialized proteins

A

Fibronectin

Laminin

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3
Q

Most abundant protein in all tissues

A

Collagen

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4
Q

Collagen approximately how many amino acids?

A

1000 AA

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5
Q

Amino acid components in collagen

A

Glycine
Hydroxyproline
Proline

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6
Q

Alanine

A

Bigger molecule
Create a bulkier chain
No room for hydroxyproline and proline to twist

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7
Q

Glycine

A

Smaller molecule

Twisting of the chain possible

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8
Q

This amino acid confer the rigidity of collagen molecule

A

Proline and hydroxyproline

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9
Q

AA that helps form water bridges that stabilize the triple helix at body temperature?

A

Hydroxproline

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10
Q

Collagen synthesis

RER would form your

A

Pro alpha collagen

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11
Q

Collagen synthesis

A

Study collagen synthesis

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12
Q

Most important class of collagen

A

Anchoring fibrils

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13
Q

Give the type of collagen that match to this class of collagen.

Fibril forming

A

1,2,3,4

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14
Q

Give the type of collagen that match to this class of collagen.

Network like

A

4,8,10

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15
Q

Give the type of collagen that match to this class of collagen.

FACITs

A

9,12,24,26,29

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16
Q

Give the type of collagen that match to this class of collagen.

Beaded filaments

A

6

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17
Q

Give the type of collagen that match to this class of collagen.

Anchoring fibrils

A

7

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18
Q

Give the type of collagen that match to this class of collagen.

Transmembrane domain

A

13, 17

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19
Q

Give the type of collagen that match to this class of collagen.

Others

A

15,17

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20
Q

Cause of osteogenesis imperfecta

A

Mutation of collagen type 1

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21
Q

Manifestation of osteogenesis imperfecta

A

Blue gray sclera (pathognomonic sign)

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22
Q

Osteogenesis imperfecta

Seen because of thin collagen type1

A

Choroidal plexus

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23
Q

Osteogenesis imperfecta

Other manifestation

A

High probability of bone fracture

Normal dentition but teeth easily fractured if eating hard food

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24
Q

Eheler’s Danlos Syndrome

Cause

A

Defect in collagen type1

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25
Q

Eheler’s Danlos Syndrome

Manifestation

A

Hyperelasticity of skin
Hypermobility of joints
Hyperextensibility of joints

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26
Q

Eheler’s Danlos Syndrome

Types

A

Type 4

Type 1,2,6

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27
Q

Eheler’s Danlos Syndrome

Most dangerous type and affect vasculature (abdominal aneurism)

A

Type 4

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28
Q

Eheler’s Danlos Syndrome

Type 1,2 and 6 manifestation

A

Hyperelasticity

Hypermobility

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29
Q

EPidermolysis Bullosa

Cause

A

Collagen type 7

No production of anchoring fibrils

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30
Q

EPidermolysis Bullosa

What is anchoring fibrils?

A

Connects the epidermis and dermis

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31
Q

EPidermolysis Bullosa

Manifestation

A

Bullae- fluid present between the epidermis and dermis

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32
Q

Achondrodysplasia

Cause

A

Collagen type 2 and 10

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33
Q

Achondrodysplasia

Manifestation

A

Larger head

Short extremities

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34
Q

Achondrodysplasia

Versus dwarfism

A

Low growth hormones

Symmetrical growth

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35
Q

Menke’s disease

Cause

A

Defect in copper transport

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36
Q

Menke’s disease

Bakit included ito sa collagen diseases?

A

Kasi ang copper ay cofactor ng ng lysil oxidase that would link together your fibrils which is excise by your pro collagen peptidase.

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37
Q

Menke’s disease

Manifestations

A

No lysil oxidase
Neurological symptoms
Twisted hair and hypopigmented hair

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38
Q

Menke’s disease

Bakit nagkakatoon ng neurological symptoms?

A

Kailangan ng cytochrome oxidase si copper, at pag walang cytochrome oxidase maapektuhan ang production ng ATP.

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39
Q

Menke’s disease

Bakit nagkakaroon ng twisted hair and hypopigmented hair?

A

Kasi ang copper ay cofactor ng melanin.

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40
Q

Defect in type 4 collagen

A

Glomerulo nephritis

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41
Q

Elastin

Function

A

Skin elasticity

Ang collagen naman, stabilize the skin

So kung walang elastin, magkakaroon ng maraming folds or saggy skin.

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42
Q

Cutis laxa

Cause

A

Deletion of elastin gene

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43
Q

Cutis laxa

Manifestation

A

Looks old for their age

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44
Q

Scleroderma

Cause

A

Excessive production of elastin

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45
Q

Scleroderma

Manifestation

A

Tightening of the skin
Inability to move the skin ( smile patients )
Lesions on the skin

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46
Q

Scleroderma

Complications

A

Dyspagia

Dyspnea

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47
Q

Fibrillin

A

Large glycoprotein

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48
Q

Fibrillin

Component of

A

Microfibrils

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49
Q

Fibrillin

Excreted by

A

Fibroblast

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50
Q

Fibrillin

Found

A

Lens
Periosteum
Aorta

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51
Q

Marfan syndrome

Cause

A

Low fibrillin

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52
Q

Marfan syndrome

Manifestations

A

Cytokine tumor growth factor
Inflammations on the lens, periosteum, aorta
Tall height
Steinberg’s sign

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53
Q

Marfan syndrome

Cytokines growth tumor

A

Kasi nga dumidikit lang si fibrillin kay cytokine, eh madami si cytokin kapag may inflammation. Eh kung low yung fibrillin eh di magkakaroon ng excessive cytokine that lead to tumor

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54
Q

Marfan syndrome

Inflammation of lens, periosteum, aorta

A

Kasi nga dahil sa tumor growth cause by increase cytokine. Kaya nag didislocate yung lens ng mata

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55
Q

Marfan syndrome

Steinberg’s sign

A

Positive

Thumb exceed the 4 fingers

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56
Q

Major glycoprotein of the ECM

No clinical significance

A

Fibronectin

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57
Q

Contains covalently linked Glycosaminoglycans

A

Proteoglycans

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58
Q

Contains a protein portion and long, unbranched polysaccharides

A

Proteoglycans

59
Q

Except for __________, all GAGs contain sulfate groups

A

Hyaluronic acid

60
Q

Classified according to the hexosamine unit

A

Glucosaminoglycans

Galactosaminoglycans

61
Q

Glucosaminoglycans

A

Heparin
Heparin sulfate
Hyaluronic acid

62
Q

Galactosaminoglycans

A

Chondroitin sulfate

Dermatan sulfate

63
Q

Most abundant non collagenous protein in basement membrane.

A

Laminin

64
Q

The _______ is the major site for cell adhesion of laminin

A

G domain

65
Q

Major component of basement membrane

A

Type 4 collagen
Laminin
Nidogen/entactin
Perlecan

66
Q

Major cell attachment factor

A

Nidogen/entacin

67
Q

Heparin sulfate proteoglycan resembling the pearls on a string, they are essential for chrondogenesis

A

Perlecan

68
Q

Major proteoglycan in the cartilage

A

Perlecan

69
Q

Intramolecular desmosine cross links

A

Elastin

70
Q

Intramolecular aldol cross links

A

Collagen

71
Q

Increase production of collagen

A

Keloid, Lung-pulmonary fibrosis, Liver cirrhosis

72
Q

Decrease production of collagen

A

Ehlers-Danlos Syndrome, Osteogenesis Imperfecta, Scurvy

73
Q

Hematuria

A

Defect in lamina and collagen 4

74
Q

Cancer

A

Fibronectin

75
Q

are the most abundant heteropolysaccharides. These molecules are long unbranched polysaccharides containing a repeating disaccharide unit

A

Glycosaminoglycans

76
Q

Provides structural integrity to cells and provides passageways between cells, allowing for cell migration

A

Glycosaminoglycans

77
Q

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

A

Proteoglycans

78
Q

Hyalunoric acid

A

Synovial fluid, connective tissue

79
Q

Chondroitin sulfate

A

Bones

80
Q

Dermatan sulfate

A

Blood vessels

Heart

81
Q

Heparan sulfate

A

Cell structures

82
Q

Heparin

A

Immune system cells

83
Q

Parang chondroitin

Intervertebral disks

A

Keratan sulfate

84
Q

The trisaccharide linker is coupled to the protein core through an _____________to a S residue in the protein

A

O glycosidic bond

85
Q

Linkage of GAGs to protein core

A

two galactose residues and a xylose residue

86
Q

Proteoglycans also called

A

Mucopolysaccharides

87
Q

Gag that do not have two kinds of sugar?

A

Keratan sulfate

88
Q

Gags that not contain sulfate groups

A

Hyaluronic acid

89
Q

Gags na hindi dumidikit sa protein core

A

Hyalunoric acid

90
Q

major component of articular cartilage

A

Aggrecan

91
Q

important to the transparency of the cornea

A

Keratocan

92
Q

a susceptibility factor for bipolar disorder

A

Neurocan

93
Q

more commonly called heparan sulfate proteoglycan of basement membrane

A

Perlecan

94
Q

Defective _________ involved in degradation of one or more drugs

A

Lysosomal hydrolase

95
Q

Hurler syndrome

Cause

A

Deficiency in alpha 1 iduronidase

96
Q

Hurler syndrome

Manifestation

A

Course feature of the face
Potrusion of the abdomen
Bullet shape fingers
Corneal clouding

97
Q

Hunter syndrome

Cause

A

Defect in irudonate sulfatase

98
Q

Hunter syndrome

Manifestation

A

X linked inherited

No corneal clouding

99
Q

Morquio syndrome

Cause

A

Defect in galactosamine 6 sulfatase

100
Q

Morquio syndrome

Manifestation

A

Knocked knees

Wide acetabulum

101
Q

Morateaux lamy

Cause

A

Defect in arylsulfatase B

N acetylgalactosamine 4 sulfate

102
Q

Morateaux lamy

Manifestation

A

Gags dont accumulate in brain

Cox vaga

103
Q

Sly syndrome

Cause

A

Defect in beta glucoronidase

104
Q

Sly syndrome

Manifestation

A

Corneal clouding
Hepatosplenomegaly
Umbilical hernia

105
Q

Organic components of bones

A

Collagen type 1
Osteocalcin
Staloprotein

106
Q

Type of bone cells

A

Osteoblast

Osteoclast

107
Q

Osteoblast

A

Bone formation

Uni cellular

108
Q

Osteoclast

A

Bone resorption

Multi cellular

109
Q

Has a ruffled border, highly proteinated

Bones resorption

A

Osteoclast

110
Q

Bone metabolism regulation

A

PTH

111
Q

Osteoporosis alteration of various factors

A

Hormones
Cytokines
Nutritional

112
Q

Most serious kind of fracture

A

Hip fracture

113
Q

Osteoclastic activity > osteoblastic activity

A

Osteoporosis

114
Q

Osteoblastic activity > osteoclastic activity

A

Osteopetrosis

115
Q

Often due to a deficiency of growth hormone

A

Dwarfism

116
Q

Due to a deficiency of vitamin D during childhood

A

Rickets

117
Q

Due to a deficiency of vitamin D during adulthood

A

Osteomalacia

118
Q

Rickets

Manifestation

A

Curved radius
Curved femur
Curved tibia and fibula

119
Q

Osteomalcia

Manifestation

A

Affects the appendicular skeleton

120
Q

Mutations in the gene encoding FGFR 1 and 2

A

Pfeiffer syndrome

121
Q

Pfeiffer syndrome

Manifestation

A

Exopthalmos
Clover shaped head
Patient die at young age

122
Q

Crouzon syndrome

Cause

A

Mutations in the gene encoding FGFR 2 and 3

123
Q

Crouzon syndrome

Manifestation

A

Exopthalmos

Very small chin

124
Q

Cartilage

Principal protein

A

Type 2 collagen

125
Q

Cartilage

Additional component

A

Type 9 collagen

126
Q

Cartilage

Proteoglycan

A

Aggrecan

127
Q

Cartilage

Avascular tissue

A

Nutrients absorbed via diffusion

128
Q

Clear, odor free, colorless, slightly acidic fluid

A

Eccrine gland

129
Q

Eccrine gland

Composition

A

Sodium chloride
Potassium
Bicarbonate
Almost full of water

130
Q

Thicker
More viscious
Milky consistency

A

Apocrine gland

131
Q

Groin
Axilla
Anal
Areola

A

Apocrine gland location

132
Q

Increase level of sodium chloride in sweat pwedeng pang diagnose ng

A

Cystic fibrosis

133
Q

Pedagra

Gout

A

Monosodium urate crystals

More on alcoholics

134
Q

Pedagra

Pseudogout

A

Calcium pyrophosphate

135
Q

Schirmers test

A

Sjogrens syndrome

136
Q

Clear viscous ultra filtrate of plasma

A

Synovial fluid

137
Q

Synovial fluid glucose level

A

Decrease: inflammation or septic disorder

138
Q

Cerebrospinal fluid compare to plasma

A
Less protein
Less calcium
Less glucose
Carbon dioxide diffuse rapidly
Bicarbonate diffuse slowly
139
Q

Semen glands

A

Copwers
Littre
Prostate
Seminal vesicle

140
Q

Semen vs plasma

A

Less calcium
Less magnesium
High fructose content

141
Q

Formed in the eye

A

Aqueous humor

142
Q

Aqueous humor

Secreted by

A

Cilliary process

143
Q

Aqueous humor

Intraocular pressure

A

Normal

12-20 mmHg

144
Q

Presence of many free fatty acids

A

Psoriasis

145
Q

Defect on skin barrier

A

Atopic dermatitis