Special tissues Flashcards
Structural proteins
Collagen
Elastin
Fibrillin
Major classes of extracellular matrix
Structural proteins
Specialized proteins
Proteoglycans
Specialized proteins
Fibronectin
Laminin
Most abundant protein in all tissues
Collagen
Collagen approximately how many amino acids?
1000 AA
Amino acid components in collagen
Glycine
Hydroxyproline
Proline
Alanine
Bigger molecule
Create a bulkier chain
No room for hydroxyproline and proline to twist
Glycine
Smaller molecule
Twisting of the chain possible
This amino acid confer the rigidity of collagen molecule
Proline and hydroxyproline
AA that helps form water bridges that stabilize the triple helix at body temperature?
Hydroxproline
Collagen synthesis
RER would form your
Pro alpha collagen
Collagen synthesis
Study collagen synthesis
Most important class of collagen
Anchoring fibrils
Give the type of collagen that match to this class of collagen.
Fibril forming
1,2,3,4
Give the type of collagen that match to this class of collagen.
Network like
4,8,10
Give the type of collagen that match to this class of collagen.
FACITs
9,12,24,26,29
Give the type of collagen that match to this class of collagen.
Beaded filaments
6
Give the type of collagen that match to this class of collagen.
Anchoring fibrils
7
Give the type of collagen that match to this class of collagen.
Transmembrane domain
13, 17
Give the type of collagen that match to this class of collagen.
Others
15,17
Cause of osteogenesis imperfecta
Mutation of collagen type 1
Manifestation of osteogenesis imperfecta
Blue gray sclera (pathognomonic sign)
Osteogenesis imperfecta
Seen because of thin collagen type1
Choroidal plexus
Osteogenesis imperfecta
Other manifestation
High probability of bone fracture
Normal dentition but teeth easily fractured if eating hard food
Eheler’s Danlos Syndrome
Cause
Defect in collagen type1
Eheler’s Danlos Syndrome
Manifestation
Hyperelasticity of skin
Hypermobility of joints
Hyperextensibility of joints
Eheler’s Danlos Syndrome
Types
Type 4
Type 1,2,6
Eheler’s Danlos Syndrome
Most dangerous type and affect vasculature (abdominal aneurism)
Type 4
Eheler’s Danlos Syndrome
Type 1,2 and 6 manifestation
Hyperelasticity
Hypermobility
EPidermolysis Bullosa
Cause
Collagen type 7
No production of anchoring fibrils
EPidermolysis Bullosa
What is anchoring fibrils?
Connects the epidermis and dermis
EPidermolysis Bullosa
Manifestation
Bullae- fluid present between the epidermis and dermis
Achondrodysplasia
Cause
Collagen type 2 and 10
Achondrodysplasia
Manifestation
Larger head
Short extremities
Achondrodysplasia
Versus dwarfism
Low growth hormones
Symmetrical growth
Menke’s disease
Cause
Defect in copper transport
Menke’s disease
Bakit included ito sa collagen diseases?
Kasi ang copper ay cofactor ng ng lysil oxidase that would link together your fibrils which is excise by your pro collagen peptidase.
Menke’s disease
Manifestations
No lysil oxidase
Neurological symptoms
Twisted hair and hypopigmented hair
Menke’s disease
Bakit nagkakatoon ng neurological symptoms?
Kailangan ng cytochrome oxidase si copper, at pag walang cytochrome oxidase maapektuhan ang production ng ATP.
Menke’s disease
Bakit nagkakaroon ng twisted hair and hypopigmented hair?
Kasi ang copper ay cofactor ng melanin.
Defect in type 4 collagen
Glomerulo nephritis
Elastin
Function
Skin elasticity
Ang collagen naman, stabilize the skin
So kung walang elastin, magkakaroon ng maraming folds or saggy skin.
Cutis laxa
Cause
Deletion of elastin gene
Cutis laxa
Manifestation
Looks old for their age
Scleroderma
Cause
Excessive production of elastin
Scleroderma
Manifestation
Tightening of the skin
Inability to move the skin ( smile patients )
Lesions on the skin
Scleroderma
Complications
Dyspagia
Dyspnea
Fibrillin
Large glycoprotein
Fibrillin
Component of
Microfibrils
Fibrillin
Excreted by
Fibroblast
Fibrillin
Found
Lens
Periosteum
Aorta
Marfan syndrome
Cause
Low fibrillin
Marfan syndrome
Manifestations
Cytokine tumor growth factor
Inflammations on the lens, periosteum, aorta
Tall height
Steinberg’s sign
Marfan syndrome
Cytokines growth tumor
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
Marfan syndrome
Inflammation of lens, periosteum, aorta
Kasi nga dahil sa tumor growth cause by increase cytokine. Kaya nag didislocate yung lens ng mata
Marfan syndrome
Steinberg’s sign
Positive
Thumb exceed the 4 fingers
Major glycoprotein of the ECM
No clinical significance
Fibronectin
Contains covalently linked Glycosaminoglycans
Proteoglycans