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
Contains a protein portion and long, unbranched polysaccharides
Proteoglycans
Except for __________, all GAGs contain sulfate groups
Hyaluronic acid
Classified according to the hexosamine unit
Glucosaminoglycans
Galactosaminoglycans
Glucosaminoglycans
Heparin
Heparin sulfate
Hyaluronic acid
Galactosaminoglycans
Chondroitin sulfate
Dermatan sulfate
Most abundant non collagenous protein in basement membrane.
Laminin
The _______ is the major site for cell adhesion of laminin
G domain
Major component of basement membrane
Type 4 collagen
Laminin
Nidogen/entactin
Perlecan
Major cell attachment factor
Nidogen/entacin
Heparin sulfate proteoglycan resembling the pearls on a string, they are essential for chrondogenesis
Perlecan
Major proteoglycan in the cartilage
Perlecan
Intramolecular desmosine cross links
Elastin
Intramolecular aldol cross links
Collagen
Increase production of collagen
Keloid, Lung-pulmonary fibrosis, Liver cirrhosis
Decrease production of collagen
Ehlers-Danlos Syndrome, Osteogenesis Imperfecta, Scurvy
Hematuria
Defect in lamina and collagen 4
Cancer
Fibronectin
are the most abundant heteropolysaccharides. These molecules are long unbranched polysaccharides containing a repeating disaccharide unit
Glycosaminoglycans
Provides structural integrity to cells and provides passageways between cells, allowing for cell migration
Glycosaminoglycans
The majority of GAGs in the body are linked to core proteins, forming
Proteoglycans
Hyalunoric acid
Synovial fluid, connective tissue
Chondroitin sulfate
Bones
Dermatan sulfate
Blood vessels
Heart
Heparan sulfate
Cell structures
Heparin
Immune system cells
Parang chondroitin
Intervertebral disks
Keratan sulfate
The trisaccharide linker is coupled to the protein core through an _____________to a S residue in the protein
O glycosidic bond
Linkage of GAGs to protein core
two galactose residues and a xylose residue
Proteoglycans also called
Mucopolysaccharides
Gag that do not have two kinds of sugar?
Keratan sulfate
Gags that not contain sulfate groups
Hyaluronic acid
Gags na hindi dumidikit sa protein core
Hyalunoric acid
major component of articular cartilage
Aggrecan
important to the transparency of the cornea
Keratocan
a susceptibility factor for bipolar disorder
Neurocan
more commonly called heparan sulfate proteoglycan of basement membrane
Perlecan
Defective _________ involved in degradation of one or more drugs
Lysosomal hydrolase
Hurler syndrome
Cause
Deficiency in alpha 1 iduronidase
Hurler syndrome
Manifestation
Course feature of the face
Potrusion of the abdomen
Bullet shape fingers
Corneal clouding
Hunter syndrome
Cause
Defect in irudonate sulfatase
Hunter syndrome
Manifestation
X linked inherited
No corneal clouding
Morquio syndrome
Cause
Defect in galactosamine 6 sulfatase
Morquio syndrome
Manifestation
Knocked knees
Wide acetabulum
Morateaux lamy
Cause
Defect in arylsulfatase B
N acetylgalactosamine 4 sulfate
Morateaux lamy
Manifestation
Gags dont accumulate in brain
Cox vaga
Sly syndrome
Cause
Defect in beta glucoronidase
Sly syndrome
Manifestation
Corneal clouding
Hepatosplenomegaly
Umbilical hernia
Organic components of bones
Collagen type 1
Osteocalcin
Staloprotein
Type of bone cells
Osteoblast
Osteoclast
Osteoblast
Bone formation
Uni cellular
Osteoclast
Bone resorption
Multi cellular
Has a ruffled border, highly proteinated
Bones resorption
Osteoclast
Bone metabolism regulation
PTH
Osteoporosis alteration of various factors
Hormones
Cytokines
Nutritional
Most serious kind of fracture
Hip fracture
Osteoclastic activity > osteoblastic activity
Osteoporosis
Osteoblastic activity > osteoclastic activity
Osteopetrosis
Often due to a deficiency of growth hormone
Dwarfism
Due to a deficiency of vitamin D during childhood
Rickets
Due to a deficiency of vitamin D during adulthood
Osteomalacia
Rickets
Manifestation
Curved radius
Curved femur
Curved tibia and fibula
Osteomalcia
Manifestation
Affects the appendicular skeleton
Mutations in the gene encoding FGFR 1 and 2
Pfeiffer syndrome
Pfeiffer syndrome
Manifestation
Exopthalmos
Clover shaped head
Patient die at young age
Crouzon syndrome
Cause
Mutations in the gene encoding FGFR 2 and 3
Crouzon syndrome
Manifestation
Exopthalmos
Very small chin
Cartilage
Principal protein
Type 2 collagen
Cartilage
Additional component
Type 9 collagen
Cartilage
Proteoglycan
Aggrecan
Cartilage
Avascular tissue
Nutrients absorbed via diffusion
Clear, odor free, colorless, slightly acidic fluid
Eccrine gland
Eccrine gland
Composition
Sodium chloride
Potassium
Bicarbonate
Almost full of water
Thicker
More viscious
Milky consistency
Apocrine gland
Groin
Axilla
Anal
Areola
Apocrine gland location
Increase level of sodium chloride in sweat pwedeng pang diagnose ng
Cystic fibrosis
Pedagra
Gout
Monosodium urate crystals
More on alcoholics
Pedagra
Pseudogout
Calcium pyrophosphate
Schirmers test
Sjogrens syndrome
Clear viscous ultra filtrate of plasma
Synovial fluid
Synovial fluid glucose level
Decrease: inflammation or septic disorder
Cerebrospinal fluid compare to plasma
Less protein Less calcium Less glucose Carbon dioxide diffuse rapidly Bicarbonate diffuse slowly
Semen glands
Copwers
Littre
Prostate
Seminal vesicle
Semen vs plasma
Less calcium
Less magnesium
High fructose content
Formed in the eye
Aqueous humor
Aqueous humor
Secreted by
Cilliary process
Aqueous humor
Intraocular pressure
Normal
12-20 mmHg
Presence of many free fatty acids
Psoriasis
Defect on skin barrier
Atopic dermatitis