Special tissues Flashcards by joseph dizon

Q

Structural proteins

A

Collagen
Elastin
Fibrillin

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Q

Major classes of extracellular matrix

A

Structural proteins
Specialized proteins
Proteoglycans

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Q

Specialized proteins

A

Fibronectin

Laminin

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Q

Most abundant protein in all tissues

A

Collagen

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Q

Collagen approximately how many amino acids?

A

1000 AA

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Q

Amino acid components in collagen

A

Glycine
Hydroxyproline
Proline

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Q

Alanine

A

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

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Q

Glycine

A

Smaller molecule

Twisting of the chain possible

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Q

This amino acid confer the rigidity of collagen molecule

A

Proline and hydroxyproline

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Q

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

A

Hydroxproline

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Q

Collagen synthesis

RER would form your

A

Pro alpha collagen

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Q

Collagen synthesis

A

Study collagen synthesis

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Q

Most important class of collagen

A

Anchoring fibrils

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Q

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

Fibril forming

A

1,2,3,4

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Q

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

Network like

A

4,8,10

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Q

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

FACITs

A

9,12,24,26,29

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Q

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

Beaded filaments

A

6

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Q

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

Anchoring fibrils

A

7

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Q

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

Transmembrane domain

A

13, 17

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Q

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

Others

A

15,17

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Q

Cause of osteogenesis imperfecta

A

Mutation of collagen type 1

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Q

Manifestation of osteogenesis imperfecta

A

Blue gray sclera (pathognomonic sign)

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Q

Osteogenesis imperfecta

Seen because of thin collagen type1

A

Choroidal plexus

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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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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