CT ECM Flashcards
1
Q
What is ECM?
A
- Composed
- Ground substance (produced by Fibroblasts)
- Fibers
- Collagen
- Reticular
- Elastic
- Tissue Fluids

2
Q
What is Ground Substances in ECM?
A
- Contain
- Glycosaminoglycans (GAGs)
- Proteoglycans (PG)
- Adhesive glycoproteins
- Proteolytic Enzymes
- Degradation
3
Q
What is Glycosaminoglycans (GAGs)?
A
- Part of Ground Substances
- Formerly called MUCOPOLYSACCHARIDES
- Long-chained polysacchrides
- Sugar used
* HEXOSAMINE (glycosamine) - Types
- Sulfated GAGs (synthesized in rER and Golgi)
- Heparan sulfate
- Dermatan sulfate
- Keratan sulfate
- Chondroitin 4-sulfate
- Chondroitin 6-sulfate
- Non-Sulfated GAGs
- Hyaluronic Acid
- No protein core
- Hyaluronic Acid
- Negatively charged
- Osmotically attract water to ECM
- Attracts Na+ ions

4
Q
What is Proteoglycans?
A
- Proteins modified by Glycosaminoglycan (GAG)
- Core Protein present
- Resist Compression

5
Q
What is Hurler Disease?
A
- Mucopolysaccharidosis type 1, MPS 1
- Lysosomal storage disease
- Altered Ground Substance
6
Q
What is Deficiency of L-iduronidase?
A
- Stop degradation of Heparan and Dermatan Sulfate
* Ground Substance related
7
Q
What is Staphylococcus aureus infection?
A
- Hyaluronidase
- Cleaves Hyaluronic acid
* Rapid spread of bacteria into tissue
8
Q
What is Osteoarthritis?
A
- Degradation of Proteoglycan
* Aggrecan - Cartilage breakdown (erosion)
9
Q
What are Glycoproteins?
A
- Contain protein moiety attached to carbohydrates
- Hold tissue structures together
- Ex
- Fibronectin
- Binding site for ECM and Integrin
- Laminin
- Binds basal lamina to epithelial cells
- Others
- Osteonectin
- Binds Ca hydroxyapatite to collagen I
- Chondronectin
- Binding sites for Collagen II
- Entactin
- Component of basement membrane
- Tenascin
- In embryonic tissue
- For cell migration

10
Q
What are Integrins?
A
- Linker proteins that bind to
- Collagen
- Fibronectin
- Laminin
- Role
- Cell adhesion
- Cell movement and migration
- Wound Healing
11
Q
What is Collagen 1?
A
- Synthesizing Cells
- Fibroblast
- Osteoblast
- Odontoblast
- Cementoblast
- Function
* Resist Tension - Location
- Dermis
- Tendon
- Ligaments
- Bone
- Dentin
- cementum
12
Q
What is Collagen 2?
A
- Synthesizing Cells
* Chondroblasts - Function
* Resists Pressure - Location
- Hyaline cartilage
- Elastic cartilage
13
Q
What is Collagen 3?
A
- Synthesizing Cells
- Fibroblast
- Reticular cells
- Smooth muscle cells
- Hepatocyte
- Function
- Structural framework of
- Spleen
- Liver
- Lymph nodes
- Smooth muscle
- Adipose tissue
- Location
- Lymphatic system
- Spleen
- Liver
- Cardiovascular system
- Lungs
- Skin
14
Q
What is Collagen IV?
A
- Synthesizing Cells
- Epithelial cells
- Muscle cells
- Schwann cells
- Function
- Forms meshwork of Lamina Densa of Basal Lamina
- Provide support and filtration
- Location
* Basal Lamina
15
Q
What is Collagen V?
A
- Synthesizing cells
- Fibroblast
- Mesenchymal cells
- Function
* Associated with Type I collagen and placental ground substance - Location
- Dermis
- Tendon
- Ligaments
- Placenta
16
Q
What is Collagen VII?
A
- Synthesizing cells
* Epidermal cells - Function
* Forms anchoring fibrils - Location
* Junction of epidermis and dermis
17
Q
What are Reticular Fibers?
A
- Type III collagen fibrils
- Prominent in Loose CT
- Found
* Lamina reticularis (basement membrane) - Role
- Endocrine glands
- Liver
- Hemopoietic tissue
- Lymphoid organs
- Thymus
- Does NOT stain with H&E
* Stain with SILVER SALTS and PERIODIC ACID-SCHIFF (PAS)

18
Q
What are Elastic Fibers?
A
- Do not form Banding Pattern
- Composed
- Elastin protein
- Fibrillin microfibrils
- Rich in GLYCINE, PROLINE, and LYSINE
- Stain
- Yellowish color in gross tissue
- Do NOT stain with H&E
- Dye: ORCEIN (BLACK)

19
Q
What is Intestitital Pulmonary Fibrosis?
A
- Overproduction of Fibroblasts
- Swelling and scarring of alveoli
- First appears during exercise
- Dry cough
- Clubbing (Enlarged fingertips)
20
Q
What is Keloid?
A
- Excessive accumulation of collagen during wound healing
21
Q
What is Ehlers-Danlos Syndrome?
A
- Abnormal synthesis and cross linking of TROPOCOLLAGEN
- Hyperextensive skin
- Severe Bruising
- Common condition in Contortionists

22
Q
What is Chondrodysplasias?
A
- Mutation in Type 2 collagen
- Bone and Joint deformities
23
Q
What is Osteogenesis Imperfecta?
A
- “Brittle Bones”
- Mutation in Type 1 Collagen
* Failure to assemble triple helix - “Child Abuse” mistaken
- Characteristics
- Multiple bone fractures
- Defective teeth
- Deafness
- Heart Valve disorder
- Blue Sclera
*Type 2 OI = Death
*Type 3 OI = Severe and progressive
24
Q
What is Scurvy?
A
- Vitamin C deficiency
- Defective HYDROXYLATION OF PROLINE
- Unstable alpha chain helices
- Characteristics
- Bleeding gums
- Edema
- Hemorrhage of mucous membrane
- Failure of wound healing
25
What is Marfan Syndrome?
1. Defect in Fibrillin-1 protein
2. Defective elastic fivers
3. Characteristics
* Tall
* Long limbs
* Long thin fingers
* Dislocation of lens
* HEART defects (mitral valve prolapse)
* Spontaneous pneumothorax
* Pectus excavatum