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General Pathology > Extracellular matrix > Flashcards

Extracellular matrix Flashcards

1
Q

Processes influenced by ECM

A

-Embryonogenesis
-Fibrosis
-Wound healing
-Cell behavior
-Carcinogenesis

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2
Q

Marfan syndrome

A

Mutation in gene for fibrillin-1 which regulates formation of elastic fibers
Also get increased levels of TGF-beta

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3
Q

Features of Marfan syndrome

A

Tall slender build, with long fingers and toes. Lens dislocation, and aortic weakening (think Abe Lincoln)

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4
Q

Structural components of ECM

A

-Collagen
-Elastin

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5
Q

Ehler-Danlos Syndrome

A

Inherited CT disorders with defective collagen synthesis
-Dermatosporaxis is a variant of this condition

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6
Q

Absorptive components of ECM

A

-Glycoaminoglycans
-Proteoglycans

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7
Q

Edema and proteoglycans

A

Edema is excessive accumulation of fluid in the ECM within body cavity
-Changes in concentration or properties of absorptive molecules in ECM can cause edema

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8
Q

Adhesive components of ECM

A

-Fibronectin
-Laminin
-Others

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9
Q

Fibronectin and wound healing

A

Fibronectin is critical for wound healing
-Binding sites for macrophages, endothelium, and fibroblasts
-Formation of granulation tissue matrix
-Influence cell function and protein expression

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10
Q

Other roles of fibronectin

A

-Cell migration and differentiation during embryogenesis
-Hemostasis
-Carcinogenesis

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11
Q

General patterns of injury to the ECM

A

-Increased destruction
-Decreased production
-Excessive production
-Deposition of abnormal substances

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12
Q

What produces collagen

A

Fibroblasts

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13
Q

What enzymes degrade collagen

A

MMP or collagenase

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14
Q

Causes of increased destruction of ECM

A

-Excessive MMP activity
-Inflammation
-Immunologic reactions

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15
Q

Morphology of increased destruction of ECM

A

-Corresponds to the different types of necrosis

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16
Q

Histologic morphology of increased destruction of ECM

A

-Collagen swollen, uniformly eosinophilic, fragmented or absent
-Inflammation usually present

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17
Q

Causes of decreased production of ECM

A

-Most are inherited diseases (collagen dysplasia)
-Nutritional deficiencies like Vitamin C

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18
Q

Decreased production of ECM morphology

A

-Skin distensible and fragile
-Tears in skin

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19
Q

Histologic morphology of decreased production of ECM

A

-No abnormalities in some cases
-Collagen of variable size and shape
-Abnormal organization

20
Q

Excessive production of ECM

A

-Associated with healing in irreversibly damaged tissue (fibrosis and scar formation)
-Excessive fibrosis can interfere with tissue and organ function

21
Q

Causes of excessive production ECM

A

-Upregulation of collagen-producing cells (mainly fibroblasts)
-Stimuli resulting in irreversibly damaged tissue (tissue replaced as part of healing)

22
Q

Morphology of Excessive production of ECM

A

-Dense, white, fibrous tissue replacing normal tissues and organs
-Abnormal relationships between tissues and organs

23
Q

Histologic morphology of excessive ECM

A

-Densely packed collagen
-Initially increased fibroblasts
-Later mostly collagen and few fibroblasts

24
Q

Common substances deposited in the ECM

A

-Amyloid
-Ca
-Fe
-Excessive collagen

25
Q

Amyloid

A

Beta pleated sheet protein. Can get deposited in ECM due to abnormal folding of amyloid proteins
-Can’t be degraded in ECM if misfolded

26
Q

Reasons for deposition of amyloid

A

-Normal proteins that misfold when produced in excess
-Abnormal proteins formed due to mutation

27
Q

Morphology of amyloid deposition

A

Deposition either locally or systemically
-Organs enlarged, firm and discolored
-Detect with Lugol’s iodine and sulfuric acid

28
Q

Histologic morphology of amyloid deposistion

A

-Eosinophilic, acellular, amorphous, fibrilllar extracellular protein displaces normal tissue
-Stain with Congo red and turns apple green in polarized light

29
Q

Primary amyloidosis

A

-Associated with immunological dyshomeostasis and deposition of AL protein
-Associated with plasma cell neoplasia that produce excessive immunoglobulin and immunoglobulin light chains

30
Q

Secondary amyloidosis

A

-Associted with chronic inflammation and tissue destruction with deposistion of AA protein
-Cytokines and cell damage associated with chronic inflammation increase SAA and abnormal breakdown of these results in deposition of ECM

31
Q

Amyloidosis of aging

A

-AB amyloid occur in brain of older animals (alzheimers)
-Less often occurs in heart, GI tract, and lungs of older dogs

32
Q

Endocrine amyloidosis

A

-Amyloid deposited in pancreas of cat in the islets
-Common cause of type I diabetes mellitus

33
Q

Familial amyloidosis

A

Systemic deposition of AA occurs in some breeds and cats. SAA may be abnormal but usually still see chronic inflammatory component. Kidney and liver most commonly affected

34
Q

Deposition of calcium

A

Can occur in normal or abnormal situation

35
Q

Causes of Calcification

A

-Dystrophic
-Metastatic

36
Q

Dystrophic calcification

A

-In necrotic tissue
-Ca binds to damaged membrane phospholipids and starts crystal formation
-Localized event

37
Q

Common locations of dystrophic calcification

A

-Heart
-Skeletal muscle
-Inflammatory lesions
-Skins

38
Q

Metastatic calcification

A

In normal tissue
-Reflection of usually hypercalcemia
-Ca initiated by local alkalinity and alteration in collagen structure
-Systemic

39
Q

Situation of metastatic calcification

A

-Renal failure (excess PTH, Dont see hypercalcemia)
-Vitamin D toxicity
-Pseudohyperparathyroidism
-Neoplastic bone destruction

40
Q

Morphology of calcification

A

-Gross: gritty and white depsosits
-Histologic: Purple material either in normal structures or disrupting the normal morphology

41
Q

Epidermolysis bullosa

A

COnditions with skin fragility and blistering/ Abnormal collagen type VII, keratin, laminin, and integrens

42
Q

Osteogenesis imperfecta

A

Abnormal type I collagen

43
Q

Muscular dystrophies

A

Defects in laminin or type VI collagen and decreased dystrophin levels

44
Q

Osteoarthritis and IVDD

A

Degeneration of multiple matrix components

45
Q

Vascular diseases

A

-Atherosclerosis
-Aneurysm

General Pathology (30 decks)

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