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31 Cards in this Set

  • Front
  • Back
What is neuronopathy?
generalized abnormality of the neuron cell body
What is axonopathy?
generalized abnormality of the axon
What is axonal degeneration?
Primary destruction of the axon with secondary disintegration of its myelin sheath.

may be caused by a focal event or axonopathy
What is Wallerian degeneration?
type of axonal degeneration
---faster, usually due to nerve being cut

-the degeneration of the distal portion of the axon that occurs after a focal injury to the axon.

-Shwann cells degrade the myelin and engulf axon fragments.
What is segmental demyelination?
disruption of the meylin sheath due to death or dysfunction of some of the Schwann cells

grows back irregularly
What are some abnormal intracellular accumulations?
lipid - steatosis

protein

glucose

Iron (hemosiderosis and hemochromatosis)

Carbon

Pigments (lipofuscin, melanin, and exogenous)
What are some abnormal tissue accumulations?
Calcification - Ca salts

Amyloidosis

Proteins (misfolded, not normal)
What is Steatosis or Fatty change?
Any accumulation of triglycerides in parenchymal cells.
Where does Steatosis occur?
most often liver

also in heart, skeletal muscles (usually after denervation), other organs
What causes Steatosis?
Toxins

Protein malnutrition

Diabetes mellitus (common in US)

Obesity

Alcohol abuse (common in US)

Anoxia - very low or total depletion of O2
When you have a bunch of nonfunctional proteins and aggregates grouped together, what are 3 possible outcomes?
-Cell Death

-Chaperones - fold and organize the proteins

- Ubiquitin and Proteasome pathway - proteins are degrades and sometimes the peptide fragments can be recycled or used in immune pathways.
What are Mallory bodies/alchoholic hyalin?
Intracellular protein accumulations in liver.
What is Lipofuscin?
Brown pigment granules representing lipid-containing residues of lysosomal digestion.
What is hemochromatosis?
Hemochromatosis is an inherited blood disorder that causes the body to retain excessive amounts of iron. This iron overload can lead to serious health consequences, most notably cirrhosis of the liver
What is hemosiderosis?
a focal or general increase in tissue iron stores without associated tissue damage.
What is calcification?
deposition of calcium salts in a matrix or cells
What are physiological and pathological causes of calcification?
physiological - growth, repair

pathological - Dystrophic and Metastatic
What is Dystrophic Calcification?
local deposition in non-viable or dying tissue
---calcification in tuberculosis lung lesion, damaged heart valves, atheromatous plaques, fat necrosis
What is Metastatic Calcification?
deposition in viable tissue - often widespread

-associated w/ distubance of calcium metabolism (hypercalcemia), like from leukemia
---endocrine disorder
---paraneoplastic syndrom
What is an Amyloid?
A proteinaceous material deposited in the interstitium

--LM appearance - amorphous eosinophilic

--EM - fibrils, non-branching 7.5-10nm wide
What is the molecular structure of an Amyloid?
beta pleated sheets
What are the 3 most common types of amyloid?
AL - light chain

AA

A or possibly ATTR (Alzhemer disease)
What is the onset of Amyloidosis?
begins insidiously (no early signs) and can lead to organ dysfunction - like in kidneys
What is the Pathogenis of amyloidosis that results with AL protein?
-production of abnormal amounts of protein

-Unknown (carcinogen?)

-Monoclonal B-lymphocye proliferation

-plasma cells

-Immunoglobulin Light Chains

-limited proteolysis

-insoluble AL protein
What is the Pathogenis of amyloidosis that results with AA protein?
-production of abnormal amounts of protein

-Chronic Inflammation

-Macrophage activation

-Interleukin 1 & 6

-Liver cells

- SAA Protein

-Limited proteolysis

-Insoluble AA Protein
What is the Pathogenis of amyloidosis that results with A or ATTR protein?
-production of normal amounts of mutant protein

-Mutaion

-Mutant transthyretin

-Aggregation

-Insoluble A or ATTR Protein
---Alzhemer's
What can happen in glomerular amyloidosis?
Kidneys quit filtering and proteins make it out into the urine
What is pressure atrophy?
wasting away of cells/tissue usually due to decreased blood supply

-can occur in any tissue

-in permanent tissue, loss is permanent
---benign tumor of meninges causes loss of cerbral cortex
How does aging affect cells/tissue?
Loss of cells with increased age especially in organs w/ permanent cells
-brain
-cardiac muscle
-skeletal muscle
What are 4 aging theories?
-Progressive accumulation of sublethal injury
---decreased oxidative phosphorylation
---decreased protein synthesis
---intracellular accumulations (lipofuscin, AGE)

-Multifactoral - Telomere shortening
---In stem and germ cells, telomerases restores length of the telomere
---In adult stem cells, the telomere does become shorter gradually

-Clock genes - biological preprogrammed clock

-Wear and tear - (especially free radical damage)
How do telomere length and telomerase activity compare between germ, stem, normal, and cancer cells