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MOD 1: DR > Chapter 2: starting at intracellular accumilation > Flashcards

Chapter 2: starting at intracellular accumilation Flashcards

1
Q

proteins can accumulate in cells d/t what

A

Defect in protein folding/trasnposrt

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2
Q

Intracellular accumulation of proteins usually appear as what (morphology)?

A

Rounded, eosinophilic droplets, vacuoles, or aggregates in the cytoplasm

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3
Q

Chronic proteinuria can cause what?

A

Increase in filtration of proteins by glomerulus => accumulation of resorption droplets of proteins in the proximal tubules of the kidneys => proteins look like pink hyaline droplets

If proteinuria decreases, it is reversible

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4
Q

What happens when cells are making proteins in large amounts?

A

ER becomes distended => produces large homegenous eosinophillic inclusions called Russl bodies**

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5
Q

What is an example of a disease with defective intracellular transport and secretion of proteins?

A

α1-antitrypsin deficiency; mutations in the protein => slow folding => buildup of partially folded intermediates in ER of the liver that are not secreted because they do not go to the Golgi =>

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6
Q

Diseases that cause problem with intracellular transport and secretion of proteins cause pathology how?

A
  1. Unfolded protein response
  2. Apoptosis d/t ER stress
  3. Loss of protein function
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7
Q

How does a1 antrypsin deficieny manifest itslf?

A

Emphysema. – decrease in circulating α1-antitrypsin

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8
Q

Accumulation of CYTOSKELETON proteins are hallmarks in cell injury. What 2 proteins can accumulate?

A
  1. Keratin intermediates => coalesce into cytoplasmic eosinophilic inclusion called alcoholic hyaline that occurs in alcoholic liver disease.
  2. Neurofilament intermediates can can coaslese into neurofibrillary tangles in Alzheimers
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9
Q

Aggregation of abnormal or misfolded protein deposit where

A

intracellulary

extracellularly or both

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10
Q

Aggregation of abnormal proteins are called what? Give an exmaple of one

A

Proteinopathies or protein-aggregation diseaase.

Ex. Certain kinds of amyloidosis

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11
Q

what kinds of lipids can accumulate in cells and which is the most common

A

TAGS* msot common
Cholesterol and cholesterol esters
Phospholipids

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12
Q

What is steatosis?

A

accumulation of TAGS in parachymal cells, most often the liver. But also heart, skeletal muscle and kidney.

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13
Q

Intracellular accumulation of cholesterol within macrophages is also characteristic of acquired and hereditary hyperlipidemic states is called what?

Found where?

A
  • Xanthomas (clusters of foamy cells)

- Subepithelial CT of the skin and tendons

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14
Q

The focal accumulations of cholesterol-laden macrophages in LP of the gallbladder is referred to what?

A

Cholesterolosis

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15
Q

What is the name of a lysosomal storage disease caused by mutations affecting enzyme involved in cholesterol trafficking, resulting in cholesterol accumulation in organs?

A

Niemann-Pick type C disease

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16
Q

In atherosclerotic plaques, smooth muscle cells and macrophages within the intimal layer of the aorta and large arteries are filled with?

A

Lipid vacuoles that are mostly composed of cholesterol and cholesterol esters

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17
Q

Extracellular cholesterol esters from ruptured arthersclereos may crystallize in the shape of?

A

Long needles, producing distinct clefts in tissue sections

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18
Q

In α1-antitrypsin deficiency may cause what in the hepatocytes?

What in the lungs?

A
  • Storage of non-functional protein in hepatocytes causing apoptosis
  • Absence of enzymatic activity in lungs causes destruction of elastic tissue —-> emphysema
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19
Q

What are hyaline changes?

A

DESCRIPTIVE HISTOLOGIC TERM (NOT A MARKER FOR CELl NJRY) that is any change that occurs IN or OUTSIDE the cell that makes it look homogenous and glassy on H/E stain. Does not rep a specific pattern of acumm

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20
Q

Examples of hyaline changes

A
  1. Russl Bodies
  2. Alcoholic hyaline
  3. Resorption of droplets
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21
Q

When does extracellular hyaline occur?

A

DM or chronic HTN: the walls of the arteroles (kidneys) become hyalinzed d/t protein deposition and deposition of BM material

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22
Q

Glycogen can lead to massive accumulation cause cell injury/ death.

What to they look like in hte cytoplasm and when can we best see it?

A

Clear vaculoes in cytoplasm

We see it best in tissuses fixed in absolte alcohol.

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23
Q

•_________ makes glycogen look rose/violet.

A

Best carmine or the PAS Reactio

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24
Q

When do we see glycogen accum

A

DM

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25
Q

What is the most common exogenous pigment?

Accumulations of this tissue in the lung is called what?

A
  • Carbon (coal dust) from pollution or carbon dust: picked up by MO => LN
  • Anthracosis (blackend lung)
26
Q

carbon dust can cause what in coal mines

A

• This causes fibroblastic emphysema that is known as coal worker’s pneumoconiosis.

27
Q

What are tattoo pigments?

A

pigemnts that NTR skin, are phagocytozied by dermal MO and live there for rest of life but do NOT cause inflammatory responpe

28
Q

What is an insoluble pigment produced from wear-and-tear? when do we see it?

A

Lipofuscin (AKA lipochrome or wear-and-tear pigment) that is physiologically seen with aging that have undergone repeated damage

29
Q

What is of a yellow-brown color and is a tell-tale sign of free radical injury and lipid peroxidation?

A

Lipofuscin (AKA lipochrome or wear-and-tear pigment)

*Physiologically seen with aging

30
Q

What is the only endogenous brown-black pigment?

What is the only other that could be considered in this category and is a result of a rare disase?

A
  • Melanin
  • Homogenistic acid, which occurs in pt’s with alkaptonuria, a rare metabolic disease, where the pigment deposits in skin, CT, and cartilage and is called ochronosis
31
Q

What is of a golden yellow-to-brown, granular or crystalline pigment?

Major storage form of what?

A
  • Hemosiderin, which is derived from Hb and a major storage form of iron
  • Iron
32
Q

When there is local or systemic excess of iron, what will form hemosiderin granules?

A
  • Ferritin

- Hemosiderin pigment are aggregates of ferritein micelles when there is local or system excess of iron

33
Q

What are the major causes of hemosiderosis (deposited in organs and tissues)?

A
  • Increased absorption of dietary iron due to inborn error of meabolism, hemochromatosis
  • Hemolytic anemia, premature lysis of RBC’s leads to release of increased iron
  • Repeated blood transfusions, transfused red cells constitute an exogenous load of iron
34
Q

Local overload of iron causes hemosideron to be where?

A

Cells, like in bruises

35
Q

Systemic overload causes deposition of hemosiderin where?

A

organs (hemosiderosis)

36
Q

What is the abnormal deposition of calcium salts, together with iron and Mg called?

A

Pathologic calcification

37
Q

What kind of calcification occurs locally in dying tissues with normal serum levels of calcium and no problems with Ca2+ metabolism?

Frequently occurs where?

A

Dystrophic calcification

Found: areas of necrosis (coag, caseous, or liquefactive), Aging or damaged heart valves, AND AREAS OF FAT SAPONIFICATION

38
Q

___________ are almost always found in the atheromas of advanced atherosclerosis.

A

dystrophic calcification

39
Q

_______ may accentuate dystrophic calcification but does not cause it

A

hypercalcemia

40
Q

Deposition of calcium salts in otherwise normal tissues is what that is almost ALWAYS THE RESULT OF HYPERCALCEMIA SECONDARY TO disturbance in Ca2+

Results from what?

A

Metastatic calcification

  • Hypercalcemia secondary to disturbance in calcium metabolism/homeost
41
Q

Hypercalemia can be d/t what

A

Increased bone resorption d/t:

  1. Increased levels of PTH from hyperparathyroidism
  2. Bone tumors
  3. Pagets dz
  4. Vit D disorder
  5. Immbolisation
  6. renal failure
42
Q

Where does metastatic calcification most often occur?

A

Gastric mucosa, kidneys, lungs, systemic arteries, pulmonary vein

all of these areas excrete acid and have an internal alkaline compartment that predisposes them to metastatic calcification

43
Q

____ is one of the strongest risk factors of chronic dis

A

age

44
Q

• What leads to cellular aging?

A

ROS (mutations), loss of DNA repair, telomere shortening (decreased cellular replication), and defective protein homeostasis (decreased cellular functions), Werners, Bloom and ataxia-telangiectasia

45
Q

What counteracts cellular aging?

A

o Decreased insulin/IGF signaling and decreased TOR leads to altered transcription and increased DNA repair and increased protein synthesis.

46
Q

Patients with Werner syndrome show what?

Defective gene is what?

A
  • Premature aging

- DNA helicase => rapid accum of chromosomal damage

47
Q

Bloom syndrome and ataxia-telangiectasia are caused by a mutation in protein that does what?

A

Repairs double strand breaks

48
Q

What is replicative senescence?

A

All normal cells have a limited capacity for replication, and after a fixed number of divisions cells become arrested in a terminally nondividing state

49
Q

Telomere attrition can cause cellular aging.

how does this occur?

A

When somatic cells replication, a small amount of the telomere is not replicated nad is lost => gets shorter and shorter => then arrests cell cyle

50
Q

How does telomerase work and how does it function in cancer cells?

A

A specialized RNA-protein complex that uses its own RNA as a template for adding nucleotides to the ends of chromosomes

  • Expressed in germ and stem cells , but NOT expressed in somatic cells, but is reactivated in cancer cells and allows them to proliferate indefinitely
51
Q

What is the telomerase like in germ, stem, and somatic cells and why is this important?

A
  • Expressed in germ cells, and in low levels in stem cells
  • Not expressed in somatic cells
  • As somatic cells age their telomeres become shorter and they eventually exit the cell cycle
52
Q

What does the CDKN2A locus encode for?

Controls what phase progression of the cell cycle?

A
  • p16 (INK4a): protects cells from uncontrolled mitogenic signals and pushes cells along senescence pathway
  • G1 to S
  • Involved in controlling replicative senscence
53
Q

Protein homeostasis: maintain proteins in

A

correctly folded confirmations (chaperones) and degrades misfolded proteins in the autophagy-lysosome/ub-proteosome sys

54
Q

o Normal folding and degradation of abnormally folded proteins ______ with age.

A

decreases

55
Q

o The expression of ____ proteins are positively correlated with longevity.

A

chaperones

56
Q

What suggests that agining is assocuation with nutrition and metabolism?

A

Calorie restistriction increases life span

57
Q

What pathways contribute to calorie restriction increasing out lifepsance?

A
  1. Insulin and IGF-1 pathway

2. Sirtuins =>

58
Q

What are sirtuins?

A

family of NAD-dep deacteylases that promote expressing of genes that promote longetivity by inhibiting metabolic actibvity, reducing apotosis, + protein folding and inhibit ROS. Also nicrease insulin sensitivity and glucose metaboism

59
Q
  1. Insulin and IGF-1 pathway

does what

A
  • Informs the cell about the availability of glucose and promotes cell growth and replication – Anabolic state
  • Activates 2 kinases: AKT and AKT’s downstream target mTOR

Calorie restriction inhibits this pathway.

60
Q

What can mimick calorie restriction to turn off insulin and IGF-1 path?

A

Rapamycin

MOD 1: DR (11 decks)

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