Packet 4 (page 12-27, Quiz 3) Flashcards
(122 cards)
1
Q
What is the other name for heat shock proteins (HSP’s)?
A
chaperone proteins
2
Q
What are heat shock proteins (HSP’s)/ chaperone proteins?
A
-a protein that aids the assembly and folding of other protein molecules in living cells
-found in the ER where protein synthesis occurs
3
Q
What are chaperonopathies?
A
-disorders relating to chaperones- either genetic or acquired
-will result in a misfolding or degradation of proteins, this is called prion diseases
-will have proteinaceous infectious particles and result in spongiform encephalopathies (holes in brain, neurodegenerative, no cure, fatal within a few months)
4
Q
What is an example of genetic chaperonopathies?
A
charcot-marie-tooth disease (peripheral neuropathy)
5
Q
What is charcot-marie-tooth disease?
A
-genetic chaperonopathy
-it is a type of peripheral neuropathy and it affects peripheral nerves and ascending tracts
6
Q
peripheral neuropathy is common in which individuals?
A
diabetics
7
Q
What is an example of an acquired chaperonopathy?
A
mad cow disease, also known as Creutzfeldt-Jakob disease (CJD)
8
Q
How does someone acquire Creutzfeldt-Jakob disease?
A
-eating cows that are contaminated with mad cow disease
-getting a cornea transplant
either of these will cause issues in heat shock proteins/chaperone proteins
9
Q
Enzymes are normally ___________ and ______ conc. in the blood
A
intracellular, LOW
(so when enzymes are released/leaked into the blood this indicates there is cell damage, cell death, hypoxia, and/or intracellular toxicity)
10
Q
Which enzymes would be elevated in the blood if there was heart damage?
test q
A
-AST
-CK-MB/CK2
-LDH
11
Q
Which enzymes would be elevated in the blood if there was liver damage?
test q
A
-ALT
-AST
-alkaline phosphatase
-LDH
-SGGT
12
Q
Which enzymes would be elevated in the blood if there was pancreatic damage?
test q
A
amylase and lipase
13
Q
Which enzymes would be elevated in the blood if there was skeletal muscle damage?
test q
A
-CK-MM/CK3
-LDH
-AST
14
Q
Which enzymes would be elevated in the blood if there was bone damage?
A
alkaline phosphatase
15
Q
What does CK or CPK stand for?
A
creatine kinase
16
Q
Where is CK1 found?
A
brain
17
Q
Where is CK-MB/CK2 found?
A
cardiac muscle/heart
18
Q
Where is CK-MM/CK3 found?
A
skeletal muscles
19
Q
What does AST/SGOT stand for?
A
aspartate aminotransferase/ serum glutamic oxalacetic transaminase
20
Q
What does ALT/ SGPT stand for?
A
alanine aminotransferase/ serum glutamic pyruvic transaminase
21
Q
What does LD/LDH stand for?
A
lactic dehydrogenase
22
Q
What does ALP stand for?
A
alkaline phosphatase
23
Q
Which enzyme in the blood is high if the patient is an alcoholic?
A
SGGT (serum gamma glutamyl transpeptidase)
24
Q
Cell death occurs when injury becomes irreversible and cells cannot recover. There are 2 types of cell death. What are they?
A
necrosis and apoptosis
25
What is necrosis?
-cell and organelle swelling
-early plasma membrane rupture and the spilling of cellular material into tissue with subsequent inflammation
-cell explodes and dies
26
What is apoptosis?
-programmed early and coordinated cell death
-Greek for "dropping off", falling away
-used to eliminate unwanted cells
-rids the body of cells that have been damaged beyond repair
-cell shrinks and dies
-a function of normal and pathological tissue changes
-its an active process of cell self destruction
-plays a role in preventing cancer
27
Huntingtons disease is where theres too many CAG repeats. What nucleus of the basal ganglia undergoes apoptosis?
caudate nucleus
28
Intracellular changes in cell death include:
-severe membrane damage
-intracellular proteins are denatured
-lysosomes leak enzymes and digest organelles
-DNA breakdown
-there are 3 changes to the nucleus due to the breakdown of DNA. What are these 3 changes?
1) pyknosis (stained dark, clumped)
2) karyorrhexis (fragmented nucleus)
3) karyolysis (no more evidence of nucleus)
29
What is pyknosis?
-irreversible shrinkage or condensation of chromatin in the nucleus of a cell undergoing necrosis or apoptosis
-DNA becomes a solid shrunken mass
-the nucleus stains blue and is basophilic
30
What is karyorrhexis?
-the destructive fragmentation of the nucleus of a dying cell
-referred to as "nuclear dust"
-chromatin is distributed irregularly throughout the cytoplasm
31
What is karyolysis?
complete dissolution of the chromatin of a dying cell due to enzymatic degradation
32
What is an apoptotic body?
-formation of membrane bound near-spherical bodies containing condensed organelles around the cell
-the last stage of apoptosis when the DNA has fragmented and the cytoskeleton has dissolved
33
Apoptotic cells are round-oval shrunken masses of intensely __________________________ containing shrunken or almost normal organelles
eosinophilic (stains blue) cytoplasm
34
What are the 5 functions of apoptosis?
1) role in embryonic development (ex: tadpole tails, neurological connections, webbing)
2) elimination of "old" cells (ex: crypts in SI, postmenopausal (atrophy of endometrium), bone cells dying during turnovers)
3) deletion of mutant cells (recognizes damaged DNA from whatever reason (UV, chemicals, autoreactive T cells in thymus)
4) defense against the spread of infections (ex: viruses contain extrachromosomal DNA and recognized as intruders)
5) many enzymatic cascade systems are involved
35
What are the 4 apoptosis recognition signals?
1) nuclear condensation
2) segregation of cellular organelles into distinct regions
3) blebs on cell membrane
4) membrane bound cell fragments
36
In normal cells, there is a specific phospholipid on the interior of cell membranes. During apoptosis this phospholipid is found on the exterior of the cell to allow macrophages to recognize it. Cell destruction occurs before inflammation starts. What is this phospholipid?
phosphatidylserine
37
What are some examples of apoptosis in physiological conditions?
-endometrial cell during mensuration
-cell removal during embryogenesis
-virus infected cells and neoplastic cells by cytotoxic T cells
38
What are some examples of apoptosis in pathological conditions?
-councilman bodies in viral hepatitis
-gland atrophy following duct obliteration as in cystic fibrosis
-graft vs host disease
39
What are the 5 infiltrates that may cause cellular damage?
1) fatty change (steatosis)
2) cholesterol
3) glycogen
4) proteins
5) pigments (endogenous or exogenous)
40
What most commonly infiltrates cells?
fat (accumulation of triglycerides within parenchymal cells)
41
Where is steatosis most commonly seen in? Where else can it occur?
the liver (major organ of fat metabolism)
can also occur in the heart, skeletal muscle, kidneys, etc
42
What is steatosis (fatty changes in cells) causes by?
-alcohol abuse
-diabetics that are obese
-protein malnutrition
-toxins
-anoxia (absence of oxygen)
43
What are the most common causes of fatty liver?
obese diabetics and alcoholics
44
Cholesterol is critical for cell membranes, but when stored in excess it causes....
atherosclerosis and cardiovascular disease
45
Excess cholesterol accumulates in which cells?
macrophages (phagocytic cells)
46
When clusters of cholesterol filled macrophage cells in subcutaneous tissue become visible this is called
xanthomas
47
What is xanthoma? What is it linked with?
-clusters of fat and cholesterol in macrophages in subcutaneous tissue
-causes a fatty lump under the skin
-often linked with hyperlipidemia (high fat levels in the blood)
48
What is xanthelasma?
-a type of xanthoma (clusters of fat and cholesterol in macrophages in subcutaneous tissue, causes fatty lump under skin)
-this type specifically is found around the eyes and eyelids
-this is also the most common type of xanthoma
49
When theres a build up of glycogen and cannot be broken down. What are the causes?
-inborn error of metabolism with glycogen storage diseases like Von Gierke's disease
-Pompe's disease
-diabetes mellitus (defective glucose or glycogen metabolism, accumulation of glycogen in beta cells in some untreated patients)
note; bc these pts cannot breakdown glycogen, they need a constant supply of glucose
50
What is the most common cause of glycogen build up that cannot be broken down?
inborn error of metabolism with glycogen storage diseases like Von Gierke's disease
51
What is the most severe glycogen disease? Why is it the most severe?
Pompe's disease
-muscles dont develop well
-severe hypotonia, like a rag doll in muscle tone
-hepatomegaly
-cardiomegaly
52
What are the signs and symptoms of Von Gierke's disease?
-not growing fast enough
-heat intolerance
-bruising easily
-low blood sugar (hypoglycemia)
-enlarged liver
-swollen belly
-weak muscles (low muscle tone)
-muscle pain and cramping during exercise
53
Many acquired and inherited diseases are characterized by intracellular accumulation of proteins. What are the 4 types of issues here?
1) alpha 1 antitrypsin deficiency
2) abnormal protein folding from prion diseases like mad cow and CJD
3) abnormal protein aggregation
4) abnormal protein transport and secretion (ex: cystic fibrosis)
54
Which enzyme deficiency is an inherited disorder where genes code for a mutant version where proteins cannot be metabolized so they are accumulated in the liver and cause liver injury and cirrhosis?
alpha 1 antitrypsin deficiency
55
What is the abnormal protein aggregation in Parkinson's disease?
lewy bodies
56
What is the abnormal protein aggregation in Alzheimer's disease?
neurofibulary tangles
57
Amyloid build up in organs is called...
amyloidosis
58
What is cystic fibrosis (CF)?
-a defective cystic fibrosis transmembrane conductive regulator (CFTR) protein is unable to maintain the balance of salt and water in the lungs and other organs
-will result in abnormal protein transport and secretion
-this abnormality will result in thick, sticky mucus that builds up and leads to blockages, damages, or infections
-can also have inflammation in lungs and pancreas
-a sweat test is done to check for CF, if the pt has CF then they will be excreting an excessive amount of salt
59
Lewy bodies is most commonly associated with which disease?
boards q
Parkinsons disease (but its also seen in alzheimers and other diseases)
60
What are Lewy bodies?
-abnormal deposits of the protein alpha synuclein in the brain which interferes with brain function
-causes:
-dementia, decline in intellectual function
-movement problems
-visual hallucinations
-sudden changes in behavior and intellectual ability
-REM (rapid eye movement) sleep behavior disorder (involved with rubrospinal tract) acting out dreams, talking, moving, walking while asleep
-develops between 50-85 y/o
-typically survive 5-7 years after diagnosis
61
What are the 3 endogenous substances that can accumulate in cells?
1) lipofuscin
2) melanin
3) hemosiderin
62
What is the “wear and tear pigment"?
lipofuscin
63
Lipofuscin is a a brownish-yellow pigment in granules which are indigestible and accumulate intracellularly in various tissues as a function of age or atrophy. This is especially found in the heart, liver, and the brain. It is a mixture of lipids and proteins containing a golden-brown pigment called _________ which is derived from free radical damage. This does not injure the cell but it is a marker of past free-radical injury. This is commonly known as brown spots or age spots
ceroid
64
Melanin is a brown-black pigment made by melanocytes in the epidermis and acts as a screen for UV radiation. Melanin is found in the ______, _______, and other organs. Melanin is synthesized from _________ in melanocytes.
skin, eyes, tyrosine
note: the amount of melanin is responsible for differences in skin color and eye color
65
Melanin is important for what?
-skin color and eye color
-preventing sunburn and skin cancer
-light shield to the iris of the eyes
66
What is the genetic disorder called where there is an absence of melanin and they are unable to produce melanin?
albinism
67
What is the condition called where the skin loses melanocytes?
vitiligo
68
An increased production of __________________ from anterior pituitary gland acts as a stimulant on melanocytes causing them to produce more melanin and darkening/bronzing on skin
adrenocorticotrophic hormone (ACTH)
note: this happens in Addisons disease
69
Hemosiderin is a golden yellow brown pigment derived from _____________ and accumulates in tissues when there is an excess of ________ in the blood. This means the patient has trouble metabolizing Fe and this is usually considered pathologic, but small amounts are normal in the bone marrow, spleen, and liver.
hemoglobin, iron
70
What is hemosiderosis?
excessive deposition of hemosiderin
71
What is hemochromatosis?
excessive accumulation of iron and hemosiderin
72
Why is hemochromatosis worse for males?
because females blood let on their periods and this helps with getting rid of excess iron and hemosiderin
73
What is Wilson's disease?
-autosomal recessive
-abnormal copper accumulation in the brain, liver, and corneas
-usually due to diet (excess of chocolate, shellfish, etc.)
-can also be due to using copper silverware or cookware
-there will be a copper ring around the iris called Kayser Flerisher ring
74
What are the 6 types of necrosis?
1) coagulative
2) liquefactive
3) caseous
4) fat
5) fibrinoid
6) gangrenous
75
What is another name for coagulative necrosis?
ischemic necrosis
76
Which necrosis type NEVER OCCURS IN THE BRAIN?
coagulative necrosis/ischemic necrosis
77
What is the most common type of necrosis?
coagulative necrosis/ischemic necrosis
78
What is coagulative necrosis/ischemic necrosis?
-generally associated with infarcts and ischemia
-everything in cell dies (nucleus, organelles) but the cell itself will take a few days to be digested by WBCs
-can occur in all solid organs EXCEPT FOR THE BRAIN
-most frequently involves the heart and kidney
79
What type of necrosis occurs with a myocardial infarction?
coagulative necrosis/ischemic necrosis
80
define infarct
blood flow is completely cut off leading to necrosis
81
define ischemia
decrease in blood flow to a tissue and hypoxia (loss in oxygen)
82
What is a focal seizure?
occurs when electrical activity is limited in 1 area of the brain
83
Wernicke's area is in which lobe?
temporal lobe
84
Broca's area is in which lobe?
frontal lobe (area 41/42)
85
What is broca's aphasia?
cannot say full sentence, hard to communicate and understand others because its just a jumble of words
86
What is wernicke's aphasia?
saying words that don't exist/don't make sense
87
What is the function of wernicke's area?
speech comprehension
88
What is the function of broca's area?
speech production
89
What is another name for liquefactive necrosis?
colliquative necrosis
90
Which necrosis type is associated with abscesses and brain infarcts?
liquefactive necrosis/colliquative necrosis
91
What is liquefactive necrosis/colliquative necrosis?
-associated with abscesses and brain infarcts
-usually due to focal bacterial but can also be a fungal infection
-microbes stimulate the accumulation of inflammatory cells and WBC enzymes to digest the tissue
-autolysis with lysosomal enzymes
-heterolysis with WBCs
-tissue is transformed into a liquid, viscous mass
-can occur within hours
-rate of dissolving is faster than repair
-results in abscesses (pus-filled cavity formed in a solid tissue)
-often caused by staph, strep, or E. coli which all of these are pyogenic (pus-forming) bacterias that lead to suppurative inflammation
92
define focal
relating to center or point of interest, or 1 area of where something is
93
Which bacteria are usually the cause of liquefactive necrosis/colliquative necrosis?
staph, strep, and E.coli
94
What is suppurative inflammation?
inflammation accompanied with pus formation
95
What is an abscess?
pus filled cavity formed in a solid tissue
96
Ischemic injury of the CNS often causes liquefactive necrosis/colliquative necrosis. What is this injury called?
stroke, brain infarct, CVA, cerebrovascular accident (all mean same thing)
its the loss of neurons and glial cells and they are digested by hydrolytic enzymes leaving a cavity, causes a glutamate storm
97
What are the 2 types of fat necrosis?
1) enzymatic
2) trauma induced
98
Enzymatic fat necrosis most commonly affects which organ?
pancreas
99
Enzymatic fat necrosis is when activated pancreatic digestive enzymes (lipases) released into the pancreas and peritoneal cavity. Lipase releases FAs from TGs (fat destruction). The FAs complex with calcium, sodium, and magnesium to form soap (saponification). These soaps appear as white chalky deposits within normal adipose tissue. This often develops when the pancreas is inflamed. What is this called?
acute pancreatitis
100
What is trauma induced fat necrosis?
-can result from trauma to the breast
-TGs are released from the adipose tissue
-may mimic a tumor if calcified
101
What are the symptoms of pancreatitis?
-upper abdominal pain (right side)
-pain radiating to the back
-abdominal pain is worse after eating
-abdomen tender to the touch
-fever (infection)
-rapid pulse
-nausea
-vomiting
102
What is the #1 cause of acute pancreatitis? What are the other causes?
alcohol abuse
other causes:
-gallstones
-poisons
-insect bites
103
Caseous necrosis is mainly seen in?
TB
104
TB is a disease of the....
lungs
105
What is caseous necrosis?
-called caseous necrosis due to "cheesy" macroscopic appearance
-casein is a milk protein
-mycobacteria have a hydrophobic, waxy outer membrane which is resistant to digestion and phagocytosis
-macrophages activate to get rid of the mycobacteria but the bacteria infects the macrophages and kills them
-the immune system then seals off the bacteria with the dead cells and creates a granuloma
-the cells are walled off in the center of the granuloma- the organism is contained but not killed
-no normal chemotaxis occurs so the dead cells are never digested!!!!!! They deteriorate but don't get digested completely by hydrolases
106
TB has what lesions (usually in the lungs)?
ghon lesions, also known as walled off lesions
107
What are ghon lesions?
-lesions that do not attract WBCs so the material remains unchanged by WBC fermentation
-occurs in TB due to mycobacterium wall with the complex waxes
-caseous has a high fat content
-calcification if frequent sequel (innate healing attempt)
108
What is pott's disease?
spinal TB with caseous necrosis of vertebral bodies
109
What is fibrinoid necrosis?
-usually immune reactions when Ag-Ab complexes are deposited in the walls of arteries
-injured blood vessels accumulate plasma proteins which cause the wall to appear bright pink with H&E stain
-polyarteritis nodosa (widespread inflammation and damage to the small-medium ateries
110
What is polyarteritis nodosa?
widespread inflammation and damage to the small and medium arteries
111
What is gangrenous necrosis?
-not a distinctive pattern of necrosis
-refers to multiple layers of tissue that have been damaged by coagulative necrosis
-usually affects lower leg, may affect fingers and toes
-caused by prolonged ischemia or inaequete oxygen
>embolism (blood clot that travels)
>acute arterial thrombosis (blood clot that stays put)
>vascular trauma
> calciphylaxis (necrosis of the skin and fatty tissue in patients with end stage kidney disease
>extreme cold injury (frostbite)
112
What is calciphylaxis?
necrosis of the skin and fatty tissue in patients with end stage kidney disease
113
What are the 3 types of gangrene?
1) dry
2) wet
3) gas
114
What is the difference between dry and wet gangrene?
both are with coagulative necrosis but wet also has a bacterial infection
115
Which gangrene is known as mummification?
dry
116
What is dry gangrene?
-usually a result of coagulative necrosis (blockage of arterial blood supply (ischemia))
-no bacteria is involved here
-technically not a true gangrene bc theres no bacteria
-dull ache, sensation of coldness, pallor
-skin appearance is dry, dark brown or black
117
What is senile gangrene?
elderly with insufficent blood supply due to sclerosis of the blood vessels and or degradation of the walls of the smaller arteries
118
What is diabetic gangrene?
arteriosclerosis induced and or high blood sugar levels result in nerve damage, particularly in the feet, which can cause a loss of sensation in the affected area making it easier to develop an injury
119
What is raynaud's disease?
-characterized by spams of the smaller blood vessels in the fingers and sometimes toes, ear, nose, acral parts
-idiopathic
-most common in young females
-secondary to SLE, RA, thoracic outlet syndrome, drugs
120
What is the order of color changes in raynauds disease?
white> blue> red
121
What is wet gangrene?
-occurs if bacteria has infected the tissue
-swelling, blistering, wet appearance, smells terrible
-may develop after a severe burn, frostbite, or injury
-often occurs in people with diabetes who unknowingly injure a toe or foot
-must be cared for immediately bc it spreads quickly and can be deadly
-ex: decubitus ulcers aka bed sores
122
What is gas gangrene?
caused by clostridium perfringens which is an anaerobic bacteria that produces hydrolytic enzymes and toxins
-usually from injuries or surgical wounds with no blood supply, its a deep wound
-bacterial infection produces toxins which releases gas and causes tissue death
-surface of skin may appear normal at first, then becomes pale, then gray-red and bubbly
-pressing the skin may cause crackling sound due to the gas in the tissue and gas will release
-life threatening condition
-pressure from gas cuts off blood supply to surrounding tissues
-spreads quickly, up to 1 in per hour
-this results in anterior compartment syndrome and the leg will need to be cut to allow it to expand due to all the pressure built up
