Term
| What activated fatty acids before they are metabolized? |
|
Definition
|
|
Term
| What is the major component of a chyolomicron? |
|
Definition
|
|
Term
| How do chylomicrons travel to the blood? |
|
Definition
| exocytosis from the intestine into the lymph and through the thoracic duct |
|
|
Term
| How does a chyolomicron become mature? |
|
Definition
| it accepts ApoE and ApoCII from HDL in blood |
|
|
Term
How long are short FA?
medium Chain?
long?
very long? |
|
Definition
|
|
Term
| How are triacylglyerols of the chylomicron digested? |
|
Definition
| by the LPL (lipoprotein lipase) attached to porteoglycans of the basement membranes of endotheolial cells that line capillaries, making the chylomicron remnant |
|
|
Term
Where does alpha oxidation of lipids occur?
beta?
omega? |
|
Definition
alpha - peroixsomes
beta - peroisomes and mitochondria
omega - ER |
|
|
Term
| What does carnitine shuttle do? |
|
Definition
| gets long chain fatty acyl CoA into the inner mitochondrial membrane |
|
|
Term
| What regulates the carnitine shuttle? |
|
Definition
| the shuttle is the rate limiting step for most cells and is regulated by malonyl CoA |
|
|
Term
What is the function of CPT1?
Translocase?
CPTII? |
|
Definition
1 - makes fatty acyl CoA into Fatty acylcarnitine
translocase - switches out carnitine for fatty acylcarnitine into matrix
CPTII - separates fatty acylcarnitine |
|
|
Term
| What are the effects of carnitine deficiency? |
|
Definition
accumulate lipid droplets, weakness,
hypotonia hypoketotic, hypoglycemia (don't confused with McArdles which accumulates glycogen) |
|
|
Term
|
Definition
| process of breaking down a fatty acid, has 2 dehydrogenase steps, creates FADH2 and NADH |
|
|
Term
| Order of enzymes in beta oxidation: |
|
Definition
acyl CoA dehydrogenase, enoyl CoA hydratase, B hydroxy acyl CoA dehydrogenase
B Keto thiolase |
|
|
Term
| What regulates B - oxidation? |
|
Definition
| FADH2, NADH and acetyl CoA, insulin indirectly which causes the conversion of acetyl CoA to malonyl CoA (all of which stop the cycle) |
|
|
Term
| What is the purpose of Acetyl CoA caboxylase? |
|
Definition
in the cytosol it turns Acetyl CoA into Malonyl CoA and is positive for insulin and negative for AMP-PK
Malonyl CoA stops B oxidation |
|
|
Term
| medium chain acyl CoA dehydrogenase deficiency |
|
Definition
most common problem with B oxidation, first step in B oxidation does not work
Symtoms: hypoglycemia, hypoketonemia, dicarboxylic acid accumulation from peroxisomes |
|
|
Term
| What are the two major ketone bodies? |
|
Definition
| acetoacetate and B-Hydroxybutyrate |
|
|
Term
| What are the enzymes that make ketone bodies? |
|
Definition
| Thiolase, HMG CoA synthease, HMG CoA lyase, D, Beta Hydroxybutyrate dehydrogenase |
|
|
Term
| How is ketone synthesis allowed? |
|
Definition
Fatty acids start appearing
No insulin so no malonyl so CPTI works, low ATP so B oxidation occurs, Oxaloacetate is being taken from TCA cycle (which is overall slowed) so that it can do gluconeogenesis |
|
|
Term
| What would a fruity smell on the breath indicate? |
|
Definition
| ketone in the blood, possible ketoacidosis |
|
|
Term
| What fats does the peroxisome metabolize? |
|
Definition
VLCFA and Branched Fatty Acids
non caritine transpoter, still as long chain fatty acyl CoA synthetase
can be given a carnitine and then transported by that pathway to the mitochondria matrix |
|
|
Term
| What happens to the NADH produce by the peroxisome during VLCFA degradation? |
|
Definition
| it does the malate or Glut shuttles to the ETC just like those from glycolysis |
|
|
Term
| w (omega) oxidation in ER |
|
Definition
creates alcohol then diacarboxilic acid and NADPH
it gets B oxidized in peroxisome and makes this dicarb acids |
|
|
Term
Zellweger syndrome
Refsum disease
XALD |
|
Definition
Zellweger - defective peroxisomal biogenesis
refsum - mutations in phytanoyl CoA hydroylase (need low phytanic diet)
XALD - peroxisomal ABC half tansporter, cannot import VLCFA |
|
|
Term
How do we make propinoyl CoA?
What gets made from propinoyl CoA?
ABBC? |
|
Definition
Odd Fatty acids
Succinyl CoA, which can be used for gluconeogenesis as it can become OAA
ABBC - ATP, Biotin, B12, CO2 |
|
|
Term
|
Definition
| accumulation of metholmalonylacid, as it gets stuck from propionyl to succinyl CoA |
|
|
Term
| OAA's purpose in glucose to FA? |
|
Definition
| shuttle out as citrate with acetyl CoA, make NADPH on way back in, as pyruvate |
|
|
Term
| Where does acetyl CoA turn into Malonyl CoA? |
|
Definition
| Cytoplasm and by Acetyl CoA carboxylase |
|
|
Term
| What enzyme take malonyl and makes Palmitate? |
|
Definition
|
|
Term
| Why does acetyl CoA join with OAA to make Citrate to leave the mitochondria? |
|
Definition
| there is no acetyl CoA transporter |
|
|
Term
| What regulates the making of Fatty acids? |
|
Definition
acetyl-CoA carboxylase
negative - glucagon, epinephrine, HIgh AMP Palmiitate
positve - insulin, citrate |
|
|
Term
| What fatty acids are essential in the diet? |
|
Definition
| linoleic and alpha-linolenic cause we cant make these double bond places |
|
|
Term
| Where is palmitate elongated and desaturated? |
|
Definition
|
|
Term
| What enzyme converts Acetyl CoA to malonyl CoA? |
|
Definition
|
|
Term
| What is the regulation of acetyl CoA carboxylase? |
|
Definition
Active without phosphate group on it (like PDC)
Positive -insulin and citrate
Negative - Palmitoyl CoA |
|
|
Term
What is the purpose of glycerol 3 phosphate?
Where is it produced in the Liver? In the adipose tissue? |
|
Definition
It is the backbone of a triacylglycerol
Liver: A glycerol kinase on glycerole or dihydroxyacetone reeduction from glycolysis
adipose tissue: Only from dihydroxyacetone from glycolysis |
|
|
Term
| Where is the triacylglycerol produced? |
|
Definition
| Smooth ER of the Liver, packaged into VLDL |
|
|
Term
| Lippoprotein Ligase (LPL) |
|
Definition
| proteoglycans of endothelial cells, cleaves triacylglycerols in VLDL and chylomicrons |
|
|
Term
| What does glucagon do to adipose tissue? |
|
Definition
| Nothing! It has no glucagon sensor. A low insulin or epinephrine or cortisol stimulates the release of Fatty Acids |
|
|
Term
| Can the liver survive on ketone bodies? |
|
Definition
| No! it makes them but cannot degrade them |
|
|
Term
| Familial Hyperchylomicronemia |
|
Definition
LPL deficiency or altered ApoCII -> pancreatitis, hepatospleenomegaly and xanthomas (skin lesions), no atherosclerosis
4 problems: LDL receptor, apo B-100, PCSK9, ARH |
|
|
Term
|
Definition
| From Liver with TAG, accept apoCII and apoE from HDL and are degraded by capillary LPL, became VLDL remnant called IDL which can be taken up by liver or by peripheral cells to be broked down or form LDL by HTGL |
|
|
Term
|
Definition
| largest, least dense lipoprotein, take up dietary TAGs |
|
|
Term
|
Definition
| from VLDL, becomes LDL which can be taken up by liver or taken to cells that produce steriods as LDL's are cholesterol rich |
|
|
Term
|
Definition
| if LDL is in excess it can be oxidized taken up by macrophages which will turn into foam cells, remember LDL can easily be oxidized in the blood if it hangs out too long |
|
|
Term
|
Definition
| Liver, intestine, from VLDL by LPL, from free apoAI |
|
|
Term
|
Definition
| gives apoproteins to nascent chylomicrons and VLDL and picks up cholesterol from peripheral tissues |
|
|
Term
|
Definition
| made by liver, enzyme that traps cholesterol in HDL from cell membranes |
|
|
Term
| Where is cholesterol made? |
|
Definition
| Liver and cytosol, otherwise is it like Ketone body synthesis which is done in the mitochondria, from two acetyl CoA's |
|
|
Term
| What is an important enzyme of cholesterol synthesis? |
|
Definition
| isopentenyl pyrophosphate (anchored and puts intermediate next to membrane) |
|
|
Term
| What is the rate limiting step of cholesterol synthesis? |
|
Definition
HMGCoA reductase
inhibited by cholesterol or statins or glucagon
positive with insulin |
|
|
Term
|
Definition
|
|
Term
From where do we get?
Asparagine and Aspartate?
Alaine?
Serine Glycine and Cysteine?
Tyrosine?
Glutamate, Glutamine, Proline, Arginine? |
|
Definition
Asparagine and Aspartate? OAA
Alaine? Pyruvate
Serine Glycine and Cysteine? Phosphoglycerate
Tyrosine? Phenylaline
Glutamate, Glutamine, Proline, Arginine? alpha ketoglutarate |
|
|
Term
| Cancer therapy with amino acids? |
|
Definition
| Cells need arginine to grow quickly so we convert it to aspartate to deprive them of it using asparaginase |
|
|
Term
|
Definition
starchy diet poor protein, thin skin and hair, fatty liver due to low synthesis of lipoprotein, edema, anemia
kwishiorkor could still wash |
|
|
Term
|
Definition
low caloric intake and specific amino acids are deficient, huge loss of body mass
Marasmus - MUST eat soon |
|
|
Term
| How do digested amino acids enter the blood? |
|
Definition
| digested by proteases in intestinal lumen and enter blood |
|
|
Term
Protein digestion with insulin?
with glucagon? |
|
Definition
insulin - synthesise proteins in the muscle
glucagon - make amino acids into glucose in liver |
|
|
Term
|
Definition
defective transport of neutral amino acids
Phe is especially reduced
Pellegra, ataxia, phtotsensitivty |
|
|
Term
|
Definition
COLA - cystiene and dibasic AA< ornithine, lysine, arginie are not transported well
accumulation of cystine and stones in the UT |
|
|
Term
|
Definition
can be used by most tissues to produce energy
V,I,L |
|
|
Term
| What are the three aminotransferase reactions? |
|
Definition
pyruvate to alanine
OAA to Aspartate
alphaketogluterate to glutamate |
|
|
Term
| Glutamate to glutamine to gluamate to alphaketogluterate |
|
Definition
| needs to gain NH4!, done by glutamine synthetase in muslce which can transfer through blood and then go to alphaketoglutamate in liver |
|
|
Term
| What are the two ways the liver gets ammonia? |
|
Definition
| Through alanine or glutamine |
|
|
Term
What enzyme will make glutamine?
What enzyme will break down glutamate? |
|
Definition
glutamine synthetase
glutamate dehydrogenase |
|
|
Term
| Where does the majority of the urea cycle occur? |
|
Definition
|
|
Term
| What causes hyperammonemia? |
|
Definition
| tremor, slurred speech and death are OCC deficieny, as ammonia is overdosed and reacts with alphaketogluterate to form glutamate in neurons |
|
|
Term
| What are the three ways to get hyperammonemia? |
|
Definition
kidney failure
liver disease
OTC deficinecy (ortic acid in urine) |
|
|
Term
What amino acids are gluco and keto?
just keto?
What are all the others? |
|
Definition
|
|
Term
PKU
classical?
nonclassical?
symptom? |
|
Definition
phenylaline hydroxylas
dihydropteridine reductase
both accumulate phenylalanine
mental retardation |
|
|
Term
|
Definition
homogentistic acid accumulates
homogentisate oxidase is broken
black urine, arthritis
think homo gentlemen names Al |
|
|
Term
|
Definition
fumarylacetoacetate accumulates
fumarylacetoacetate hydrolase broken
liver failure, death early
think pyro (fume) with tyro |
|
|
Term
|
Definition
tyrosine accumulates
broken tyrosine aminotransferase
neurologic defects |
|
|
Term
|
Definition
cystathionine
cystathionase broken
benign |
|
|
Term
|
Definition
homocysteine
cystathioine B-synthase broken
cardiovascular problems and nuerological problems |
|
|
Term
|
Definition
glyoxylate accumulates
glycine transaminase broken
renal failue due to stone formation |
|
|
Term
| Maple syrup urine disease |
|
Definition
alpha keto acids of branched amino acids accumulate
branch chain alpha keto acid dehydrogenase is broken
mental retardation |
|
|
Term
What enzymes starts to break down ethanol?
What does it turn it into?
What drug inhibits it? |
|
Definition
alcohol dehydrogenase, CYP2E1, Peroxisome catalase
acetaldehyde
Fomepizole |
|
|
Term
| What breaks dorn acetaldehyde? |
|
Definition
aldehyde dehrdrogenase (inhibited by disulfiram)
to acetate which can go to tissue to be acetyl CoA |
|
|
Term
| What are the acute effects of high ethanol intake? |
|
Definition
NADH/NAD ratio is high
accumulaiton of H+ and acidosis |
|
|
Term
| Why do asians blush with alcohol? |
|
Definition
| bad ALDH and acetaldehyde accumulates |
|
|
Term
| What happens to an alocholics liver? |
|
Definition
| the high ethanol causes high NADH, this means no TCA so instead we get Fatty Acid Synthesis, Fatty Liver |
|
|
Term
| How do we get ketoses with alcholism? |
|
Definition
| no gluconeogenesis, low PDC, high levels of acetyl CoA makes ketone bodies! Possible ketoacidosis |
|
|
Term
| Ethanol affects on the brain |
|
Definition
1) increase GABA (the stop signal)
2) reduces glutamate (the go signal for neurons) |
|
|
Term
|
Definition
| confusion, ataxia, nystagmus, do to alcholism |
|
|
Term
|
Definition
| vitamin B deficincy, problem with absorption |
|
|
Term
|
Definition
pyruvate dehydrogenase
alpha ketoglutarate
BCAA alpha ketpacid dehydrogenase |
|
|
Term
| What are some reasons for hypoglycemia? |
|
Definition
too much insulin release
no glucose made by liver
alcohol metabolism
increased need in tissue |
|
|
Term
|
Definition
take glucose to acetyl CoA (PK, PDC)
make Fats (acetyl CoA carboxylase) and make glycogen (Glycogen synthase, not glycogen phosphorylase nor phosphorylase kinase) |
|
|
Term
|
Definition
| pancreas does not produce enough insulin, too much glucagon in blood |
|
|
Term
|
Definition
degrade muscle
gluconeogenesis
glycogenolysis possible hyperglycemia
Lipolysis possible ketoacidosis
Low LPL activity possible hypertriglyceridemia |
|
|
Term
| What are the two non autosomal recessive LSD's? |
|
Definition
| Fabrys, and Hunters which are X-Linked! |
|
|
Term
| Which LSD's are more common in Ashkenazi Jews? |
|
Definition
GTP
Gaucher
Tay-Sachs
Niemann Picks Type A |
|
|
Term
|
Definition
| inside the proteoglycans,degraded by lyosomes after endocytosis, problems with this cause diseases known as mucopolyaccharidoses |
|
|
Term
|
Definition
Enzume - alpha-L-iduronidase
accumlates - Herparan Sulfate + dermatan sulfate
developmental decay, gargoylism, airway obstruction, CLOUDING! |
|
|
Term
|
Definition
enzyme :Iduronate sulfatase
accumulates: heparan sulfate + dermatan sulfate
mild Hurlers and aggressive behavior, NO clouding |
|
|
Term
|
Definition
| Mucopolysacharidoses - heparan sulphate only accumulates |
|
|
Term
| Mucopolysaccharidosiss pathway |
|
Definition
iduronate sulfates to alpha L iduronidase to heperan N sulfatase
is goes mucopolysaccharidosis II then I |
|
|
Term
| Sphringolipidosose Lysomal Storage Diseases |
|
Definition
Fabry
Gaughers
NPD
Tay Sachs
Krabbes
Metachromatic Leukodystrophy |
|
|
Term
| Generalized Gangliosidosis |
|
Definition
Beta Galactosidase
GM1 accumulates |
|
|
Term
|
Definition
alpha galatosidase
ceramide trihexoside accumulates
peripheral (hands and feet neuropathy - painful joints), angiokeratomas, cardio and renal disease |
|
|
Term
|
Definition
Beta - glucocerebrosidase
Glycocerebroside accumulates
hepatosplenomegaly, the neuropathic are usually fatal, macrophages look like tissue paper |
|
|
Term
|
Definition
No man Pick his nose with his sphinger
sphingomyelinase
sphingomyelin accumulates
neurodegeneration, foam cells, cherry red sport on macula
(yes heptospjenomegaly vs Tay Sax) |
|
|
Term
|
Definition
hexosamindase A (Tay Sax and heXo)
GM2 ganglioside accumulates
cheery red spot, neurodegenertaion, lysosomes with onion skin, (no heptospjenomegaly vs NP) |
|
|
Term
|
Definition
Galactocerebrosidase
Galactocerebroside
Peripheral neuropathy, developmental Delay, globoid cells |
|
|
Term
| Metachromatic Leukodystrophy |
|
Definition
Arylsulfatase A
Cerebroside sulfate accumulates
demyelination with ataxia and dementia |
|
|
Term
| What are the types of anchoring junctions between cells? |
|
Definition
adherens, actin linked cell matrix adhesions
desmosomes
hemidesmosomes |
|
|
Term
| What are the types of occluding junctions in vertebrates? |
|
Definition
| tight junctions, composed of claudins and occludins |
|
|
Term
| channel forming junctions in animals? |
|
Definition
|
|
Term
| what is the difference in tanscellular and paracellular transport? |
|
Definition
trans - across a cell
para - transported between cells |
|
|
Term
| What are the two purposes of tight junctions? |
|
Definition
separates the basal and apical membranes
seals cells so that they do not leak from one side to the other |
|
|
Term
| What are the two chemicals making up a tight junction? What do they require? |
|
Definition
claudins and occludins, requires calcium
have sealing strands that holds adjacent cells together |
|
|
Term
| What is the purpose of an anchoring junction? |
|
Definition
| to attach the cytoskeletons of cells to another cell or extracellular matrix |
|
|
Term
| What are zona adherens (intermediate junction)? |
|
Definition
cadherins connect to actin, CADherins are CA2+ dependent ADhesion molecules
use transmambrane glycoproteins and intracellular anchor proteins |
|
|
Term
adherens junction
transmembrane?
extracellular ligand?
intracelluar attachment?
intracellular anchor? |
|
Definition
transmembrane? cadherin
extracellular ligand? cadherin in other cell
intracelluar attachment? actin
intracellular anchor? catenin |
|
|
Term
desmosome
transmembrane?
extracellular ligand?
intracelluar attachment?
intracellular anchor? |
|
Definition
transmembrane? cadherin
extracellular ligand? desmoglein
intracelluar attachment? intermediate filaments
intracellular anchor? plakoglobin |
|
|
Term
actin linked cell matrix adhesion
transmembrane?
extracellular ligand?
intracelluar attachment?
intracellular anchor? |
|
Definition
integrin
extracellular matrix proteins
actin filaments
talin, vinculin, FAK |
|
|
Term
hemidesmosome
transmembrane?
extracellular ligand?
intracelluar attachment?
intracellular anchor? |
|
Definition
transmembrane? integran (laminin), collagen
extracellular ligand? matrix proteins
intracelluar attachment? intermediate filaments with plectin
intracellular anchor? plectin, dystonin |
|
|
Term
| What can adheren junctions cause a group of cells to do? |
|
Definition
|
|
Term
| Which cell junction causes the most strength? |
|
Definition
|
|
Term
|
Definition
antibodies against desmogliens destroy desmosomes, causes lots of blisters
autoimmmune disease |
|
|
Term
| focal adhesions (actin linked cell matrix adhesion) |
|
Definition
aka focal contacts,
mediate attachment to substrata, important in cell movement
Integrins: fibronectin, laminin
attachment proteins: vinculin,talin and actinin
use stress fibers |
|
|
Term
Gap junction
purpose?
made of? |
|
Definition
allows cell to communicate
made of connexon |
|
|
Term
|
Definition
cell adhesion molecules, not as organized,
examples: cadherins, Ig family, selectins, integrins, transmembrane proteoglycans |
|
|
Term
E cadherin
P cadherin
N cadherin
VE |
|
Definition
epithelial
placenta
nerve, muscle Vascular
VERY CALCIUM DEPENDENT
homophilic |
|
|
Term
|
Definition
transient, heterophilic interactions
white blood cells
platelets
endothelial |
|
|
Term
|
Definition
nueral cell adhesion molecule
CALCIUM INDEPENDENT
not that strong but very specific |
|
|
Term
|
Definition
| lots of blistering, collagen, integrin, laminin mutations, stop are worse than missense |
|
|
Term
| synaptic cleft junction proteins |
|
Definition
| neurexin and neuroligin and canherin |
|
|
Term
|
Definition
| a core protein linked to many GAGs |
|
|
Term
| What cell type produces the ECM? |
|
Definition
|
|
Term
Which matrix molecules are soluble?
which are insoluble? |
|
Definition
sol - proteoglycans, hyaluronan, glycoproteins
insol - collagens and elastic fibers |
|
|
Term
What are GAG's?
Some types? |
|
Definition
sulfated, negative, form a gel
hyaluron, chondritin, dermatan sulfate, heparan sulfate, keratan sulfate |
|
|
Term
Gag synthesis
Where are they made?
What enzyme? |
|
Definition
ER and golgi
glycosyl transferases |
|
|
Term
|
Definition
simpliest and longest GAG
heart valve, joints
it is the only GAG that exists free of the proteoglycan |
|
|
Term
| What links a GAG chain to the core? |
|
Definition
|
|
Term
| What is the proteoglycan in cartilage? |
|
Definition
| Aggrecan! which has chondroitin sulfate and keratan sulfate GAGs |
|
|
Term
| What do proteoglycans do in the kidney? |
|
Definition
|
|
Term
| What is the most abundant protein in mammals? |
|
Definition
|
|
Term
|
Definition
|
|
Term
| What is the amino acids in collagen? |
|
Definition
| GLY-hydroxylysine-hydroxyproline |
|
|
Term
|
Definition
Be (So Totally) Cool, Read Books
1- Bone,Skin, tendon
2 - cartwoledge
3 - reticulin, skin, blood vessels, uterus, fetal tissue
4- Basement membrane or basal lamin (four and floor) |
|
|
Term
| what is the most abundant form of collagen? |
|
Definition
|
|
Term
|
Definition
| Type 4 collagen mutation, irregular and thickened glomerular filter |
|
|
Term
|
Definition
|
|
Term
| What are the steps of collagen synthesis? |
|
Definition
hydroxylation
glycosylation
self assembly
form helix
secretion
cleavage of propeptides
self assembly into fibril |
|
|
Term
|
Definition
osteogenesis imperfecta
chondrodysplasia
Ehlers-Danlos Syndrome Type 4 |
|
|
Term
|
Definition
Scury
prolyl hydroxylase that hydroxylates prolines that make collagen to collagen is slowly degraded |
|
|
Term
|
Definition
deficient procollagen peptidase
deficient lysyl hydroxylase
joint dislocation and hypereleasticity of the skin |
|
|
Term
|
Definition
| loss of elastic fibers in skin, made only in fetal and juvenile fibroblasts |
|
|
Term
|
Definition
| loss of elastic fibers in skin, made only in fetal and juvenile fibroblasts |
|
|
Term
|
Definition
polymer of tropoelastin
50% of aorta
no glycosylation
high in glycine and proline
crosslinking to be made linear |
|
|
Term
|
Definition
they cover elastic fibers
made of fibrillin |
|
|
Term
|
Definition
connective tissue is too elastic, poor recoil
chromosome 15
chest deformity, long arm, arachnodactyly, mitral valve prolapse
caused by deficincy in protein fibrillin 1 |
|
|
Term
| congential contractural arachnodactyly |
|
Definition
mutation in chromosome 5 in fibrillin 1 gene
affects skeleton system but not cardio or occular |
|
|
Term
| What are two examples of adhesive glycopoteins? |
|
Definition
|
|
Term
|
Definition
heterodimer, in plasma for wound healing
Structure is RGD (Arg-Gly-Asp)
it is an integrin (snakes destroy this) |
|
|
Term
|
Definition
trimer of alpha, beta, gamma
bind to cells and proteoglycans
assembly into sheet
receptors are integrins
promotes adhesion of epithelial cells to basement membrane |
|
|
Term
| What is the bassal lamina made of? |
|
Definition
type 4 collagen
laminin
integrin
perlecan
nidogen |
|
|
Term
|
Definition
|
|
Term
|
Definition
| becomes damaged and allows proteins through, remember this is basal lamina damage |
|
|
Term
| congenital muscular dystrophies |
|
Definition
defects in laminin alpha 2 chain
links actin skeleton to extracellular matrix
bridge goes wrong and mature muscle cells undergo apoptosis |
|
|
Term
| Enzymes in ECM degradation |
|
Definition
metalloproteases like collagenase
serine proteases like urokinase-type plasminogen activator |
|
|
Term
| What are the three types of cytoskeleton filaments? |
|
Definition
intermediate (vimentin) - structure
microtubules (tubulin) - organization, moves things within cell
microfilaments (actin) - crawling, pinching |
|
|
Term
| Two Ends of the actin filament |
|
Definition
Pointed - slow polymerization
barbed end - rapid polymerization |
|
|
Term
microtubules
+?
-?
right?
left? |
|
Definition
+? rapid polymerization
-? slow polymerization
right? short
left? long |
|
|
Term
|
Definition
| Antiparallel structure goes to 2 dimers and makes 8 protofilaments to a filament |
|
|
Term
|
Definition
|
|
Term
Motor Proteins
What do they travel on? |
|
Definition
Actin
Microtubules (Dynein and Kinesin)
Nucleic Acid Based, move on DNA to produce force |
|
|
Term
| What are the two parts of a flagellar motions? |
|
Definition
Effective stroke
Recovery Stroke |
|
|
Term
| 3 Parts of the cell movement |
|
Definition
Lamellipodium (fast moving cells)
Filopodia (slow)
Membrane Ruffle (projects upward) |
|
|
Term
| How do we get Lamellipodium protrusion? |
|
Definition
| actin polymerization, inhibited by cytochalasin |
|
|
Term
| How do we get movement adhesions? |
|
Definition
| stress fibers contract to break focal adhesions |
|
|
Term
Where in the cell are.......nucleated?
microtubules?
microfilaments?
intermediate filaments? |
|
Definition
microtubules? centrosome
microfilaments? plasma memrane
intermediate filaments? nucleus to cell surface |
|
|
Term
What is ...........made of?
microtubules?
microfilaments?
intermediate filaments? |
|
Definition
tubulin
actin
lamins, keratins |
|
|
Term
| What is the microtubule structure? |
|
Definition
alternating alpha and beta tubulin, hollow tube
9-2 |
|
|
Term
|
Definition
cilia, centriole to cytoplasm, needed for division
Cell Shape
Polarity
two way traffic
segregate chromosomes |
|
|
Term
| What is the role of microfilaments? |
|
Definition
| muscle contractions, cell movement, cell-cell anchoring |
|
|
Term
| What is the filament structure? |
|
Definition
| 8 actin tetramers twisted into a rope |
|
|
Term
| What is the role of intermediate filaments? |
|
Definition
| mechanical strength, cell-cell anchoring |
|
|
Term
|
Definition
| Eicosanoids are signaling molecules made by oxidation of twenty-carbon essential fatty acids, (EFAs |
|
|
Term
| What are the three classes of cell surface receptors? |
|
Definition
G-protein coupled
Enzyme coupled
ion channel coupled |
|
|
Term
| What accounts for 60% of all prescription drugs? |
|
Definition
| G protein coupled receptors |
|
|
Term
| What happens to the trimer (alpha, beta, gamma) after a hormone binds to a G protein? |
|
Definition
| disassociates, only the alpha stands alone |
|
|
Term
| What are the three types of G protein second messengers? |
|
Definition
cAMP
DAG (diacylglycerol)
IP3 |
|
|
Term
| How is the G protein alpha deactivated? |
|
Definition
| GTPase takes off the GTP and phosphate |
|
|
Term
| What is an example of an enzyme coupled surface receptor? |
|
Definition
tyrosine kinase subfamilies, have tyrosine kinase inside cell and cysteine or immunoglobin outside
can be a signal that brings dimers together |
|
|
Term
Each of these is an example of what?
Glucocorticoid receptor?
Estrogen Receptor?
Thyroid Receptor? |
|
Definition
Glucocorticoid receptor? hooks up in cytoplasm with receptor
Estrogen Receptor? hooks up in nucleus with receptor
Thyroid Receptor? hooks up to receptor already bound to DNA |
|
|
Term
| What is an orphan receptor? |
|
Definition
| one who's function we do not know |
|
|
Term
| What are the pathways of desensitization? |
|
Definition
receptor sequestration in vesible
receptor degradation in lysosome
receptor inactivationinactivation of signaling protein
production of inhiitory protein |
|
|
Term
| What are the three most important intracellular messengers? |
|
Definition
|
|
Term
What does adenyl cyclase do?
Who activates or inactivates it? |
|
Definition
|
|
Term
How do DAG and IP3 get activated by?
Who do they activate? |
|
Definition
GPCR to phospholipase C to phosphotidylinositol 4,5 bisphosphate to inositol, 1,4,5 triphosphate (IP3) and diacyl glycerol (DAG)
They acrivate:
IP3 - release from Ca2+ from ER
DAG- im membrane fro protein kinase C |
|
|
Term
| What does cAMP stimulate? |
|
Definition
| PKA and CA2+ stimulated calmodulin-dependent protein kinase |
|
|
Term
| What do PKA, PKC and CaM kinases phosphorylate in target proteins? |
|
Definition
|
|
Term
How does the GPCR signal stop?
Galpha?
IP3?
cAMP?
Ca2+? |
|
Definition
Galpha? leaves as GDP
IP3? is dephosphorylated
cAMP? hydrolyzed by phosphodiesterase
Ca2+? pumped out |
|
|
Term
autocrine?
paracrine?
endocrine?
intracrine? |
|
Definition
autocrine? cell pumps out its own signal
paracrine? signal to local cells
endocrine? in blood to far away cells
intracrine? never leaves cell |
|
|
Term
| Which signalling pathways directly bind to DNA? |
|
Definition
| Steriod hormones, thyriod hormones |
|
|
Term
| Which signalling pathways use a cytoplasmic kinase and phosphorylate a factor in the nucleus? |
|
Definition
PKA to CREB
receptor tyrosine kinases to MAP
T cell receptor to cytoplasmic tyrosine kinase |
|
|
Term
| What are examples of the signalling pathway that phosphorylate transcription factors in the nucleus? |
|
Definition
Cytokines to STAT
TGFbeta to Smads
TNF to NfkappaB
Delta to Notch
Hedgehog
Wnt to Beta catenin
TCR to NF-AT |
|
|
Term
What is the structure of PKA?
What does it do when it is phosphorylated? |
|
Definition
| PKA is 4 subunits (2 C and 2 R), the R's and C's separated with the R's phosphorylated by cAMP |
|
|
Term
| What is the adenylate cyclase pathway? |
|
Definition
| Galpha activated adenylate cyclase which makes cAMP which activated PKA |
|
|
Term
|
Definition
It is activated by PKA and stands for cAMP-Response element binding as PKA is downstream of cAMP
CREB is a transcription factor |
|
|
Term
| What is the difference in how GTP and ATP activate a signal? |
|
Definition
| GTp usually binds to the substrate while it is working, ATP just gives the phosphorus |
|
|
Term
|
Definition
|
|
Term
|
Definition
| calcium release from Smooth ER |
|
|
Term
| What is a phosphoinositides? |
|
Definition
| specific phospholipid molecules, think of them as docks that can be activated activated with 3 phosphorus that can activate intracellular proteins by bringing them closer together |
|
|
Term
What are some examples of Tyrosine kinases?
What are some serine/therosnine kinases? |
|
Definition
EGF, PDGF, insulin, JAK
PKA, PKC, CAM, PKB, MAP, TGFBeta |
|
|
Term
| Two types of protein kinase C |
|
Definition
gamma - growth factors
beta - GCPR |
|
|
Term
| EGF can work through MAP kinase, what is the pathway? |
|
Definition
| EGF receptor, PKCgamma, SH2, RAS or P-tyrosine, RAF then MAP kinase pathway (RAS,MEK,ERK) |
|
|
Term
| What is the difference in a scaffold and activated receptor? |
|
Definition
scaffold already has many proteins living on it
activated receptor recruits several proteins after it is activated |
|
|
Term
| RTK and dominant negative inhibition |
|
Definition
| RTK needs to bring two monomers together and phosphorylate, however one bad mutant monomer stops both and is therefore a dominant negative |
|
|
Term
|
Definition
cytokines, growth hormone, prolactin
brinigs two dimers together, cross phosphorylate on tyrosine, grabs SH@ and STAT and state goes to the nucleus |
|
|
Term
|
Definition
stress, inflammatory, immune
TNFalpha trimer to IKK complex (with NEMO) reacts with NFkB to liberate it from IkB which goes to nucleus |
|
|
Term
| to what are tyrosine kinases bound? |
|
Definition
the membrane!
most serine/theorine kinases are not! |
|
|
Term
| What is another function of the STAT in JAK-STAT? |
|
Definition
| it goes to mitochondria for oxidative phosporylation if phosphorylated on Serine instead od Tyrosine |
|
|
Term
| What makes a receptor disassociate from its ligand? |
|
Definition
| the acid environment of the endosome/lysosome |
|
|
Term
| What is a peripheral protein quality control system? |
|
Definition
| ubiquitinate to make a peripheral proteins death, CHIP mediate, brought to lysosome by ESCRT |
|
|
Term
| Where can a receptor go if it was damaged through endocytosis? |
|
Definition
|
|
Term
|
Definition
Southern = DNA
Northern = RNA
W = Proteins |
|
|
Term
What does ribonucleotide reductase do?
What inhibits it? |
|
Definition
make NDP into dNDP (Rna to DNA
hydroxyurea |
|
|
Term
| Where does PRPP come from? |
|
Definition
| pentose phosphate pathway |
|
|
Term
| What is the main symptom of folate deficincy? |
|
Definition
|
|
Term
| What does folate make that is important for the body? |
|
Definition
|
|
Term
| What vitamin can untrap folate? |
|
Definition
|
|
Term
| How do you distinguish between folate defincy and B12 def? |
|
Definition
both have homocysteine accumulate
B12 will have the nurologi disorder with it from the conversion of methylmaolonyl CoA to succinyl CoA from odd chain FA and branched amino acids |
|
|
Term
|
Definition
cancer treatment, not dUMP to dTMP
inhibits thymidylate synthase |
|
|
Term
|
Definition
| blocks dihydrofoltae reductase, which is make FH4 from FH2 |
|
|
Term
|
Definition
no hyperammonia
inability to convert orotic acid to UMP
defective orotic acid phosphooribosyltransferase or orotidine 5 phosphate decarboxylase |
|
|
Term
| Purine synthesis inhibiting drugs |
|
Definition
Mycophenolic Acid - IMP dehydrogenase
6-mercaptopurine and Allopurinol inhibit glutamine phosphoribosyl amindotransferase |
|
|
Term
|
Definition
| Inhibits Hypoxanthine oxidase at 2 points to we cant make uric acid and no gout! |
|
|
Term
|
Definition
adenosine deaminase deficiency
accumulation of deoxyadenosine stop ribonucleotide reductase, kill T and B cells |
|
|
Term
|
Definition
HGPRT (he's got purine recovery trouble)
gout and self mutaliation
purines cant be recovered, so they just get degraded |
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