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Biochem Complex Lipid Metabolism
KYCOM Block 3
109
Biochemistry
Graduate
11/05/2012

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Term
describe the structure of phospholipids
Definition
alcohol with a phosphodiester bond to diacylglycerol or sphingosine
Term
describe the polarity of phospholipids
Definition
amphipatic, polar, ionic
Term
what is the prodominate lipid of cell membranes
Definition
phospholipids
Term
what are examples of the hydrophoic portions of phospholipids
Definition
glycolipids, proteins, cholesterol
Term
what is the function of membrane phospholipids
Definition
reservoir for intracellular messengers and anchors for proteins
Term
what is the function of non-membrane bound phospholipids
Definition
components of lung surfactant and essential components of bile acting as a detergent to solublilze cholestrol
Term
describe the structure of a glycosphingolipid
Definition
glycerol back bone, 2 fatty acyl groups on C1 and C2, phosphate on C3
Term
what is the simplest glycerophospholipid
Definition
phosphatidic acid
Term
what is another name for glycerophospholipid
Definition
phosphoglyceride
Term
what are all phosphoglycerides a derivative of
Definition
phosphatidic acid
Term
describe the structure of sphingophospholipids
Definition
sphingosine backbone, long chain FA at carboxyl (palmotyl CoA), long chain FA at amino that is desaturated, phosphate group
Term
what is a sphingosine composed of
Definition
amino alcohol
Term
what are all phosphoglycerides formed from
Definition
phosphatidic acid with alcohol esterified to the carbon-3-phosphate
Term
what alcohols can be esterified to the carbon-3-phosphate of phosphoglycerides
Definition
serine, ethanolamine, choline, inositol, glycerol
Term
describe the structure of a plasmalogen
Definition
FA at C2 is repalced by an unsaturated alkyl group attached by an ether rather than an ester link
Term
describe the structure of platlet activating factor
Definition
unusual ether glycerophospholipid, saturated alkyl group eith ether link to C1, acetyl residue at C2
Term
what is the function of platlet activating factor
Definition
binds to surface receptors, triggers potent thromboitic and acute inflammatory events, activates imglammatory cells and mediates hypersensitivity, acute inflammation, anaphylactic reactions
Term
what does platlet activating factor cause to happen (in other cells)
Definition
platlets to aggregate and degranulate, neutrophils and alveolar macrophages to generate superoxide radicals
Term
what is the function of superoxide radicals
Definition
kill bacteria
Term
describe the structure of cardiolipin
Definition
two PA molecules esterified through phosphates to molecule of glycerol
Term
where is cardiolipin found
Definition
bacteria and eukaryotes
Term
where is cardiolipin in eukaryotes
Definition
inner mitochondrial membrane
Term
what is the function of cardiolipin
Definition
meintience of respiratory complexes of electron transport chain
Term
what is cardioipin recognized by, what does that make it
Definition
it is antigenic, recognized by antibodies raised against treponema pallidum
Term
what does treponemia pallidum cause
Definition
syphilis
Term
describe the structure of sphingomyelin
Definition
sphingosine backbone, unsaturated 16 C FA at C3, long chain FA at amino through amide link, C1 of spingosine esterified to phosporylcholine
Term
what is the function of sphingomyelin
Definition
make myelin fibers
Term
what is sphingomyelin a precursor for
Definition
glycolipids
Term
what is the only significant sphingophosphilipid in humans
Definition
sphingomyelin
Term
what does CDP stand for
Definition
nucleotide cytidine diphosphate
Term
what are the two ways you can activate an intermediate in phospholipid synthesis
Definition
donation of phosphatidic acid from CDP diacylglycerol to alcohol.
donation of phosphomonoester of alcohol from CDP alcohol to 1,2-DAG
Term
what is released as a side product of glycerophospholipid synthesis
Definition
cistidine monophosphate (CMP)
Term
where are most phospholipids made
Definition
smoother ER
Term
after made, where do phospholipids go
Definition
organells, plasma membrane, secreted to cell exterior by exocytosis
Term
what is the precursor for many phosphoglycerides
Definition
PA, phosphatidic acid
Term
what substrates do you need to make PA
Definition
glycerol phosphate and two fatty acyl CoA
Term
what is the only cell that cannot make phospholipids
Definition
mature RBC
Term
what does PE stand for
Definition
phosphatidylethanolamine
Term
what does PC stand for
Definition
phosphatidylcholine
Term
what are the must abundent phospholipids in eukaryotic cells
Definition
PC and PE
Term
what are the steps in producing PE and PC from pre-existing pools
Definition
1. kinase phosphorlyation of choline or ethanolamine
2. convert activated form CDP-choline. ethanolamine
3. choling-phosphate or ethanolamine-phosphate is transfered to a molecule is transfered to a molecule of diacylglycerol
Term
what phospholipids can the liver make
Definition
PC, PS, PE
Term
what does PS stand for
Definition
phosphatidylserine
Term
how do you form PC from PE or PS in the liver
Definition
PC from serum lipoproteins is secreted as bile, PS dis decarboxylated to PE by PS decarboxylase, PE is methlyated 3 times to make PC
Term
what does PS decarboxylase need to work
Definition
pyridoxal phosphate coenzyme
Term
why do we need to reutilize choline
Definition
de novo synthesis of choline in humans is not enough, choline is an essential nutrient, choline is used for synthesis of acetylcholin
Term
what is acetylcholin
Definition
a neurotransmitter
Term
what is dipalmitoylphosphatidylcholine, what is its function
Definition
surfactant that decreases surface tension in lungs, reduces pressure needed to imflate aveoli, prevents alveolar collapse
Term
what is another word for aveolar collapse
Definition
atelectasis
Term
describe the structure of DPPC
Definition
palmitate at positions 1 and 2 on glycerol
Term
what is the major lipid component of lung surfactant
Definition
DPPC
Term
what is DPPC made by
Definition
type 2 pneumocytes
Term
what causes respiratory distress syndrome
Definition
insufficient lung surfactant
Term
how can lung formation in babies be accepelated
Definition
mother takes glucocorticoids shortly before delivery
Term
how can respiratory distress syndrome be treated
Definition
natural or synthetic surfactant
Term
how can fetal lung matirity be gauged
Definition
measuring DPPC, sphingomyelin ratio in amniotic fluid
Term
what does a ratio of 2 or higher mean in lung maturity
Definition
mature, sphingomyelin synthesis has shifted to DPPC in pneumocytes
Term
where does lung maturity usually occur
Definition
32 weeks of gestation
Term
why would an adult have RDS due to insufficient surfactant
Definition
surfactant making pneumocytes are damaged by infection or trauma
Term
why is PS made
Definition
base exchange reaction between ethanolamine of PE and free serine
Term
what is PS used for
Definition
membrane synthesis
Term
is PS production reversible
Definition
yes
Term
what is PI
Definition
phosphatidylinositol
Term
describe the structure of PI
Definition
steric acid at C1 and archidonic acid on C2
Term
what is the function of PI
Definition
resivour of arachidonic acid in membranes and substrate of prostaglandin synthesis
Term
what is PI made from
Definition
free inositol and CDP-diacylglycerol
Term
describe the role of PI in signal transduction
Definition
phosphorlyation of membrane bound phosphatidylinositol makes polyphosphoinositides, degration of PIP2 by phospholipase C in response to a variety of neurotransmitters, hormones, and growth factors binding receptors, produces IP3 and DAG
Term
what does GPI stand for
Definition
glycosylphosphatidylinositol
Term
what forms GPI
Definition
covalent link between protein and Pi via a carbohydrate
Term
what is the function of GPI
Definition
bind cell surface proteins, anchor proteins allowing them to increase lateral mobility on surface of plasma membrane
Term
where are cell surface proteins bound by GPI found
Definition
in parasitic protozoans like trypanosomes and leishmania
Term
how can a protein be cleaved rom a GPI anchor
Definition
cleaved by phospholipase C releasing diacylglycerol
Term
what does a deficiency in GPI cause
Definition
hemolutic disease, paroxysmal nocturnal hemoglobinuria in hematopoietic cells
Term
how is phospharitylglycerol and cardiolipin made
Definition
CDP diacylglycerol and glycerol 3 phosphate
Term
what is cardiolipin made from
Definition
2 phosphatidic acid connected by glycerol
Term
what is the precursor for cardiolipin
Definition
phosphatidylglycerol
Term
where is phosphatidylglycerol found in large amounts
Definition
mitochondrial membranes
Term
describe the process of making cardiolipin
Definition
transfer of diacylglycerophosphate from CDP diacylglycerol to a pre-existing molecule of phosphatidylglycerol
Term
how is sphingomyelin made
Definition
palmitoyl CoA condenses with serine, CoA and CO2 released
Term
what does reduction in sphingomyelin production, what is the product at this point
Definition
NADH, sphinganine
Term
what coenzyme do you need to make sphingomyelin, where does it come from
Definition
pyrdoxal phosphate, vitamin B6 derivative
Term
describe the process of sphingomyelin production
Definition
1. sphingosine acylated at amino with a long chain FA desaturated to make cremide
2.phosphorylcholine from phosphatidylcholine is transfered to ceramide making sphingomyelin and DAG
Term
what is a major component of skin and regulates skin water permability
Definition
ceramide with a 30 C FA
Term
what type of FA does sphingomyelin of myelin sheath have
Definition
long chain like lingoceric acid, nervonic acid
Term
what FA does gray made sphingomyelin have
Definition
steric acid
Term
what degrades phosphoglcerides, where
Definition
phospholipases in all tissues and pancreatic juice
Term
what things outside the body act as phospholipases
Definition
toxins and venoms, pathogenic bacteria use it to dissolve membranes and spread infection
Term
what is sphingomyelin degraded by, what is it classified as
Definition
lysosomalphospholipase sphingomyelinase
Term
what do phospholipases do to the molecule
Definition
hydrolyze phosphodiester bonds
Term
what makes phospholipases specific
Definition
each cleaves the phospholipid at a specific spot
Term
what are the major enzymes that degrade phospholipids
Definition
phospholipase A1, A2, C, D
Term
what does lysophospholipase need as a substrate
Definition
lysphosphoglyceride
Term
how is lysphosphoglyceride made
Definition
removal of FA at C1 oe C2
Term
what is the secondary functions of phospholipases
Definition
remodel phospholipids, release molecular messengers like DAG, iP3 or substrates for synthesis of messengers
Term
what is an example of a substrate for synthesis of messengers
Definition
arachidonic acid
Term
what do phospholipase A1 and A2 do
Definition
remove specific FA from membrane bound phospholipids
Term
what replaces after A1 and A2 do its job, what enzyme facilitates this
Definition
alternative FA using acyl CoA transferse
Term
how can unique lung surfactant be made
Definition
replacing with alternativie FA after using A1 and A2 phospholipases
Term
what degrades sphingomyelin
Definition
sphingomyelinase
Term
where does sphingomyelinase come from
Definition
lysosome
Term
how does sphingomyelinase do its job, what happens to the product to complee degredation
Definition
hydrolytically removes phosphorylcholine leaving ceramide which is cleaved by cermidase into sphingosine and FA
Term
what can released sphingosine and FA function in, how
Definition
regulate signal transduction pathways by influencing activity of protein kinase C by phosphorlyating its substrates, promote apoptosis
Term
what type of inheritance is niemann-pick disease
Definition
autosomal recessive
Term
what causes niemann-pick disease
Definition
inability to degrade sphingomyelin
Term
what is deficnent in niemann-pick disease
Definition
sphingomyelinase a type of phosphilipase C
Term
what are the symptoms of type A neimann-pick disease
Definition
lipid deposits in liver and spleen, liver and spleen enlarged, sphingomyelin cannot be degraded, severe infantile
Term
what happens to infants with severe lysosomal storage disease, why
Definition
rapid neurodegeneration due to sphingomyelin in CNS deposition causing early death
Term
what are the symptoms of type C niemann-pick disease
Definition
little to no damage to neural tissue, lungs spleen liver and bone marrow affected making it chronic, life expectancy to adulthood
Term
what population is more likley to have type A neimann pick disease
Definition
ashkenazi jewish population
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