Brainscape's Knowledge GenomeTM


Top Flashcards (Certified)
All Flashcards

Biochemistry > FA Metabolism > Flashcards

FA Metabolism Flashcards

1
Q

Lipases in Body

A

lingual- small amt of fats
stomach- short chain
Pancreas- in duodenum, most fat, co lipase & BA= 2 monoacylglycerol

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
2
Q

Lipoprotein lipase

A

outside endothelial & adipocytes. Chylomicrons & VLDL. Fat taken up by FA transport protein & CD36 or diffusion. LPL expression stimulated by insulin (adipose) and glucagon + epi in other tissues.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
3
Q

Adipose tissue triglyceride lipase ATGL

A

cleaves triacylglycerol to diacylglycerol & FA

Activated by PKA

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
4
Q

Hormone sensitive lipase HSL

A

Cleaves triacylglycerol & diacylglycerol to FA & monoacylglycerol in adipocytes, I by insulin, stimulated by somatotropin, epi & glucagon using PKA
PKA aslo hyperP perilipins (non P= inhibit lipase access to lipid droplets)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
5
Q

Monoacylglycerol lipase

A

in adipocytes, cleaves mono acylglycerol into FA & glycerol
Glycerol transported to liver & enters glycolysis via glycerokinase & a glycerophosphate dehydrogenase
PKA activates it

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
6
Q

FA degradation

A 
Short < 6 C
medium 6-12
long 14-24
very long >24 C 
most cells but not in nerve (can't cross BBB)
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
7
Q

FA activation

A

Acyl coA synthetase (thiokinase) makes FA adenylate whichs then converts to acyl Co A using thiokinase AGAIN

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
8
Q

Carnitine shuttle into mitochondrium

A

carnitine formed in liver & kidney from lys & met
<12 C atoms pass by diffusion
rate limiting step of FA breakdown

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
9
Q

Systemic primary carnitine def

A 
renal re uptake transport OCTN2 (Na+) 
Hypoketotic hypoglycemia
Reye syndrome
Lethargy
HEpato & cardio megaly
Give carnitine orally
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
10
Q

Carnitine palmitoyltransferase I def

A

hypoketotic hypoglycemia, Reye, heptaomegaly, m. weakenss

Prevent hypoglycemia with shorth & medium chain FA in diet

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
11
Q

Carnitine acylcarnitine translocase def

A

presents w/in hours of birth
Seizures, bradycardia, breathing problems, sudden infant death
Low lipid intake, med chain FA, avoid excercise, carnitine

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
12
Q

Carnitine palmitoyltransferase II def

A

Adult: m. problems, attacks during exercise, fasting, high fat diet, infection. Variable w/ age
Infantine- <1 yo, hypoketonic hypoglycemia, loss of consciousness & seizures
neonate- onset hours to days after birth, rapidly fatal. Neuronal migration defect

Low lipid intake, med chain FA, carnitine,avoid excercise
Bezafibrate- induce enz expression

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
13
Q

Peroxisome Proliferator activated R

A

nuclear R proteins that bind FFA, eicosanoids etc. dimerize w/ RXR & control expression of increased number of peroxisomes.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
14
Q

Jamaican vomiting sickness

A

Posioning from MCPA-Co A inactivates carnitine acyl transferase I & II.
Short chain acyl CoA dehydrogenase also (NADH production)
Treat: supportive

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
15
Q

B oxidation

A

occurs in mitochondrial matrix
Forms acetyl Co A from acyl CoA (which then gets reformed and recycled)

remove H to form 2x bond @ FADH2
add H2O to 2x bond
Reduce alcohol to ketone, form NADH
Make/break C-C on carbonyl C using coA- SH as energy

*can use w oxidation but not major pathway!

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
16
Q

Unsaturated FA

A

problems in B oxidation
Cis double bonds
Use 3 additional enzymes to change 2x bonds from wrong direction

17
Q

Propionic Acidemia

A

defect in propionyl co A carboxylase, frequent in Mid east.
Vomiting, ketoacidosis, pancytopenia, hypogammaglobulineamia, protein intolerance
Treat: low protein diet

18
Q

Methylmalonic Aciduria CBL

A

Defect mitoch transport of B12 or defect conversion to adenyl form
Acidosis
Megaloblastic Anemia
Treat: B12

19
Q

Methylmalonic aciduria mut

A

acidosis only , no anemia
Sudden infant death
Therapy: nutritional manage, treat infections, carnitine

20
Q

SCAD

A

generalized or muscle only. Metabolic acidosis, fialure to thrive, dev delay, seizures, myopathy
NO HYPOGLYCEMIA

21
Q

MCAD

A

no FAD binding, after fasting or stress, fatal hypoglycemia (high gluconeogenesis, low KREBS), mitoch changes
Long term= slowed cognitive dev, cerebral edema, encephalopathy, no exercise, fatty liver
10% sudden infant death

22
Q

LCAD

A

usually with VLCAD

23
Q

VLCAD

A

missense mutations/deletions, some respond to bezafibrate.
Mem associated protein
Cardiomyopathy, non ketotic hypoglycemia, hepatic disf, sudden death
En handles upper 14-24 C not very long >24 C

24
Q

TFP

A

mitochondria tri functional protein
long change, hydroxyacyl Co A dehydrogenase & thiolase complex.
One is defective but rarely all 3 are.
Fulminant neonatal liver failure
Preg may lead to acute fatty liver of pregnancy in mother.

25
ABCD1
adrenoleucodystrophy ABCD proteins are ABC type half transporters forming dimers Transport long & very long FA into peoxisome X linked Fail to metabolize VLCFA leads to accum in brain & adrenals= demyelinization of white matter= death in adolescence Loss of vision, hearing, neuro Addison's disease Lorenzo's Oil
26
Zellweger Syndrome
Defect in peroxisome formation genes Cerebrohepatorenal syndrome- facial features, polycystic kidneys, liver, CNS probls, chondral calcification, high Fe & Cu, elevated pipecolic acid Type 1= disparity b/t serum & cell results type 2= disparity in matrix protein import into peroxisomes in adj cells from same indivs No cure! Death w/in 1st half year
27
Adult refsum disease
progressive cerebellar degenration & periph neruopathy, retinitis pigmentosa, cataract, ataxia, diff hearing Caused by phytanoyl CoA dioxygenase or peroxin 7 mutations Treat by restricting fish fat & plasmapheresis
28
Ketone Bodies
Liver turns unneeded FA into ketone bodies occurs when Glc supply is low ketone bodies tranported by blood to target organs like m. , heart Ketone body [ ] low to cross BBB In long term starvation= 2/3 of brain E Ketoacidosis= excess ketone bodies in urine
29
FA syn
convert carb to fat via acetyl Co A | liver, milk glands, adipose tissue, kidney & lung
30
Malate shuttle
acetyl co A in mitochondria from glucose, transported to cytosol for FA syn (1 ATP) Malate dehydrogenase- oxaloacetate & NADH to malate & NAD+ Malic enz- malate & NADP+ to pyruvate & NADPH Pyruvate carboxylase= pyruvate to oxaloacetate & ADP
31
Pyruvate Carboxylase Def N Amer group
lactic, pyruvate & alanine acidemia Severe metnal, psychomotor & dev retardation Inactive enz Treat with thiamin, lipoate & dichloroacetate to utilize PDH
32
Pyruvate Carboxylase Def French
resp distress, increased serum, lactate, ammonia, pro, lys intracell redox distrubance cytosolic more red than mitoch which is more ox don't survive past 3 months No enz
33
Pyruvate Carboxlase Def Benign
preservation of motor & metnal abilities metabolic acidoss w/ elevated lactate, pyruvate, alanine etc. (rehydrate & bicarb) Anemia with increased reticulocytes R shift O2 curve due to increase in 2,3 BPG
34
FA synthesis
empty enz rechard w/ acetyl Co A Enzy contains 4 phosphopantetheine as acyl binding & cys SH syn like B ox but w/ NADPH in cytosol reducing from PPP & malic enz acetyl coA carboxylase I by palmitate & cAMP, stim by citrate unsat FA & insulin decrease acetyl Co A carboxylase & FA synthase
35
Polyketide
acetyl or propionyl Co A condensed w/ malonyl or methylmalonyl co A Gets additional modification (cyclization, methylation) Used as antibiotic, chemotherapuetic, immunosuppresant Erythromycin, amphotericin etc.
36
Elongation & desaturation
FA activated to acyl co A, then react with malonyl co A | Then oxidase introduces OH groups, H2O removal to form 2x bond on some C
37
Regulation
FA syn & B oxidation must not occur at same time. FA syn in cytosol, breakdown in motchondria Malonyl Co A shuts down carnitine shuttle FA syn regulated @ acetyl co A carboxylase: citrate allosteric insulin: deP, activation glucaon & epi- increase cAMP- increase P- inactivation gene expression- more insulin, less glucagon

Biochemistry (8 decks)

Brainscape helps you reach your goals faster, through stronger study habits.
© 2025 Bold Learning Solutions. Terms and Conditions