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where in a eukaryotic cell does fatty acid synthesis occur?
cytosol
where does oxidation of very long-chain and branched fatty acids take place?
peroxisomes
genetic deficiency of _______ (enzyme) is responsible for 10% of all SIDS cases
Medium chain acyl CoA DH
what is the target of the statin class of cholesterol lowering drugs and how do they work?
HMG-CoA Reductase through competitive inhibition
NSAID naproxy sodium (alleve) works as a _____ inhibitor of _____
competitive inhibitor of COX 1 and COX 2
Bile salts are derived from which lipid?
phosphatidylcholine
what is the correct sequence of the four fatty acid spiral (4 steps of beta oxidation) in terms of functional group "ACTED UPON"
alkane, alkene, 2nd alcohol, ketone
The carbon atoms from a fatty acid with an odd number of carbons will enter the citric acid cycle as:
as acetyl-CoA and succinyl CoA
What enzymes are involved in the citrate (tricarboxylate) transport system
pyruvate carboxylase in mito matrix
citrate synthase in mito matrix
malate DH in cytosol
ATP citrate lyase in cytosol
a deficiency of which vitamins will make FAS (fatty acid synthesis) not possible?
B3, B5 and B7
what is the source of reducing power for fatty acid synthesis
NADPH
what is the main control point of beta oxidation (fatty acid oxidation) and what is it inhibited by?
Carnithine palmitoyltransferase 1 (CPT 1) ; malonyl-CoA
What activates hormone sensitive lipase (HSL)
(directly): phosphorylation by PKA
(indirectly): epinephrine, glucagon, beta-lipotropin
what is the role of HSL (hormone sensitive lipase)
hydrolyze triacylglycerols stored in adipose tissues
how do medicines Wegovy, Ozempic and Mounjaro work as weight loss drugs and/or treat Type 2 diabetes?
activation of the GLP-1 receptor
to oxidize the fatty acid linoleic acid- what enzymes are needed in addition to the enzymes needed for beta oxidation?
BOTH enoyl-CoA isomerase and 2,4-dienoyl-coA reductase
What is the NET # of ATP if caprylic acid (C8:0) is oxidized completely (through beta oxidation and onwards)
50
If malonyl-CoA is synthesized from 14CO2 and unlabeled acetyl-CoA, and the labeled malonate is then used for fatty acid synthesis, the final product (fatty acid) will have radioactive carbon in:
no part of the molecule
the sphingolipid catabolism disease caused by a defective glucocerebrosidase is;
Gauchers
_____ are lipoproteins that transport dietary lipids from the intestines to other locations in the body
chylomicrons
what inhibits acetyl coa carboxylase (ACC) and subsequently inhibits fatty acid synthesis (FAS)
AMP-activated protein kinase
what catalyzes the formulation of butyryl ACP from crotonyl ACP in fatty acid synthesis
enoyl reductase
which step in beta oxidation (fatty acid oxidation) involves a reverse claisen condensation
beta-ketothiolase
which ketone body is formed by slow, spontaneous decarboxylation of acetoacetate?
acetone
The synthesis of both glycerophospholipids and triacylglycerols involves ____ as an intermediate
phosphatidic acid
T/F: the building blocks for sphingosine biosynthesis are threonine and palmitoyl coa
FALSE
what are the essential amino acids
histidine, isoleucine, leucine, lysine, methionine, phenylalanine, threonine, tryptophan, valine
PVT TIM HaLL (histidine only adults)
What AA are ketogenic?
Lysine and leucine
which AA are BOTH ketogenic and glucogenic?
IPTTT, isoleucine, phenylalanine, tryptophan, tyrosine, threonine
can glutamate DH carry out reaction in either direction?
yes
what AA get catabolized into pyruvate
cysteine. alanine, glycine, threonine, tryptophan
deficiency in what vitamin in pregnancy results in neural tube defects like spina bifida?
folic acid (B9)
what enzyme catalyzed reactions involve THF or a THF derivative (ex: N5, N10-methylene THF, N5, methyl THF)
glycine cleavage system (GCS)
reactions catalyzed by dihydrofolate reductase (DHFR)
reactions catalyzed by MTHFR
conversion of homocysteine to methionine by methionine synthase
serine hydroxymethyl transferase (SHMT)
what AA gets catabolized into alpha ketoglutarate
arginine, histidine, proline, glutamine
mutation in which enzyme results in genetic disorder : Phenylketonuria (PKU)?
phenylalanine hydroxylase (PAH)
what metabolite is an activator of the main control point in the urea cycle?
N-Acetyl glutamate
which urea cycle enzymes involve the urea cycle metabolites (both reactants and products) that are ONLY IN CYTOSOL
argininosuccinase
the first amino group entering the urea cycle comes from:
either glutamate or glutamine
net products of the urea cycle are:
one molecule of urea, one molecule of AMP, two molecules of ADP and one molecule of fumaric acid
what building block (molecule) does the tau nitrogen on the His ring come from?
ATP
what is a building block needed to make all 3 aromatic amino acids (Phe, Tyr, Trp)
erythrose-4-phosphate and PEP (phosphoenolpyruvate)
glutamine is converted to glutamate and NH4+ by
glutaminase
what is the correct order in the catabolism of methionin into the krebs cycle?
Met --> SAMe --> homocystine --> alpha KB --> succinyl coa
the catabolism of all three benzene ring containing aromatic acids (Trp, Phe, and Tyr) involve several reactions involving _________
Q2
how does glyphosate affect amino acids in plants
competitive inhibitor of the synthesis of chorismate in plants
disrupts shikimate pathway
the first step in the catabolism of most L-amino acids once they have reached the liver is promoted by _________
aminotransferases in the cytosol
what is the main control point of AA biosynthesis?
glutamine synthetase
what substrate does glutamine synthetase use (for E)
ATP
what EC classification is glutamine synthetase?
EC 6 ligase
what AA is needed to provide part of the carbon skeleton for the biosynthesis of heme?
glycine
does serine dehydratase catalyze a transamination reation?
no
does serine dehydratase involve a deamination?
yes
what cofactor does serine dehydratase require?
PLP cofactor
what does serine dehydratase convert ____ to _____
serine to pyruvte
trypsinogen is activated by
enterokinase and trypsin