Final PP 1 Flashcards
What is needed for the transport of FA from the cytoplasm to mitochondria?
ATP
Carnitine
Co-A
Write down the enzymes of urea cycle+localization
Carbomyl -p synthase (mitochondria) Ornithine carbomyl transferase (mitochondria) Argino-succinate synthase (cytosol) Argino-succinate lyase (cytosol) Arginase (cytosol)
LCAT action?
Catalyze esterification of cholesterol to form cholesterol ester
What is the reaction catalyzed by Glu DH?
Glu + NAD+ +H2O -> NH4 + a-KG +NADH +H
Give examples for enzymes that are activated upon phosphorylation
Glycogen phosphorylase
Phosphorylase kinase
What AA can be converted to serine in a single reaction?
Glycine
When does -plasma level of unesterified FA is increased?
Starvation
By glucagon
By ACTH
Localization and action of Acyl co-A DH?
Mitochondria
Trans double bond
What takes part in intestinal lipid digestion?
Pancreatic Lipase and bile acids
Human genetic PKU can result in:
Inability to convert Phe to Tyr
Write down the reaction catalyzed by Adenosine Deaminase
Adenosine +H2O -> Inosine +NH3
Action of ABCA1?
Efflux of cholesterol from the cell
What is the enzyme that catalyze
Phe -> Tyr
Phe Hydroxylase
What is the activity of Lipoprotein Lipase?
Hydrolysis of TAG of plasma Lipoproteins to supply FA to the tissues
Von Gierke disease is a result of the deficiency in
G-6-Pase
What is the role of Glutathione reductase in RBC?
It is needed for the removal of H2O2
What is the reaction catalyzed by HMG coA Reductase?
What is needed?
HMG coA -> Mevalonic acid
NADPH is needed for the reaction
What is the consequences of Acyl coA deficiency?
No ketone bodies synthesis
When do bile pigments are formed?
In the degradation of Heme
Orotic aciduria (inherited metabolic disease) when orotic acid is accumulated in the body Occurs in which metabolic pathway?
Pyrimidine synthesis
What stimulates the transcription of ABCA1?
High intracellular conc of cholesterol
Where does B oxidation occurs?
Mitochondria
What apolipoprotein is an activator of LCAT?
Apo A-1
What is essential for the synthesis of FA?
CO2 and HCO3-
Lipoprotein Lipase activity?
Hydrolyses TAG from VLDL and chylomicrons
Enzymes of urea cycle are located in:
Mitochondria
Cytosol
Which aAA will enter the citric acid cycle as a-KG?
GAG PH
Gln Arg Glu Pro His
Substrates for ribonucleotide reductase?
ADP
GDP
CDP
UDP
Write down 3 inhibitors of Gluconeogenesis
F-2,6-BP
ADP
AMP
Which of this two will produce mitochondrial NADH?
Pyrimidine de novo synthesis
Purine de novo synthesis
Pyrimidine de novo synthesis
Enzyme converting inosine to hypoxanthine?
PNP (Purine Nucleoside Phosphorylase)
Which enzyme catalyzes the rate limiting step of cholesterol synthesis?
HMG coA Reductase
What happens to conjugated bilirubin in the serum during liver disease?
Decreased
What can we use as HMG coA Reductase inhibitors?
Statins
Which enzyme is allosterically modulated by malonyl co A?
Inhibition/activation?
Carnitine palmtoyl transferase 1
Inhibited
Where does the synthesis of NO starts from?
Arginine
What AA participate in the synthesis pf purine nucleotides?
Gln
Asp
Gly
How many subunits does PKC have?
1-single polypeptide chain
How many units does PKA have?
Tetramer
What receptor in the CNS function as Ca channel?
NMDA
What provides the methyl group here-
NE -> E
SAM
What is Glutathione?
Tripeptide made of Glu, Cys, Gly
What is the consequence of inherited Adenosine Deaminase deficiency?
Immunodeficiency
What are the negative allosteric effectors of PFK1?
ATP
Citrate
H
What is stimulated under high conc of F-2,6-Bi-P
Glycolysis
Upon phosphorylation of PFK2 by PKA it is-
Inactivated, and the function of the FBPase is activated
The glycogen branching enzyme catalyzes-
Formation of a1 -> 6 linkage
What can induce gluconeogenesis in the kidney cortex?
Catecholamines
What are the glucose transporters of the brain?
GLUT1 and 3
Who has more mitochondria, astrocytes or neurons?
Neurons
What is the action of AMP-activated protein kinase in the muscle?
It phosphorylates acetyl co-A carboxylase and decreases malonyl co-A concentration in the muscle
Describe the GABA shunt?
KG-Glu-GABA-succinate semi aldehyde-succinyl coA
What activates tPA? (2)
- Fibrin
- some of the plasmic fibrin degregation products stimulates it
What provides precursor for the synthesis of nucleotides?
PPP
Epinephrine triggers an increase rate of glycogen breakdown in muscle by causing-
Conversion of glycogen phosphorylase b to a
Write down enzymes that needs Biotin as a prosthetic group (3)
Pyruvate carboxylase
Acetyl coA carboxylase
Propionyl coA carboxylase
Following glycogenolysis, G1P is converted to
G6P
Precursor for the biosynthesis of pyrimidine ring system includes:
Carbomyl phosphate and Asp
LPL htdrolyze TAG from?
mature chylomicrons and VLDL
Glu -> aKG is reductive deamination OR reductive amidation?
reductive amidation
which proteins/enzymes participate in the reverse cholesterol transport?
Apo A1 and LCAT
what is the consequence og mutation of the Ryanodine receptors?
malignant hyperthermia
what are the characteristic of malignant hyperthermia?
rigidity of skeletal muscles
which requires more than one step?
Ala->Pyruvate Phe->Succinate Asp->OAA Glu->aKG Pro->Glu
Pro->Glu
Phe->Succinate
where do Heme Oxygenase plays a role?
in the degregation of Heme
which of the next signaling components play a role in the action of glucagon?
- B receptor
- PKA
- cAMP
- Gs protein
- Gi protein
- PKA
- cAMP
- Gs protein
(B receptor is for NE)
what are leptins?
polypeptides produced by the adipose tissue
clinical symptoms of antiphospholipid syndrom can be treated wirh? (2)
vit K antagonist
Heparin
what is the first step of fructose metabolism?
phosphor of fructose to F-1-P
order this by ATP energy
Ala
palmitate
glucose
palmitate>glucose>Ala
What is the subunit composition of the ganglion type nicotinic receptor?
ax2
bx3
What activates Tyr hydroxylase?
Ca
Put this in the right order of GABA shunt
GABA Succinate semialdehyde Glu aKG Succinyl co A
KG->Glu->GABA->succinate semialdehyde->succinyl co A
Substrates for thrombin in blood coagulation?
Fibrinogen V VIII XI XIII Platelets
What is the primary target of strophantin?
Other name?
Ouabain
Its a cardiac glycoside
Inhibits Na/K ATPase
Write down the mechanism of action of Ouabain:
Na/K ATPase inhibition ->accumulation of intracellular Na -> reduced activity of Na/Ca exchanger -> elevation of intracellular Ca -> higher cardiac contractility
Open probability of ion channel can range from -
0-1
Phosphorylation decrease/increase the activity of CFTR Cl channel
Increase
The gating charge in the voltage sensor of voltage gated K channels is provided by conserved _____ and _____ residues on the ____ helix
Arg
Lys
S4
Myasthenia gravis happens because-
Autoantibodies directed against the nicotinic acetyl choline receptor
How is the formation of blood coagulation complexes inhibited in the clinic?
Vitamin K inhibitors
What kind of molecule exerts irreversible inhibition of Ach esterase?
Organic phosphate
What does phosphorylated CFTR Cl channel need for gating?
ATP
The gate of the K channel id located close to the _________ side
Intracellular
How does dopamine influences the activity of Na/K ATPase in the kidney?
Decreases
Name the metabolite that is formed in the brain in the metabolism of NE and can be detected in the urine
MHPG
What is the coenzyme for methylmalonyl mutase?
B12
Amino acids with 4 carbons?
Asn
Asp
Thr
What happens to Vmax in the presence of competitive inhibitor?
Stay the same
What happens to Vmax in the presence of non-competitive inhibitor?
Decreases
what does the deficiency in branched-chain α-ketoacid dehydrogenase complex causes?
Maple syrup disease
two NADPH producing enzymes of the PPP?
G-6-P DH
6-phosphogluconate DH
two pathways for arachidonic acid?
Cycloxygenase
Lipoxygenase (produces leukotriens)