EXAM 1 Flashcards
Gly
Glycine
Ala
Alanine
Val
Valine
Leu
Leucine
Ile
Isoleucine
Phe
Phenylalanine
Trp
Tryptophan
Met
Methionine
Pro
Proline
Asp
Aspartate
Glu
Glutamate
Ser
Serine
Thr
Threonine
Asn
Asparagine
Gln
Glutamine
Cys
Cysteine
His
Histidine
Lys
Lysine
Arg
Arginine
Acidic aminos acids are ?
Aspartate (asp)
Glutamate (glu)
Basic amino acids are?
Histidine (his)
Lysine (lys)
Arginine (arg)
Polar amino acids are ?
Serine/ ser Threonine/ thr Tyrosine/ tyr Asparagine/ asn Glutamine / gln Cysteine/ cys
Nonpolar aminos acids are?
Glycine/ gly Alanine/ ala valine / Val Leucine / leu Isoleucine / ile Phenylalanine / phe Tryptophan / trp Methionine / met Proline / pro
T/F only L form of amino acids are used in human metabolism
T
Racemic mixture is?
Means there is a 1 to 1 ratio L and D isomers of the substance
Eg. racemic mixture 100mg of glycine
50 mg of L 50 mg of D (D not useful)
Essential amino acids?
Phenylalanine
Valine
Tryptophan
Threonine
Isoleucine
Methionine
Histidine
Lysine
Leucine
Conditionally essential AAs?
Arginine
Glutamine
Glycine
Taurine
Non essential amino acids are?
Alanine Asparagine Aspartic acid Cysteine Glutamic acid Proline
When should we test amino acids?
Neonatal screening Poor digestion High cholesterol Alzheimer’s Chronic fatigue Aging Cancer Pregnancy
2 exclusively ketogenic amino acids
Lysine
Leucine
AA that is principle supplier of nitrogen in the body
Glutamine
Protein turnover leads to hydrolysis and resynthesis of Xg of protein a day
300 to 400
2 major enzyme systems responsible for protein degradation
- Ubiquitin-proteasome system cytosol ATP dependent
2. Lysomal enzymes ATP independent
Typical grams of protein consumed daily in American diet
70-100
T/F only free amino acids are found in the portal vein after a meal
T
T/F the concentration of free amino acids is lower outside the cell than inside ?
False
T/F cystinuria is the most common genetic error of amino acid metabolism
t
2 sources of urea nitrogen
Ammonia
Aspartate
Allosteric regulator of the urea cycle?
NAG/N-Acetyl-glutamate
Where are 2 nitrogen’s provided from to form urea?
- Free ammonia (NH3)
2. Aspartate (ultimately from glutamate)
T/F hyperammonemia is toxic to the CNS?
T
Glucogenic amino acids are amino acids whose catabolism yields X or Y?
X = pyruvate Y = other intermediate of Citric Acid Cycle
*** THEN PRODUCE GLUCOSE VIA GLUCONEOGENESIS
T/F Glucogenic amino acids produce glucose via glycolysis?
F; thru gluconeogenesis
Branch chain amino acids are?
Valine
Leucine
Isoleucine
BCAAs are good for what type of activity?
Strenuous muscle activity; create a flow of alanine and glutamine from skeletal muscles
Those AAs then used for gluconeogenesis
T/F Valine and Isoleucine used to creat succinate? Why is that important?
T
Succinate is TCA intermediate (create energy)
Elevations in BAIB present with what conditions (x3)?
- B6 deficiency
- Leukemia
- BAIB pyruvate transaminase deficiency
Amino acids involved in heme, nucleotide, and cell memebranes
Glycine
Alanine
Aminotranferase/transaminase enzymes always requires what b vitamin cofactor?
B6
Function of phosphotidalcholine/lecithin
EMULSIFIES FAT
What is the most toxic of amino acids?
Methionine
What is the PRIMARY function of amino acids?
Protein synthesis
Responsibilities of mTOR? (3)
Mitochondrial retraction (when mitochondria shrink for survival reasons during INCREASED OXIDATIVE STRESS)
Deficiency in SULFUR AMINO ACIDS and IRON (mTOR ^ when Sulfur/Iron down)
Reversing Chronic Hypometabolic Compensatory States
When to use amino acid testing? (9)
- NEONATAL SCREENING
- Poor digestion
- High cholesterol
- Alzheimer’s
- Chronic fatigue
- Chronically ill
- Aging
- Cancer
- Pregnancy
Arginine requires what mineral as a cofactor?
MANGANESE
What is the most dominant free amino acid in fasting plasma
GLUTAMINE (gln)
Signs of ARGININE deficiency
- HYPERAMMONIA (confusion, disorientation)
- Rash
- Hair loss
- Poor wound healing
- FATTY LIVER
Function of ADMA
ADMA INHIBITS the synthesis of Nitric Oxide
Low NO = Endothelial dysfunction
What condition is associated with a SNP to the ORNT1 gene?
“HHH”
Hyperammonemia
Hyperargininemia
Hyperornithinemia
T/F Treatment of HHH requires a low protein diet, B6, and Mg?
T
What reaction does L-Asparaginase catalyze?
Asparagine —> Aspartate + Ammonia
What conditions should you AVOID supplementing with GLUTAMINE (GLN)? (3)
Cancer
Liver disease
Chron’s
Is GABA inhibitory or excitatory?
INHIBITORY
Large neutral amino acids are?
Met Phe Tyr Trp Leu Ile Val
Methionine synthase requires which cofactors? (3)
B9
B12
Betaine
What nutrients are needed for the Branched Chain Ketoacid Dehydrogenase Complex? (5)
- B1
- B2
- B3
- B5
- Lipoic acid
What is the cofactor for BCAA Transaminase (hint: “when in doubt”)
B6
Uses of threonine (3 main)
- Protein synthesis
- MS (manage spasticity)
- Glutathione generation
What is the cofactor for histidine decarboxylase? (hint: when in doubt)
B6
Symptoms of prenatal protein deficiency (hint: thrift)
- Increased eating
- Increased preference for calorie rich foods
- Increased IR, hypertension, adiposity, glucose intolerance
- Activation of THRIFT GENES leading to Met Syn
How to perihepatic portal cells respond to increased local acidity? (Hint: it transport Gln through SN1-SN2 transporter to create what 2 THINGS?)
Ammonia?
HC03
Elevations in BAIB may be present in which condition?
Leukemia
Contraindications of Trp? (3 conditions you should avoid supplementation?)
- SSRIs
- Pregnancy
- Asthma
What nutrients are needed for the creation of hydroxyproline and hydroxylysine?
Proline
Lysine
VITAMIN C
Dietary collagen
What does Methionine adenosyltransferase enzyme catalyze?
The formation of SAMe
Tryptophan metabolism requires what 4 cofactors?
- BH4
- Fe
- B6
- B3
Know the difference between histamine being degraded in
- The liver
- Via extra hepatic-pathways
Enzymes?
Cofactors?
- Histamine —> Imidazole acetaldehyde
ENZYME = DAO COFACTOR = Copper, B2
- Histamine —> SAH + N-Methylhistidine
ENZYME: Histamine N-methyltransferase
COFACTOR = SAMe
Enzyme that converts Histidine to HistaMINE
Histidine decarboxylase
Histamine degradation: LIVER (enzyme/cofactor)
LIVER
E: DAO
C: Copper + B2/FAD
Histamine depredations: EXTRA HEPATIC
E: Histamine N-methyltransferase
C: SAMe
What amino acid should you NEVER supplement with?
Glu/Glutamate
T/F serine is a precursor for the synthesis of phospholipids (cell membranes)
T
High ethanolamine may reflect poor X status? (Hint: methylation)
SAMe
Amino acids involved in BONE/COLLAGEN
PROLINE
P AA that creates “bend” in the protein
Proline
Collagen metabolism is inhibited by x & Y?
LACTATE
CADMIUM
Are amino acids elevated at night or in the morning?
MORNING
Common amino acidopathies
PKU
Maple syrup urine disease
Homocystenuria
Cirtulle
High cholesterol associated with low levels of what 2 AAs?
Arginine (arg)
Glycine (gly)
If there is a build up of orotate, what enzyme is implicated?
Carbomoyl phosphate synthetase (CPS)
Hyperammonemia is most common clinical sign of which enzyme deficiency involved in the urea cycle ? (Hint: O)
OTC (ornithine transcarbomoylase)
X is required for BCAAs to be incorporated into sketetal muscle? (Hint: sugar)
Insulin-like growth factor 1 (IGF-1)
Maple Syrup Urine disease associated with genetic mutation of which enzyme ? (What would be elevated in urine)?
BCKDC
Branched Chain KETOACIDS (not amino)
What nutrients may be deficient when AANB is elevated?
Thr
T/F In the CNS, histamine can be used as a neurotransmitter? (Critical function eg. Aroused)
T
Control over arousal/sleep
Individuals with Down syndrome have what AA deficiency? (Eg. L)
Lysine
PKU means there is a problem converting Phe to ??
Tyr
T/F Indican is a marker of bacterial metabolism of Trp?
T
NMDA v GABA (neuro inhib or excitatory?)
NMDA = excitatory
GABA = inhibitory
Is oxidative stress high or low is homocysteine goes down trans-sulfuration pathway?
HIGH
Methionine is best absorbed with a little bit of ?
CARB
Non animal source of SAMe?
Sunflower seeds
Low Hcy would impair production of?
Glutathione
High homocysteine effects which 3 body systems?
CNS
Musculoskeletal
Cardiovascular
