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50 Cards in this Set
- Front
- Back
Essential Amino Acids
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Pvt Tim Hall
Phenylalanine, Valine, Threonione, Tryptophan, Isoleucine, Methione, Histadine, Argine, Leucine, Lysine |
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What is tyrosine synthesized from and why is it important?
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Phenylaine
Used in signal transduction Reciever of Pi group Used for synthesis of Catecholamine (NT) Presursor to Melanin |
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What does cysteine require for synthesis?
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The sulfur from methinoine
It is good for treatment of hangovers |
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Arginine
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Important in cell division and wound healing
Immediate precursor of Nitric Oxide Not required in adults |
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Non-essential AA
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Produced from carbs via intermediates of TCA or Acetyl CoA
Folllowed by tansamination to obtain Amino Nitrogen |
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Kwashiorkor
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Infant is weaned from breast milk onto a starchy portein poor diet
Poor growth, muscle wasting, edema, diarrhea, increased infection |
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Marasmus
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Inadequeate caloric intake
Elderly Alcoholics |
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Pepsin
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Secreted as pepsinogen by cheif cells
Starts cutting long chains into smaller peptides |
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Enteropeptidase
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Enzyme in the intestine that activates trypsinogen into trypsin which in turns activates more pancreatic enzymes to break AA into di and tri-peptides
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Enterocyte
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Where di and tri peptides are hydrolyzed to their amina acids and the free ones are released into the portal system
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Endopeptidases
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Hydrolyze peptide bonds within the peptide chain
Pepsin,Trypsin, Chymotrypsin, Elastase |
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Aminopeptidases
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EXOPEPTIDASE
Remove AA from the N-terminus |
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Carboxypeptidase
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EXOPEPTIDASE
Remove AA from the C-terminus |
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Absorbtion of AA
Seconday Active Transport |
Na+ dependent carriers Transport Na+ and AA TOGETHER INTO the cell.
Na+ is pumped OUT in exchange for K+ by Na+,K+-ATPase On serosal side the A is carried by facilitated transporter DOWN its conc. to blood |
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Absorbtion of AA
Gamma-Glutamyl Cycle |
GGT (transpeptidase) on the extracellular side reacts with a AA bring it inside the cell
Glutathione reacts to form a complex Glutathione is then re-synthesized which requires energy |
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Glutathione
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Helps cells in response to stress
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What AA's does the body circulate Nitrogen in?
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Mainly Glutamine and Alanine
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What do you need to remove to use AA for energy?
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α-amino group
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Transamination
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α-amino groups are transfered off an AA and onto α-ketoglutarate to form Glutamate leaving behind a α-keto acid
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What groups are linked in the Transamination
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Alanine - Pyruvate
Glutamate - α-ketoglutarate Aspartate - Oxaloacetate |
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What AA can NOT undergo Transamination and what cofactor is involved in teh rxn?
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Lysine and Threonine
PLP Vitamin B |
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AST
ALT |
Aspartate - Oxaloacetate
Alinine - Pyruvate Liver function is tested by these ALT is more specific but AST is more sensitive |
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Deamination (Oxidative)
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Oxidation/reduction rxn that removes the NH4+
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Glutamate Dehydrongenase
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Emzyme that removes the ammonia ultimately from Glutamate
Requires NAD or NADP Reversible |
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Activators of Deamination
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ADP and GDP push rxn towards α-ketoglutarate
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Inhibitors of Deamination
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ATP and GTP push rxn towards glutamate
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Where do the two Nitrogens for the Urea cycle come from?
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NH4+ from deamination of glutamate and from Aspartate
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Where do the first 2 rxns of the urea cycle occur and then where do the rest?
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First 2 in the mitchondria
The rest in the cytosol |
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What enzyme is used in Rxn 1 of the urea cycle?
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CPS-1 which is the rate limiting step requires ATP
Activated by large amounts of AA in circulation |
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What enzyme is used in Rxn 2 of the urea cycle and what happens if their is a defect?
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Ornithine Transcarbamoylase
A complete deficiency is death and partial leads to increase Ammonia, respiratory alkalosis |
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What connects the Urea Cycle and TCA
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Dynamically connected by intermediates being pulled off and shuttled in
Aspartate - Oxaloacetate Fumerate - Maltate |
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Activation of Carbamoyl Phosephate Synthetase (CPS-1)
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Arginine stimulates the synthesis of NAG which stimulates CPS-1
NAG has no other known functions |
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Glucogenic
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AA used to produce glucose
Also produce intermediates in the TCA |
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Ketogenic
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AA used to produce Ketone Bodies or Acetyl CoA
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BCAA
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Valine, Leucine and Isoleucine
Favorite of muscle NOT used by liver, gut |
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Valine
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Glucogenic
Forms Succinyl CoA Used for energy or converted to Glucose |
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Leucine
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Ketogenic
Forms Acetyl CoA Used for energy or production of Ketone Bodies |
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Isoleucine
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Forms both Acetyl CoA and Succinyl CoA
Carbon skeleton can be used for both glucose or ketone or used for energy |
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PKU
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Deficiency in Phenylalanine Hydroxylase Causing Phe
Mental retardation Failure to catch early results in drop of 1 IQ point a week |
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Tyrosinemia
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Deficiency of 4-Hydroxylphenyl Pyruvate Dioxygenase Causing TYr, Phe in blood
Mental retardation Hypopigmenation Growth retardation |
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Alcaptonuria
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Deficiency of Homogentisate oxidase
Darkens urine Arthritic joint pain |
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Albinism
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Tyrosine gene deficiency, Catecholamine unaffected
Absent of pigment in skin, hair, eyes, sensitive to UV |
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MSUD
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Defciency of Branched Chain α-ketoacid dehydrongenase
Elevated levels of BCAA Mental retardation Urine smells like maple |
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Homocystrinuria
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Deficinecy of Cystathionine Synthetase
Methionine in blood Mental Retardation Osteporosis Dislocation of lens heart disease |
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3 pathways that BH4 affects
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Tyrosine, catecholamine and serotonin synthesis
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Glutamate and Aspartate
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Excitatory NT of brain
Synthesized in CNS |
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Glutamate and GABA
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Inhibitory NT of brain
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Serotonin
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Derived from Tryptophan
Vasoconstrictor and NT for Smooth Muscle |
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Whats happens in excitatory neurons?
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Glutamine is turned into Glutamate and released
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What happens in inhibitory neurons?
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Glutamine is turned into Glutamate and then decarboxylated (w/PLP) into GABA and released
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