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50 Cards in this Set

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