Amino Acids Self test Flashcards
Non-Essential Amino acids:
1) Tyr 2) Gly 3)Ala 4)His 5)Ser 6)Asn
7) Gln 8)Cys 9)Glu 10)Asp
15 and 6 ?
15 - N-Acetylglutamate
6 - Argenine
Arginine will form at the end of Urea cycle, Allosterically activate N-Acetylglutamate synthase, which its product will activate CPS 1 the rate limiting step of Urea cycle.
Keeps it rolling…
2 - Where can it alternativelycome from ?
3 - Why is it important?
5 - Importance of this product?
2 - Citruline - Can come from the Gut as well
3 - Aspartate - it is an Ammonia carrier
5 - Fumarate - Link to TCA cycle, Energy supply.
Which enzymes are Intramitochondrial and which are extramitochondrial (Urea cycle)
Intra -CPS 1 + Ornithine Carbamoyltransferase
Extra - Argininosuccinate Lyase, Argininosuccinate Synthase, Arginase
9 and 10?
And the corresponding deficiencies (Red trinagles)
9 - CPS 1
10 - Ornithine Carbamoyltransferase
Deficiency (of both) will cause Hypperammonemia and Mental Retardation
Which are 1 and 3 ?
2 Enzyme - Importance?
1)Serine and 3)Glycine
2 - is Serine Hydroxymethyl transferase
Methylation of THF
will ultimately lead to formation of Methionine and SAM
4 - Enzyme , Cofactor , Deficiency
Homocysteine Methyl transferase
aka Methionine Synthase
B12 (Coblamin) wil carry the Methyl group
Deficiency - Folate/Methyl trap
Will result in - Cell Cycle Malfunction, Megaloblastic Anemia, Homocysteine elevated.
It also stopes Methionine from getting Methyl groups- AUG - Protein Synthesis Malfunction.
Common for 1 2 3
5 Deficiency
1 2 3 - Branched Amino
Leucine , Valine , Isolecine respectivaly
5 -BCAA Dehydrogenase Complex
Maple syrup Disease:
Ketoacidosis and Mental Retardation
Amino acids forming Acetyl-CoA
Leucine and Isoleucine - Through BCAA Complexes
Phe and Tyr - Through Acetoacetate
Lysine and Tryptophan - Through Acetoacetatyl-CoA
Amino acids forming Oxaloacetate
Aspartate and Aspargine - Through transdeamination
Amino acids forming Fumarate
Phe and Tyr - Same pathway also gives Acetyl-CoA
Aspartate and Aspargine - Going through Urea Cycle
Amino acids forming Alpha - Ketoglutarate
Glutamine and Glutamate - Transdeamination
Proline, Histidine and Arginine - Multiple steps to Glu
Amino acids forming Succinyl-CoA
Threonine and Methionine
(Dehydratase and SAM cycle)
both form an intermediate called Alpha-Ketobutyrate through there it is converted to Propionyl-CoA and then to Succinyl-CoA
Valine and Isoleucine
through BCCA degradation complexes to Propionyl-CoA
Essential Amino Acids - PVT TIM HALL
1)His 2)Val 3)Leu 4)Ile 5)Arg 6)Thr 7)Lys
8)Trp 9)Met 10)Phe
1 -Pepsin
3 - HCl
2 - Chymotrypsin and Elastase
5 - Trypsin
4 - Enteropeptidase
6 - Aminopeptidase, Carboxypeptidase and Dipeptidase