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Amino Acid Biosynthesis
UC MED 2015
27
Medical
Graduate
10/25/2011

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Term
What 4 amino acids are produced from glucose through components of the glycositic pathway?
Definition
serine(S), glycine(G), cysteine(C), and alanine(A)
Term
Alpha-ketoglutarate (from the TCA cycle) is a prescursor for what amino acids?
Definition
glutamate(E), glutamine(Q), proline(P), and arginine(R)
Term
Oxaloacetate (from the TCA cycle) is a precursor for what amino acids?
Definition
Aspartate(E) and Asparigine(N)
Term
What is the quintessential coenzyme of amino acid metabolism. In degradation, it is involved in the removal of amino groups, principally through transamination reactions and in donation of amino groups for various amino acid biosynthetic pathways? It is also required for certain reactions that involve the carbon skeleton of amino acids.
Definition
Pyridoxal phosphate (derived fromvB6)
Term
What is a coenzyme that is used to transfer one-carbon groups at various oxidation states? It is used in both amino acid degradation (e.g.,serine and histidine) and biosynthesis(e.g.,glycine).
Definition
FH4 (Tetrahydrofolate)
Term
What is a cofactor that is required for ring hydroxylation reactions (e.g.,phenylalanine to tyrosine)?
Definition
BH4 (Tetrahydrobiopterin)
Term
Tyrosine(W) is synthesized from which essential amino acid?
Definition
phenylalanine (F)
Term
Where does Cysteine(C) get its S(sulfur)?
Definition
from Methionine(M)
Term
If an amino acid's carbon skeleton can be converted to a precursor of glucose, it can be considered to be?
Definition
glucogenic
Term
If an amino acid's carbon skeleton can be converted directly to acetyl-CoA or acetoacetate, it can be considered to be?
Definition
ketogenic
Term
Which amino acids produce pyruvate when degraded?
Definition
serine(S), alanine(A), cysteine(C), threonine(T), glycine(G), and Tryptophan(W)
SACTGW
Term
What two essential amino acids are strictly ketogenic and only produce acetoacetate and acetyl-CoA?
Definition
lysine(K) and leucine(L)
Term
What two amino acids are derived from OAA and go back to that when degraded?
Definition
Aspartate and Aspargine
Term
What three amino acids are derived from Fumarate and go back to that when degraded?
Definition
Aspartate(D), Tyrosine(Y), and Phenylalanine(W)
Term
What four enzymes go through several conversions to eventually enter the TCA cycle as succinyl-CoA?
Definition
Valine(V), Threonine(T), Isoleucine(I), and Methionine(M)
Term
What amino acid enters the TCA cycle as a-ketoglutarate?
Definition
glutamate(E)
Term
What 4 amino acids convert to glutamate before entering the TCA cycle as a-ketglutarate?
Definition
Arginine(R), Glutamine(Q), Histidine(H), and Proline(P)
Term
What 4 amino acids are degraded into acetyl-CoA?
Definition
Threonine(T), Isoleucine(I), Lysine(K), and Tryptophan(W)
Term
What produced from Glycine can precipitate with calcium and from a kidney stone?
Definition
Oxalate
Term
A lack of the transaminase that can convert glyoxylate to glycine leads to the disease that has a consequence of renal failure attributable to excessive accumulation of oxalate in the kidney?
Definition
Primary Oxaluria I (PHI)
Term
Individuals with a genetically normal cystathionase can also develop cystathionuria from a dietary deficiency of...
Definition
B6
Term
What produces alanine from pyruvate?
Definition
alanine aminotransaminase (ALT)- if this is seen in the blood, probable liver leakage from damage to the liver
Term
Certain types of tumor cells, particularly leukemic cells, require _______ for their growth. Therefore this can be used as a treatment.
Definition
asparagine- treat with asparaginase- converts asparagine in the blood to aspartate reducing the amount of free asparagine
Term
What deficiency will lead to an accumulation of a-keto acids in the blood because of an inability of pyruvate dehydrogenase, a-ketoglutarate dehydrogenase, and branched-chain a-ketoacid dehydrogenase to catalyze their reactions?
Definition
thiamine deficiency
Term
What disease of the branched-chain a-keto acid dehydrogenase that oxidatively decarboxylates the branched-chain amino acids is defective? As a result, the branched-chain amino acids and their a-keto analogs (produced by transamination) accumulate. They appear in the urine, giving it the odor of maple syrup or burnt sugar. The accumulation of a-keto analogs leads to neurologic complications.This condition is difficult to treat by dietary restriction, because abnormalities in the metabolism of three essential amino acids contribute to the disease.
Definition
Maple syrup urine disease (degradation of valine, leucine, and isoleucine can't occur, ie they are the branched chain a.a.)
Term
What occurs when homogentisate, an intermediate in tyrosine metabolism, cannot be further oxidized because the next enzyme in the pathway, homogentisate oxidase, is defective? Homogentisate accumulates and auto-oxidizes, forming a dark pigment, which discolors the urine and stains the diapers of affected infants. Later in life, the chronic accumulation of this pigment in cartilage may cause arthritic joint pain.
Definition
Alcaptonuria
Term
The symptoms of pellagra are dermatitis, diarrhea, dementia, and, finally, death, this results from deficiencies in?
Definition
niacin and tryptophan
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