Term
| Name the metabolic didseases associated with the urea cycle that are autosomal recessive? |
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Definition
| Carbamoylphosphate Synthetaste 1 deficiency |
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Term
| Name the metabolic diseases associated with the urea cycle that are X-linked? |
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Definition
| Ornithine Transcarbamoylase deficiency |
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Term
| What metabolic diseases associated with the urea cycle have many abnormalities of the central nervous system? |
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Definition
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Term
| What is the metabolic disease associated with the reaction of phenylalanine to tyrosine? |
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Definition
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Term
| Name the metabolic disease associated with the reaction of tyrosine to homogentisate with tyrosine transaminase as the enzyme |
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Definition
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Term
| What is the metabolic disease assoc with the reaction of homogentisate to maleylacetoacetate? |
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Definition
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Term
| Name the metabolites of phenylalanine found in the urine that are diagnostic of PKU. ?, ?, ? melanin, tyrosinyl enzymes, and thyroid hormones. |
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Definition
| dopamine, norepinephrine, epinephrine |
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Term
| Name the enzyme associated with Parkinson's Disease and the metabolite used as treatment |
|
Definition
Enzyme: Tyrosine Hydroxylase
Metabolite Used: Tetrahydrobiopterin |
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Term
| Name the enzyme deficiency and the metal deficiency both of which can lead to albinism |
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Definition
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Term
| Name the end product of degradation of histidine and the cofactor needed for formation of this product. Name the urinary compound used as a diagnostic in this enzyme |
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Definition
End product is glutamate and N5-formimino H4folate
FIGLU is the urinary compound used as a diagnostic in this enzyme
Folate is the coenzyme |
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Term
| Name the end product of the degradation of lysine |
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Definition
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Term
| In regards to the pathway conversion of propionyl CoA into succinyl CoA what is the result of a biotin deficiency? |
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Definition
| Excretion of propionic Acid |
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Term
| In regards to the pathway conversion of propionyl CoA into succinyl CoA what is the result of a B12 deficiency? |
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Definition
| Excretion of methylmalonic Acid |
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Term
| Serine is the starting material for ? and ? |
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Definition
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Term
| H4folate or THF or tetrahydrofolate is the cofactor derived from ? |
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Definition
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Term
| The reaction of Folate to THF requires what as energy? |
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Definition
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Term
| THF consists of 3 groups: pteridine, amino benzoate, and ? ? |
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Definition
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Term
| THF carries carbons at ? position or ? position |
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Definition
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Term
| SAM also carries Carbon as a methyl group but it is not as versatile as ? |
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Definition
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Term
| What is the 21st amino acid? |
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Definition
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Term
| What is the precursor to selenocysteine? |
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Definition
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Term
| Selenocysteine formed from serine after formation of the ?-? |
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Definition
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Term
The two steps involved in creation of selenocysteine
1. Serine is phosphorylated by a ?
2. Converted to selenocysteine by a ? |
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Definition
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Term
| Selenocysteine can only be synthesized when serine is esterified to a tRNA that bears the anticodon ? |
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Definition
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Term
| Excessive production of oxalate forms the insoluble calcium oxalase salt which may lead to ? ? |
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Definition
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Term
| Glycine is the precursor of ? which can be transaminated back to glycine or oxidized to oxalate. |
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Definition
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Term
| What are the derivatives of Serine: ? , ? , choline, and glycine |
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Definition
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Term
| Essential Amino Acids ? ? ?, and , histidine, lysine, valine, leucine, isoleucine, phenylalanine, tyrosine, tryptophan |
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Definition
| Threonine, methionine and cysteine |
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Term
| What two amino acids are just gluconeogenic? |
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Definition
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Term
| What are the two ketogenic amino acids: ? and ? |
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Definition
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Term
| What five amino acids are both Ketogenic and gluconeogenic? |
|
Definition
: P.I.T.T.T.
Phenylalanine
Isoleucine
Threonine
Tryptophan
Tyrosine |
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Term
| Gluconeogenic intermediates that lead to the formation of ? |
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Definition
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Term
| Ketogenic intermediates that lead to the formation of |
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Definition
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Term
| The precursor for biopterin is ? |
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Definition
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|
Term
Biopterin Rxn is as follows
1. Tryptophan 5 –mono oxygenase (tryptophan) hydroxylase
2. Tyrosine hydroxyl;ase
3. ? ? |
|
Definition
| Phenylalanine hydroxylase |
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Term
| Carbons from MET are destined for intermediary metabolism but the sulfur is conserved through transfer to serine to form ? |
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Definition
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Term
| It is only the sulfur from ? that goes on to make cysteine. The rest of the carbons produce ? which can be further metabolized to propionyl CoA and finally ?. |
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Definition
homocysteine
alpha-ketobutyrate
Succinyl CoA |
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Term
| Branched chain amino acids are ? , ? , ? |
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Definition
| Valine, Isoleucine, and Leucine |
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Term
| What two types of energy are formed during the metabolism of Valine, Isoleucine, and Leucine? |
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Definition
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Term
| Valine, Isoleucine and Leucine (the branched amino acids) produce a lot of energy for? |
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Definition
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Term
| Valine, Isoleucine, and leucine are all oxidized by ? |
|
Definition
| Methylacyl CoA dehydrogenase (a.k.a. alpha keto acid dehydrogenase) |
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Term
| Branches chain amino acid aminotransferase products are: ?, ?, beta-hydroxy valeric acid |
|
Definition
| Propionyl CoA, Acetyl CoA |
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