Term
|
Definition
| these 2 amino acids are exclusively ketogenic |
|
|
Term
| Isoleucine, Phenyalanine, Tyrptophan, Tyrosine, Threonine |
|
Definition
| Name the ketogenic and glucogenic AA's |
|
|
Term
|
Definition
| aspargine is deaminated by ____ yeilding asparate and ammonia and this can be used to treat Leukemia |
|
|
Term
|
Definition
| Glutamine is deaminated by this enzyme to form glutamate |
|
|
Term
|
Definition
| Glutamate-->alpha ketoglutarate is a transaminase reaction catalyzed by what enzyme? |
|
|
Term
|
Definition
| What does the histidine load test measure to estimate folate cells? |
|
|
Term
|
Definition
| If we have a block of histidase what product will not be produced? |
|
|
Term
|
Definition
| alanine, cystine, glycine, serine, threonine can all be converted to ? |
|
|
Term
| phenyalanine hydroxylase; THB |
|
Definition
| when phenyalanine is converted to tyrosine, what is the enzyme and its cofactor? |
|
|
Term
| fumarate and acetoacetate |
|
Definition
| what are the two products that tyrosine can form? |
|
|
Term
|
Definition
| this disease is caused by a deficiency in tyrosine metabolism and phenyalanine hydroxylase |
|
|
Term
|
Definition
| What does phenyalanine hydroxylase require as a coenzyme? |
|
|
Term
|
Definition
| The intermediate stage from Isoleucine and Valine--> succinyl CoA is? what vitamin does it involve? |
|
|
Term
| Propionyl CoA carboxylase |
|
Definition
| If you have a biotin deficiency and you see a build up of propionyl CoA, what enzyme is deficient? |
|
|
Term
|
Definition
| Mehtylmalonic Aciduria is unresponsive to B12 and it is associated with developmental retardation and chronic metabolic acidosis. What is the deficient enzyme? |
|
|
Term
| Isoleucine, Leucine, Valine, and Tryptophan |
|
Definition
| what are the four essential AA's |
|
|
Term
| Branched chain alpha keto acid dehydrogenase |
|
Definition
| MSUD is caused by what type of deficiency? |
|
|
Term
| Methalonyl CoA+ Succinyl CoA |
|
Definition
| In the biosynthesis of Nonessential Amino acids the two end products formed by Valine are? |
|
|
Term
| Acetyl CoA and Propionyl CoA |
|
Definition
| In the biosynthesis of Nonessential Amino acids the two end products formed by Isoleucine? |
|
|
Term
| acetyl CoA and acetoacetate |
|
Definition
| In the biosynthesis of Nonessential Amino acids the two end products formed by Leucine |
|
|
Term
|
Definition
| tyrosine hydroxylase and tryptophan hydroxylase requires this co-enzyme |
|
|
Term
| Guthrie Card Bacteria Assay |
|
Definition
| What is the test that is used to asses Maternal PKU? |
|
|
Term
|
Definition
| this is driven by hydrolysis of all 3 phosphate bonds in ATP |
|
|
Term
| Cystathinonine Beta Synthase |
|
Definition
| What is the enzyme that is deficient in Homosytinuria? |
|
|
Term
|
Definition
| Complete albinism results from a deficiency of _____ activity |
|
|
Term
| homogenetisic acid oxidasse |
|
Definition
| Alkaptonuria is caused by a deficiency in this enzyme resulting in accumulation of homogentisic acid |
|
|
