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22 Cards in this Set
- Front
- Back
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What are the products of phenylalanine metabolism?
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Tyrosine which can produce DOPA - Dopamine - NE - Epi and also melatonin
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Which step of the metabolism of phenylalanine is Vit C dependent?
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Conversion of Dopamine to NE
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What are the primary products of tryptophan metabolism?
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Serotonin and melatonin
Nicotinamide and Nicotinate (2 forms of niacin - vit B3) |
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What other vitamin plays a role in conversion of tryptophan into the 2 forms of niacin?
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Vit B6 (PLP - pyridoxal phosphate)
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What amino acids are released during metabolism of glutamate and what enzymes cause the release?
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Aspartic acid - AST (aspartate aminotransferase)
Alanine - ALT (alanine aminotransferase) |
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What catalyzes the conversion of glutamate to GABA?
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PLP aka vit B6
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What are the primary products of metabolism of glycine in the LIVER?
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delta-aminolevulinic acid (ALA)
Porphobilinogen Porphyrinogen Protoporphyrin IX Heme Hemoglobin |
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What are the primary products of metabolism of glycine in the mm/brain?
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Creatine
Creatine phosphate Creatinine |
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Why is creatinine an excellent measure of kidney function?
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Creatinine can ONLY be excreted via the kidney
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At what two points does lead inhibit the metabolism of glycine and what is the end result?
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delta-aminolevulinic acid dehydrase (prevents production of porphobilinogen)
ferrochelatase (prevents protoporphyrin IX from becoming heme) Results in microcytic anemia |
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Metabolism of what protein can benefit Alzheimers pts and why?
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Arginine
Metab of arginine releases NO which dilates blood vessels in the brain that have amyloid plaque deposits |
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What is the primary methylator in the body?
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SAM (S-adenosyl methionine)
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Why does buildup of homocysteine causes issues in the body?
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Excess Homocysteine stops conversion of SAH to homocysteine thus tying up all the adenosine in SAH preventing production of ATP d/t decreased amounts of adenosine
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What is the deficiency in albinism? What is its inheritance?
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Tyrosinase deficiency
X-linked: Ocular albinism Auto recess: oculocutaneous albinism |
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What is the deficiency in PKU?
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Phenylalanine hydroxylase (PAH)
OR Tetrahydrobiopterin (THP) |
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What is the deficiency in alkaptonuria?
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Homogentisate oxidase deficiency
Causes ochronosis (black connective tissue - ears, nose), dark pigmented urine when exposed to air (black diapers), and degenerative arthritis - all from deposition of oxidized homogentisate |
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What is the deficiency in Maple Syrup Urine dz?
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alpha-ketoacid dehydrogenase
I Love Vermont Maple Syrup Leads to a build up of branched chain amino acids (Isoleucine, Leucine, Valine) |
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Where is the highest incidence of Maple Syrup dz found in the world?
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Old Order Mennonites of Lancaster and Lebanon counties in Pennsylvania
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What is the deficiency in homocystinuria? What is the result?
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Cystathionine synthase deficiency
Homocysteine oxidizes to homocystine Lens dislocation, MR/ID, traps adenosine in SAH limiting amount of ATP that can be made |
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How do you treat homocystinuria?
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decrease methionine in diet, HIGH B6 supplementation, B12 and folate to help shunt homocysteine to methionine
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What is the deficiency in cystinuria?
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Defect in membrane transport of dibasic amino acids
COAL: cysteine, ornithine, arginine, lysine |
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What is the deficiency in Hartnup disease?
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Deficiency in membrane transport of tryptophan impairing renal reabsorption and initial reabsorption from the intestine
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