1.04 UTF-8 0 0 0 the amino acids with the longest biosynthetic pathways are the ________ amino acids 2 essential 1 what two amino acids are considered to be semiessental 2 arginine and histidine bc they are produced in the body, but not in sufficient quantities 1 people that are decient in one or more amino acids go into what kind of a nitrogen balance 2 negative this means that they excrete more nitrogen from their bodies than they ingest 1 when more protein is being synthesized than is being degraded a person is said to have a ___________ _________ __________ 2 positive nitrogen balance this occurs in children or convalescing adults 1 what is kwashiorkor? 2 a disease that children in 3rd world countries when they are weened onto starchy protein-deficient diet 1 what is the benefit to measuring 3-methylhistidine in the urine 2 it is an indirect measurement of nitrogen balance. seen when muscle proteins are degraded at a high rate. 1 what is one treatment of leukemic cells that inhibits their growth 2 L-asparaginase 1 pathways that produce acetyl CoA or acetoacetyl CoA are considered to be ________ 2 ketogenic 1 alpha amino nitrogens can be removed by ________________ which is freely reversible. 2 transamination 1 what elevated serum level is a clinical indicator or marker of a myocardial infarction 2 AST and AST/ALT 1 what is a serum AST/ALT ratio that is low an indicator of 2 liver disease 1 how are ammonia levels kept low in the body 2 by the liver by the urea cycle 1 what are some of the symptoms of hyperammonemia 2 tremors slurred speech coma death 1 many reactions of amino acid metabolism involve what vitamin 2 pyridoxine (B6) 1 transfer of CO2 groups to organic compounds (carboxylation) often requires the action of _________ 2 the B vitamin biotin 1 one carbon groups are carried and transferred through the action of ____________ 2 tetrahydrofolate which is derived from the B vitamin folic acid (folate) 1 severe folate deficiency causes _____________in which immature red blood cells accumulate in the bone marrow due to a block in their DNA replication 2 megaloblastic anemia 1 what is the name of the cancer chemotherapeutic agent that inhibits the enzyme dihydrofolate reductase 2 methotrexate (an antifolate) 1 diydrofolate reductase is required to synthesize ______________ 2 tetrahydrofolate 1 a deficiency in tetrahydrofolate blocks the synthesis of ___________ bases and of _________nucleotides, thus ingibiting DNA repliaction which is why it is used as an anti-cancer drug 2 purine thymidine 1 cells that are resistant to cancer drugs such as methotrexate have undergone gene amplification for the ____________________ _____________ gene 2 dihydrofolate reductase 1 what are two types of antifolates that are used as antibacterial antibiotics 2 aminopterin sulfa drugs (sulfanilimide) 1 what is the function of sulfa drugs such as sulfnilimide? 2 similar in structure to para-amino benzoic acid (PABA) which inhibits the incoporation of PABA into folic acid, thus producting a folate deficiency in bacteria 1 what is the function of SAM 2 biological methylation reactions: creatine methylated RNA and DNA epinephrine carnitine 1 homocystinuria may play a role in what major diseases and symptoms? 2 osteoporosis atherosclerosis lens dislocation metal dysfunction mental retardation lethargy thrombosis 1 what can reduce the levels of homocysteine 2 supplements of vit B6, B12, and folic acid 1 what is the result of a vit B12 deficiency 2 pernicious anemia 1 how is B12 absorbed into the ileum 2 intrinsic factor 1 what is the danger of high folic acid supplimentation 2 it can mask anemia and thus result in mental symptoms from those that have a vit B12 deficiency that leads to pernicioius anemia (megaloblastic anemia with neurological deterioration) 1 what are the two main sources of Vit B12 2 stomach of animal foods baterial fermentation of plant products 1 PKU is commonly caused by a genetic defect in the ___________ ______ structural gene or by a defect in the synthesis or regeneration of the _______ cofactor 2 phenylalanine hydroxylase biopterin 1 why are the levels of phenylpyruvate/phenyllactate measured of all newborns? 2 check for diagnosis of PKU to avoid mental retardation 1 what is the second type of melanin fromed in red haired people called? 2 pheomelanin 1 albinism is most commonly caused by a genetic defect in the _________ structural gene 2 tyrosinase 1 what causes Parkinson's disease and how is it treated? 2 caused by a lowered ability by the substantia nigra to synthesize dopamine treated with DOPA 1 what drug is given to Parkinson's Disease patients to counter the effects of DOPA outside of the Central nervous system 2 carbidopa (bc it doesn't cross the blood brain barrier, but does inhibit DOPA decarboxylase) 1 transaminases are ________________ dependend enzymes 2 pryidoxal phosphate 1 i thas been demonstrated that ammoniium intoxication leads to a depletion of the ________ pool 2 ATP 1 pyridoxal phosphate which is involved in many reations of amino acid metabolism is derived from ____________ 2 pryidoxine (vit B6) 1 what is the MO of pyridoxal phosphate 2 forms a Shiff base intermediate which acts as an electron sink, destabilizing the groups around the alpha carbon 1 marginal folate deficiencies are responsible for the majority of ______________ cases which is why women take folate supplements before becoming pregnant 2 spina bifida 1 what do folates do with homocysteine and why is it important 2 regenerate methionine which then become SAM which does tons of stuff 1 marginal deficiencies of B6, B12, and folic acid are risk factors for both _________ and ___________ 2 atherosclerosis mental dysfunction 1 a vit B12 deficiency will lead to an accumulation of folates in the form of __________________ 2 N-methyl-THF 1 what are two symptoms of alkaptonuria 2 urin turns black on studing due to oxidation of the homogentisic acid that accumulates darkening of connective tissues and a form of arthritis 1 pheomelanin (the melanin in red heads) is formed from what? 2 cysteine resideus from glutathione tyrosine 1 what is the formation process of melanin 2 tyrosine--> DOPA--> Dopaquinone---> Melanin 1 what is used to treat thyperthyroidism? 2 thiourea dn related compouds (thyroid ablation or treatment with radioactive iodine, to partially destroy thyroid cells aae also used) 1