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Phenylketonuria (PKU) is an autosomal recessive genetic disorder characterized by a deficiency in the enzyme phenylalanine hydroxylase (PAH). This enzyme is necessary to metabolize the amino acid phenylalanine to the amino acid tyrosine. When PAH is deficient, phenylalanine accumulates and is converted into phenylpyruvate (also known as phenylketone), which is detected in the urine.
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What is the definition of Phenylketonuric?
The term "phenylketonuric" is an individual whose body cannot process certain substances (phenylalanine) into the amino acid tyrosine. This can cause a toxic buildup of phenylketone (aka phenylpyruvate) in the body tissues.The condition is a genetic defect that is ordinarily treated by dietary changes. It has gained prominence due to warnings for the artificial sweetener aspartame (Nutrasweet, Equal). Because the human body breaks down the sweetener into other substances, including phenylalanine, the consumption of the product could elevate the level of that chemical in phenylketonuric individuals.
What is the difference between essential and non essential acids?
Essential amino acids are those the body cannot produce. There are 20 basic amino acids. There are eight essential amino acids unless the individual is a phenylketonuric. There are nine essential amino acids for phenylketonurics as they cannot convert phenylalanine to tyrosine. Essential amino acids have to be taken into the body by eating. This answer is for informational purposes only. All individuals should ask advice of their physician.
How many drinks containing Aspartame can you safely consume each day?
If you have been tested and found to NOT be phenylketonuric, go for it. If you ARE phenylketonuric, any amount of aspartame can cause serious problems. Laws in most states require that infants be tested for phenylketonurea before they are allowed out of the hospital. If you were born in a hospital, you have been tested. If you have eaten a banana and not gone into siezures or suffered serious brain damage, you are NOT phenylketonuric and you could safely consue the amount of aspartame that is in 12 diet cokes every day. On the other hand, you SHOULD be concerned with the phosphoric acid and some of the other chemicals that are in soft drinks, and restrict your intake of them. === === Note: This information required a Freedom Of Information Act request to pry it from the reluctant hands of the FDA. Nutrasweet (brand name for Aspartame) was not approved until 1981, in dry foods. For over eight years the FDA refused to approve it because of the seizures and brain tumors this drug produced in lab animals. The FDA continued to refuse to approve it until President Reagan took office (a friend of Searle) and fired the FDA Commissioner who wouldn't approve it. Dr. Arthur Hull Hayes was appointed as commissioner. Even then there was so much opposition to approval that a Board of Inquiry was set up. The Board said: "Do not approve aspartame". Dr. Hayes OVERRULED his own Board of Inquiry. Shortly after Commissioner Arthur Hull Hayes, Jr., approved the use of aspartame in carbonated beverages, he left for a position with G.D. Searle's Public Relations firm. Long-Term Damage. It appears to cause slow, silent damage in those unfortunate enough to not have immediate reactions and a reason to avoid it. It may take one year, five years, 10 years, or 40 years, but it seems to cause some reversible and some irreversible changes in health over long-term use. METHANOL (AKA WOOD ALCOHOL/POISON) (10% OF ASPARTAME) Methanol/wood alcohol is a deadly poison. People may recall that methanol was the poison that has caused some "skid row" alcoholics to end up blind or dead. Methanol is gradually released in the small intestine when the methyl group of aspartame encounter the enzyme chymotrypsin. The absorption of methanol into the body is sped up considerably when free methanol is ingested. Free methanol is created from aspartame when it is heated to above 86 Fahrenheit (30 Centigrade). This would occur when aspartame-containing product is improperly stored or when it is heated (e.g., as part of a "food" product such as Jello). Methanol breaks down into formic acid and formaldehyde in the body. Formaldehyde is a deadly neurotoxin. An EPA assessment of methanol states that methanol "is considered a cumulative poison due to the low rate of excretion once it is absorbed. In the body, methanol is oxidized to formaldehyde and formic acid; both of these metabolites are toxic." The recommend a limit of consumption of 7.8 mg/day. A one-liter (approx. 1 quart) aspartame-sweetened beverage contains about 56 mg of methanol. Heavy users of aspartame-containing products consume as much as 250 mg of methanol daily or 32 times the EPA limit. See the nearby links for an official FDA view and an independent assessment.
What are some words that have k in them?
15-letter wordsacknowledgement, acknowledgments, alkalinizations, ankylostomiases, ankylostomiasis, backscatterings, childlikenesses, cholecystokinin, counterattacked, counterattacker, counterblockade, counterchecking, counterpicketed, disembarkations, electrokinetics, greensicknesses, heartbreakingly, heartsicknesses, kaffeeklatsches, keratinizations, kindergarteners, kindheartedness, kinesthetically, kittenishnesses, kremlinologists, lackadaisically, leukaemogeneses, leukaemogenesis, lickerishnesses, marketabilities, microearthquake, mountebankeries, musculoskeletal, outspokennesses, persnicketiness, pharmacokinetic, phenylketonuria, phenylketonuric, phytoplanktonic, plainspokenness, prekindergarten, stockbrokerages, straightjackets, straitjacketing, strikebreakings, talkativenesses, telekinetically, thanklessnesses, thinkablenesses, thunderstricken, thunderstriking, unknowabilities, unknowledgeable, unsportsmanlike, unworkabilities16-letter wordsacknowledgements, antiracketeering, cantankerousness, cholecystokinins, counterattackers, counterattacking, counterblockaded, counterblockades, counterclockwise, counterpicketing, heterokaryosises, knowledgeability, leukodystrophies, microearthquakes, pharmacokinetics, phenylketonurias, phenylketonurics, prekindergartens, remarkablenesses, straightjacketed, unskillfulnesses, unthinkabilities17-letter wordscounterblockading, gentlemanlikeness, kaleidoscopically, kindheartednesses, knowledgeableness, persnicketinesses, plainspokennesses, straightjacketing, triskaidekaphobia18-letter wordscantankerousnesses, gedankenexperiment, knowledgeabilities, triskaidekaphobias19-letter wordsgedankenexperiments, gentlemanlikenesses, knowledgeablenesses, phosphofructokinase20-letter wordsbuckminsterfullerene, keratoconjunctivites, keratoconjunctivitis, phosphofructokinases21-letter wordsbuckminsterfullerenes22-letter wordskeratoconjunctivitides, keratoconjunctivitises
What makes the essential amino acids different from the non-essential amino acids?
Essential amino acids are those the body cannot produce. There are 20 basic amino acids. There are eight essential amino acids unless the individual is a phenylketonuric. There are nine essential amino acids for phenylketonurics as they cannot convert phenylalanine to tyrosine. Essential amino acids have to be taken into the body by eating. This answer is for informational purposes only. All individuals should ask advice of their physician.
What are the disorders of Phenylalanine or Tyrosine Metabolism in Humans?
Phenylalanine Metabolism in HumansThe metabolism of the aromatic amino acids-phenylalanine and tyrosine in man provides a best example of a gene-controlled, enzyme catalyzed biochemical reaction. In man, phenylalanine is an essential amino acid which must be supplied in the dietary proteins. Once in the body, phenylalanine may follow any of three paths. It may be (1) incorporated into cellular proteins, (2) converted to phenylpyruvic acid, or (3) converted to tyrosine. The conversion of phenylalanine into tyrosine takes place in the presence of phenylalanine hydroxylase enzyme, in the liver cells. Tyrosine is converted in turn to 3-4-dihydroxy phenylalanine (nick-named DOPA) by another enzyme and DOPA serves as a precursor for the hormones adrenaline and noradrenaline and for the black pigment, melanin.Tyrosine itself serves as a precursor of the hormones thyroxine and triiodothyronine. Excess tyrosine is degraded to carbon dioxide and water by a series of steps which involves the formation of p-hydroxyphenyl pyruvate, 2-5 dihydroxy phenyl pyruvate, homogentisic acid, maleylacetoacetic acid, fumaryl acetoacetic acid and fumaric and acetoacetic acid. Excess phenylalanine is degraded by a series of steps to compounds which include phenylpyruvic acid and phenyl lactic acid.Genetic disorders of the phenylalanine metabolism and resulted diseases-Five rare diseases in man result from improper functioning of five enzyme systems (i.e., metabolic blocks) involved in the metabolism of phenylalanine, tyrosine and their derivatives. All of these diseases have been found to be due to mutant, autosomal recessive genes in homozygous conditions. These diseases are following:1.Phenylketonuria-Persons with genotype pp fail to produce enzyme phenylalanine hydroxylase (parahydroxylase) with the result that phenylalanine fails to convert into tyrosine and consequently, the concentration of phenylalanine rises in the blood plasma, cerebrospinal fluid and urine. The urine of phenylketonuric (PKUJ patient contains (in addition to phenylalanine) elevated amounts of phenylpyruvic acid, phenyl lactic acid and other derivatives of phenylalanine. PKU patients have light pigmentation and are physically and mentally retarded. The feeble mindedness in PKU patients is thought to -be due to an impairment of the brain tissues by the phenylpyruvic acid in the cerebro-spinal fluid.2. Alkaptonuria- The persons with genotype hh fail to produce the enzyme homogentisic acid oxidase which catalyzes the oxidation of homogentisic acid. Therefore, in them, normal oxidation of homogentisic acid into water and carbon dioxide does not occur and large amounts of homogentisic acid are excreted in the urine, which turn black upon exposure to the air. Moreover, the homogentisic acid accumulate in the body and become attached to the collagen of cartilage and other connective tissues, due to which, the ear and sclerae are stained black. Persons with such phenotypic abnormalities are said to have alkaptonuria disease.3. Tyrosinosis-The recessive gene, t in its homozygous condition, blocks the conversion of p-hydroxyphenylpyruvate into 2, S-dihydroxyphenyl pyruvate. This leads to the accumulation of tyrosine, excesses of which are excreted via the urine. This condition is called tyrosinosis. It is reported in only one human and cause no harmful effect.4. Goitrous cretinism-The persons with cc genotype fail to produce the enzyme which is required for the conversion of tyrosine into thyroxine and triiodothyronine hormones in their thyroid glands. This condition is called goitrous cretinism which is accompanied by a considerable degree of physical and mental retardation and hypertrophy of thyroid gland.5. Albinism- The persons with recessive AA genotype lack in the tyrosinase enzyme system which is required for the conversion of 3, 4-dihydroxyphenyl alanine (DOPA) into melanin pigment inside the melanocytes. In an albino patient melanocytes are present in normal numbers in their skin, hairs, iris, etc., but lack in melanin pigment.
What are ph words that sound like f in the initial?
Philippines phaeton phaetons phage phages phagocyte phagocytes phagocytic phagocytize phagocytized phagocytizes phagocytizing phagocytose phagocytosed phagocytoses phagocytosing phagocytosis phagocytotic phalange phalangeal phalanger phalangers phalanges phalansteries phalanstery phalanx phalanxes phalarope phalaropes phalli phallic phallically phallicism phallicisms phallism phallisms phallist phallists phallocentric phallus phalluses phanerogam phanerogams phanerophyte phanerophytes phantasied phantasies phantasm phantasma phantasmagoria phantasmagorias phantasmagoric phantasmagorical phantasmal phantasmata phantasmic phantasms phantast phantasts phantasy phantasying phantom phantomlike phantoms pharaoh pharaohs pharaonic pharisaic pharisaical pharisaically pharisaicalness pharisaicalnesses pharisaism pharisaisms pharisee pharisees pharmaceutical pharmaceutically pharmaceuticals pharmacies pharmacist pharmacists pharmacodynamic pharmacodynamically pharmacodynamics pharmacognosies pharmacognostic pharmacognostical pharmacognosy pharmacokinetic pharmacokinetics pharmacologic pharmacological pharmacologically pharmacologies pharmacologist pharmacologists pharmacology pharmacopeia pharmacopeial pharmacopeias pharmacopoeia pharmacopoeial pharmacopoeias pharmacotherapies pharmacotherapy pharmacy pharos pharoses pharyngeal pharynges pharyngitides pharyngitis pharynx pharynxes phase phaseal phased phasedown phasedowns phaseout phaseouts phases phasic phasing phasis phasmid phasmids phat phatic phatically pheasant pheasants phellem phellems phelloderm phelloderms phellogen phellogens phelonia phelonion phelonions phenacaine phenacaines phenacetin phenacetins phenacite phenacites phenakite phenakites phenanthrene phenanthrenes phenate phenates phenazin phenazine phenazines phenazins phencyclidine phencyclidines phenetic pheneticist pheneticists phenetics phenetol phenetols phenix phenixes phenmetrazine phenmetrazines phenobarbital phenobarbitals phenobarbitone phenobarbitones phenocopies phenocopy phenocryst phenocrystic phenocrysts phenol phenolate phenolated phenolates phenolic phenolics phenological phenologically phenologies phenology phenolphthalein phenolphthaleins phenols phenom phenomena phenomenal phenomenalism phenomenalisms phenomenalist phenomenalistic phenomenalistically phenomenalists phenomenally phenomenas phenomenological phenomenologically phenomenologies phenomenologist phenomenologists phenomenology phenomenon phenomenons phenoms phenothiazine phenothiazines phenotype phenotypes phenotypic phenotypical phenotypically phenoxide phenoxides phenoxy phentolamine phentolamines phenyl phenylalanine phenylalanines phenylbutazone phenylbutazones phenylephrine phenylephrines phenylethylamine phenylethylamines phenylic phenylketonuria phenylketonurias phenylketonuric phenylketonurics phenylpropanolamine phenylpropanolamines phenyls phenylthiocarbamide phenylthiocarbamides phenylthiourea phenylthioureas phenytoin phenytoins pheochromocytoma pheochromocytomas pheochromocytomata pheromonal pheromone pheromones phew phi phial phials philabeg philabegs philadelphus philadelphuses philander philandered philanderer philanderers philandering philanders philanthropic philanthropical philanthropically philanthropies philanthropist philanthropists philanthropoid philanthropoids philanthropy philatelic philatelically philatelies philatelist philatelists philately philharmonic philharmonics philhellene philhellenes philhellenic philhellenism philhellenisms philhellenist philhellenists philibeg philibegs philippic philippics philistia philistine philistines philistinism philistinisms phillumenist phillumenists philodendra philodendron philodendrons philological philologically philologies philologist philologists philology philomel philomels philoprogenitive philoprogenitiveness philoprogenitivenesses philosophe philosopher philosophers philosophes philosophic philosophical philosophically philosophies philosophise 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phonies phoniest phonily phoniness phoninesses phoning phono phonocardiogram phonocardiograms phonocardiograph phonocardiographic phonocardiographies phonocardiographs phonocardiography phonogram phonogramic phonogramically phonogrammic phonogrammically phonograms phonograph phonographer phonographers phonographic phonographically phonographies phonographs phonography phonolite phonolites phonologic phonological phonologically phonologies phonologist phonologists phonology phonon phonons phonos phonotactic phonotactics phons phony phonying phooey phorate phorates phoronid phoronids phosgene phosgenes phosphatase phosphatases phosphate phosphates phosphatic phosphatide phosphatides phosphatidic phosphatidyl phosphatidylcholine phosphatidylcholines phosphatidylethanolamine phosphatidylethanolamines phosphatidyls phosphatization phosphatizations phosphatize phosphatized phosphatizes phosphatizing phosphaturia phosphaturias phosphene phosphenes phosphid phosphide phosphides phosphids phosphin phosphine phosphines phosphins phosphite phosphites phosphocreatine phosphocreatines phosphodiesterase phosphodiesterases phosphoenolpyruvate phosphoenolpyruvates phosphofructokinase phosphofructokinases phosphoglucomutase phosphoglucomutases phosphoglyceraldehyde phosphoglyceraldehydes phosphoglycerate phosphoglycerates phosphokinase phosphokinases phospholipase phospholipases phospholipid phospholipids phosphomonoesterase phosphomonoesterases phosphonium phosphoniums phosphoprotein phosphoproteins phosphor phosphore phosphores phosphoresce phosphoresced phosphorescence phosphorescences phosphorescent phosphorescently phosphoresces phosphorescing phosphoric phosphorite phosphorites phosphoritic phosphorolyses phosphorolysis phosphorolytic phosphorous phosphors phosphorus phosphoruses phosphoryl phosphorylase phosphorylases phosphorylate phosphorylated phosphorylates phosphorylating phosphorylation phosphorylations phosphorylative phosphoryls phot photic photically photics photo photoautotroph photoautotrophic photoautotrophically photoautotrophs photobiologic photobiological photobiologies photobiologist photobiologists photobiology photocathode photocathodes photocell photocells photochemical photochemically photochemist photochemistries photochemistry photochemists photochromic photochromism photochromisms photocoagulation photocoagulations photocompose photocomposed photocomposer photocomposers photocomposes photocomposing photocomposition photocompositions photoconductive photoconductivities photoconductivity photocopied photocopier photocopiers photocopies photocopy photocopying photocurrent photocurrents photodecomposition photodecompositions photodegradable photodetector photodetectors photodiode photodiodes photodisintegrate photodisintegrated photodisintegrates photodisintegrating photodisintegration photodisintegrations photodissociate photodissociated photodissociates photodissociating photodissociation photodissociations photoduplicate photoduplicated photoduplicates photoduplicating photoduplication photoduplications photodynamic photodynamically photoed photoelectric photoelectrically photoelectron photoelectronic photoelectrons photoemission photoemissions photoemissive photoengrave photoengraved photoengraver photoengravers photoengraves photoengraving photoengravings photoexcitation photoexcitations photoexcited photofinisher photofinishers photofinishing photofinishings photoflash photoflashes photoflood photofloods photofluorographies photofluorography photog photogenic photogenically photogeologic photogeological photogeologies photogeologist photogeologists photogeology photogram photogrammetric photogrammetries photogrammetrist photogrammetrists photogrammetry photograms photograph photographed photographer photographers photographic photographically photographies photographing photographs photography photogravure photogravures photogs photoinduced photoinduction photoinductions photoinductive photoing photointerpretation photointerpretations photointerpreter photointerpreters photoionization photoionizations photoionize photoionized photoionizes photoionizing photojournalism photojournalisms photojournalist photojournalistic photojournalists photokineses photokinesis photokinetic photolithograph photolithographed photolithographic photolithographically photolithographies photolithographing photolithographs photolithography photolyses photolysis photolytic photolytically photolyzable photolyze photolyzed photolyzes photolyzing photomap photomapped photomapping photomaps photomask photomasks photomechanical photomechanically photometer photometers photometric photometrically photometries photometry photomicrograph photomicrographic photomicrographies photomicrographs photomicrography photomontage photomontages photomorphogeneses photomorphogenesis photomorphogenic photomosaic photomosaics photomultiplier photomultipliers photomural photomurals photon photonegative photonic photonics photons photonuclear photooxidation photooxidations photooxidative photooxidize photooxidized photooxidizes photooxidizing photoperiod photoperiodic photoperiodically photoperiodism photoperiodisms photoperiods photophase photophases photophobia photophobias photophobic photophore photophores photophosphorylation photophosphorylations photopia photopias photopic photoplay photoplays photopolarimeter photopolarimeters photopolymer photopolymers photopositive photoproduct photoproduction photoproductions photoproducts photoreaction photoreactions photoreactivating photoreactivation photoreactivations photoreception photoreceptions photoreceptive photoreceptor photoreceptors photoreconnaissance photoreconnaissances photoreduce photoreduced photoreduces photoreducing photoreduction photoreductions photoreproduction photoreproductions photoresist photoresists photorespiration photorespirations photos photosensitive photosensitivities photosensitivity photosensitization photosensitizations photosensitize photosensitized photosensitizer photosensitizers photosensitizes photosensitizing photoset photosets photosetter photosetters photosetting photosphere photospheres photospheric photostat photostated photostatic photostating photostats photostatted photostatting photosynthate photosynthates photosyntheses photosynthesis photosynthesize photosynthesized photosynthesizes photosynthesizing photosynthetic photosynthetically photosystem photosystems phototactic phototactically phototaxes phototaxis phototelegraphies phototelegraphy phototoxic phototoxicities phototoxicity phototropic phototropically phototropism phototropisms phototube phototubes phototypesetter phototypesetters phototypesetting phototypesettings photovoltaic photovoltaics phots phpht phragmoplast phragmoplasts phrasal phrasally phrase phrased phrasemaker phrasemakers phrasemaking phrasemakings phrasemonger phrasemongering phrasemongerings phrasemongers phraseological phraseologies phraseologist phraseologists phraseology phrases phrasing phrasings phratral phratric phratries phratry phreatic phreatophyte phreatophytes phreatophytic phrenetic phrenic phrenological phrenologies phrenologist phrenologists phrenology phrensied phrensies phrensy phrensying pht phthalic phthalin phthalins phthalocyanine phthalocyanines phthises phthisic phthisical phthisics phthisis phut phuts phycocyanin phycocyanins phycoerythrin phycoerythrins phycological phycologies phycologist phycologists phycology phycomycete phycomycetes phycomycetous phyla phylacteries phylactery phylae phylar phylaxis phylaxises phyle phyleses phylesis phylesises phyletic phyletically phylic phyllaries phyllary phyllite phyllites phyllo phylloclade phylloclades phyllode phyllodes phyllodia phyllodium phylloid phylloids phyllome phyllomes phyllos phyllotactic phyllotaxes phyllotaxies phyllotaxis phyllotaxy phylloxera phylloxerae phylloxeras phylogenetic phylogenetically phylogenies phylogeny phylon phylum physed physeds physes physiatrist physiatrists physic physical physicalism physicalisms physicalist physicalistic physicalists physicalities physicality physically physicalness physicalnesses physicals physician physicians physicist physicists physicked physicking physicochemical physicochemically physics physiocratic physiognomic physiognomical physiognomically physiognomies physiognomy physiographer physiographers physiographic physiographical physiographies physiography physiologic physiological physiologically physiologies physiologist physiologists physiology physiopathologic physiopathological physiopathologies physiopathology physiotherapies physiotherapist physiotherapists physiotherapy physique physiques physis physostigmine physostigmines phytane phytanes phytoalexin phytoalexins phytochemical phytochemically phytochemist phytochemistries phytochemistry phytochemists phytochrome phytochromes phytoflagellate phytoflagellates phytogeographer phytogeographers phytogeographic phytogeographical phytogeographically phytogeographies phytogeography phytohemagglutinin phytohemagglutinins phytohormone phytohormones phytoid phytol phytols phyton phytonic phytons phytopathogen phytopathogenic phytopathogens phytopathological phytopathologies phytopathology phytophagous phytoplankter phytoplankters phytoplankton phytoplanktonic phytoplanktons phytosociological phytosociologies phytosociology phytosterol phytosterols phytotoxic phytotoxicities phytotoxicity
