Term
| Porphyrins are named with a word and number or roman numeral. The word denotes ______________. The number denotes __________. |
|
Definition
Word: kind of substituents (A, P, V, M)
Number: arrangement of side chains (1,2,3,4) |
|
|
Term
| There are 4 major series of porphyrins: I, II, III and IV. Which of these 2 do not occur in natural systems? |
|
Definition
|
|
Term
| If the prefix uro means urine and copro means filth/feces, what does proto mean? |
|
Definition
| first in the series, highest in rank |
|
|
Term
| Porphyria cutanea tarda is due to a deficiency of ______. |
|
Definition
| Uroporphyrinogen Decarboxylase |
|
|
Term
| The average lifespan of RBCs in an adult is ______. The average lifespan of RBCs in a newborn is ______. |
|
Definition
Adult: 120 days
Neonate: 60-70 days |
|
|
Term
| Heme contains 4 five-membered nitrogen containing rings called ______. |
|
Definition
|
|
Term
| The name of the German chemist who worked and elucidated the structures of porphyrins – not to be confused with Emil. |
|
Definition
|
|
Term
| A mineral cofactor for the enzyme porphobilinogen synthase |
|
Definition
|
|
Term
| The product of the first reaction in the biosynthesis of heme is ______. |
|
Definition
| 5-amino (delta-amino) levulinic acid |
|
|
Term
| This cobalt containing molecule has some chemical resemblance to heme. |
|
Definition
|
|
Term
| The correct terminology for the ring system found in vitamin 12 or cyanocobalamin is ______. |
|
Definition
|
|
Term
| This magnesium containing molecule has some chemical resemblance to heme. (The tetrapyrrole nucleus is actually referred to as chlorin.) |
|
Definition
|
|
Term
| This heme is bound covalently to the protein of cytochrome c through tioester bonds to 2 cysteine residues. |
|
Definition
|
|
Term
| In uroporphyrin each 5-membered ring contains an ______ and a ______ side chain. |
|
Definition
| A + P (acetate and propionate) |
|
|
Term
| In coproporphyrin each 5-membered ring contains a ______ and a ______ side chain. |
|
Definition
| M + P (methyl and propionate) |
|
|
Term
| The first reaction of the biosynthesis of heme involves the condensation of ______ and ______. |
|
Definition
|
|
Term
| The protein found in the sclera (conjunctiva) which is first to react with bilirubin in jaundice. |
|
Definition
elastin
(can also be seen under the tongue) |
|
|
Term
| The word jaundice is derived from the French word “jaune” meaning ______. |
|
Definition
|
|
Term
| Some patients with chronic jaundice actually look greenish in appearance. This is due to ______. |
|
Definition
| reoxidation of bilirubin --> biliverdin |
|
|
Term
| The first annular (ring) compound formed in the biosynthesis of heme is ______. |
|
Definition
| porphobilinogen (PBG) (first amino group with A + P) |
|
|
Term
| The enzyme which finishes the biosynthesis of heme by adding iron in the form of ferrous ion is known as ______. |
|
Definition
|
|
Term
| The most common form of genetic porphyria is ______. |
|
Definition
| acute intermittent porphyria |
|
|
Term
| A drug that is classically associated with precipitating symptoms of acute intermittent porphyia is ______. |
|
Definition
| phenobarbital (barbiturates, alcohol, chloramphenicol, steroid hormones) (all due bc of delta 5-db) |
|
|
Term
| The degradation of heme gives a linear tetrapyrrole ______ which is ______ in color. |
|
Definition
|
|
Term
| Which methylene bridge carbon is affected in the breakdown of heme to biliverdin? |
|
Definition
| alpha (between ring and ring 2) |
|
|
Term
| The main sites of heme catabolism are the ______ and the ______. |
|
Definition
|
|
Term
| The indirect van den Bergh test measures ______ bilirubin. |
|
Definition
|
|
Term
| In the van den Bergh assay ______ is coupled to bilirubin. |
|
Definition
| diazotized sulfaniliac acid |
|
|
Term
| Bilirubin encephalopathy is normally referred to as ______. |
|
Definition
|
|
Term
| A drug used in photodynamic therapy for the treatment of age-related macular degeneration is ______. |
|
Definition
|
|
Term
| A drug used in photodynamic therapy for the treatment of esophageal cancer is ______. |
|
Definition
|
|
Term
| Tryptophan pyrrolase is a heme-containing enzyme which oxidizes tryptophan to ______. |
|
Definition
|
|
Term
| The heme structure normally contains ______ side chains. |
|
Definition
|
|
Term
| An inherited disorder of bilirubin metabolism resulting from predominantly unconjugated hyperbilirubinemia. |
|
Definition
|
|
Term
| This vitamin as the phosphate cofactor is essential for the activity of ALA synthase. |
|
Definition
|
|
Term
| A procedure where some blood is removed from circulation to reduce hepatic iron overload as in porphyria cutanea tarda (PCT). |
|
Definition
|
|
Term
| An herbicide responsible for cases of porphyria cutanea tara in Vietnam veterans. |
|
Definition
|
|
Term
| A drug used in photodynamic therapy for the treatment of non-hyperkeratotic keratoses. |
|
Definition
Levulin Kerastik
(aminolevulinic acid) |
|
|
Term
| How does Levulan Kerastick (aminolevulinic acid) work? |
|
Definition
topically
- converted to porphyrin and if you shine a light on it it converts free oxygen radicals (causes aptosis of the cell) |
|
|
Term
|
Definition
|
|
Term
| When using PDT, what is the substance which ultimately leads to cytotoxic effects? |
|
Definition
|
|
Term
| Porphobilinogen synthase is also known as ALA ______ and is sensitive to inhibition by ______. |
|
Definition
|
|
Term
| Head to tail condensation of 4 molecules of porphobilinogen produces a linear tetrapyrrole intermediate known as ______. |
|
Definition
|
|
Term
| 85% of heme is biosynthesized in the ______. |
|
Definition
|
|
Term
| A deficiency of uroporphorynogen III cosynthase produces congenital ______. |
|
Definition
|
|
Term
| A deficiency of uroporphorynogen I synthase is the major cause of ______. |
|
Definition
| acute intermittent porphyria |
|
|
Term
| King George III suffered from ______. |
|
Definition
| acute intermittent porphyria |
|
|
Term
| A wheat fungicide widely used in Turkey in the 1950s which caused thousands of children to develop acquired porphyria. |
|
Definition
|
|
Term
| A solvent required to solubilize unconjugated bilirubin in the indirect van den Bergh assay or test. |
|
Definition
|
|
Term
| When heme is degraded, a small amount of a gas is formed. This is ______. |
|
Definition
|
|
Term
| Variegate porphyria is another type of porphyria. It is relatively common in the white African population of South Africa. The enzyme defect is ______. |
|
Definition
| Protoporphyrinogen Oxidase |
|
|
Term
| Biliverdin is excreted in the feces of ______ and ______. |
|
Definition
|
|
Term
| A yellow bile pigment which colors the urine is ______. |
|
Definition
|
|
Term
| Neonatal “physiologic jaundice” is due to a deficiency of ______ and a reduced activity of ______. |
|
Definition
UDP-Glucuronic Acid
UDP-Glucronyl Transferase |
|
|
Term
| Neonatal physiologic jaundice is treated with the drug ______ or by exposing babies to ______. |
|
Definition
phenobarbital
visible light |
|
|
Term
| The two main sites of heme biosynthesis are the ______ and the ______. |
|
Definition
|
|
Term
| A bile pigment formed by colonic bacteria and which gives feces a brown color. |
|
Definition
|
|
Term
| A popular OTC iron product (as caplets) containing carbonyl iron which is prepared by the chemical decomposition of iron pentacarbonyl. NOTE: the tablets marketed under the name contain ferrous sulfate. |
|
Definition
|
|
Term
| A congenital absence of bilirubin UDP-glucuronyl transferase activity leads to the ______. |
|
Definition
|
|
Term
| In type I Crigler-Najjar syndrom serum bilirubin usually exceeds ______ and is usually fatal within the first ______ months of life. |
|
Definition
20mg/dL
15 months of life |
|
|
Term
| An iron porphyrin enzyme that degrades hydrogen peroxide is known as ______. |
|
Definition
|
|
Term
| Cytochromes ______ are key enzymes involved in drug and xenobiotic detoxification. |
|
Definition
|
|
Term
| The P in P450 means ______. The 450 in P450 means ______. |
|
Definition
P = pigment
P450: wavelenght that it will show absorption in UV spectrum |
|
|
Term
| In the protoporphyrin series there are ______ possible combinations of the side chains. |
|
Definition
|
|
Term
| In the conversion of uroporphyrinogen III to protoporphyrinogen IX a total of ______ carbon dioxide molecules are lost. |
|
Definition
|
|
Term
| The oxidation of protoporphyrinogen IX to protoporphyrin IX by protoporphyrinogen oxidase results in the loss of ______ protons and ______ electrons. |
|
Definition
|
|
Term
| Protoporphyrins are heme-compounds which contain the side chains methyl, propionate and ______. |
|
Definition
|
|
Term
| Of the two ionic forms of iron, ______ is the form which is best absorbed. |
|
Definition
|
|
Term
| David Shemin and David Rittenberg were the first scientists which demonstrated that all of heme’s carbon and nitrogen atoms are derived from ______ and ______. |
|
Definition
|
|
Term
| An acid which when diazotized will react with bilirubin and produce a reddish-purple compound which can be measured spectrophotometrically. |
|
Definition
| (diazotized) sulfanilac acid |
|
|
Term
| The two most common forms of iron storage are known as ______ and ______. |
|
Definition
|
|
Term
| What is the Rotor syndrome? |
|
Definition
Inherited disorders of bilirubin metabolism that results from conjugated hyperbilirubinemia
It is related to another disorder known as Dubin-Johnson syndrome. |
|
|
Term
| What is the drug hemin (Panhematin) used for? |
|
Definition
| treating the symptoms of acute intermittent porphyria |
|
|
Term
| Porphobilinogen syntase, also known as aminolevulinic acid dehydratase can be inhibited by |
|
Definition
|
|
Term
Which of the following is NOT an intermediate in the synthesis of heme?
A coproporphyrinogen III
B porphobilinogen
C aminolevulanate
D Uroporphyrin IX
E protoporphyrin IX |
|
Definition
|
|
