Term
Porphyrins are named with a word and a
number or roman numeral.
The word denotes_______________ |
|
Definition
| the kinds of substituents |
|
|
Term
| The number denotes ______________ |
|
Definition
| how the substituents are arranged |
|
|
Term
| Porphyria cutanea tarda is due to a deficiency of __________________. |
|
Definition
| uroporphyrinogen decarboxylase |
|
|
Term
Heme contains 4-five-membered nitrogen containing rings
called _____________. |
|
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 or 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 B12 or cyanocobalamin
is____________________ |
|
Definition
|
|
Term
This magnesium containing molecule has some
chemical resemblance to heme _________. |
|
Definition
|
|
Term
This heme is bound covalently to the
protein of cytochrome c through thioester
bonds to 2 cysteine residues: |
|
Definition
|
|
Term
The protein found in the sclera (conjunctiva)
which is first to react with bilirubin in jaundice |
|
Definition
|
|
Term
The first annular (ring) compound formed in the
biosynthesis of heme is _______. |
|
Definition
|
|
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
porphyria |
|
Definition
|
|
Term
| Which methylene bridge carbon is affected in the breakdown of heme to biliverdin |
|
Definition
|
|
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 sulfanilic 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 _________. |
|
Definition
| Porfimer sodium or Photofrin |
|
|
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
| an orally effective iron chelator used to reduce iron overload in patients receiving long-term blood transfusions |
|
Definition
|
|
Term
A drug used in photodynamic therapy for the
treatment of non-hyperkeratotic keratoses
____________. |
|
Definition
| aminolevulinic acid (Levulan Kerastick |
|
|
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 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
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-glucuronyl transferase |
|
|
Term
The two main sites of heme biosynthesis are
the __________ and the _________. |
|
Definition
|
|
Term
A congenital absence of bilirubin
UDP-glucuronyl transferase activity leads
to the ____________ |
|
Definition
|
|
Term
| In type I Crigler-Najjar syndrome serum bilirubin usually exceeds ___________ and is usually fatal within the first ___________ months of life. |
|
Definition
|
|
Term
An iron porphyrin enzyme that degrades
hydrogen peroxide is known as |
|
Definition
|
|
Term
| In the conversion of uroporphyrinogen III to protoporphyrinogen IX at 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
Of the two ionic forms of iron _________
is the form which is best absorb |
|
Definition
|
|
Term
| an acid which when diazotized will react with bilirubin and produce a reddish-purple compound which can be measured spectrophotometrically |
|
Definition
|
|
Term
| Which of the following is NOT an intermediate in the synthesis of heme? |
|
Definition
|
|
Term
The two most common forms of iron storage
are known as __________ and ________. |
|
Definition
|
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