Shared Flashcard Set

Details

Heme Metabolism
Heme Metabolism
49
Pharmacology
Professional
12/16/2009

Additional Pharmacology Flashcards

 


 

Cards

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
pyrroles
Term
a mineral cofactor for the enzyme porphobilinogen synthase
Definition
zinc
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
Vitamin b-12
Term
The correct terminology for the ring system
found in vitamin B12 or cyanocobalamin
is____________________
Definition
corrin
Term
This magnesium containing molecule has some
chemical resemblance to heme _________.
Definition
chlorphyll
Term
This heme is bound covalently to the
protein of cytochrome c through thioester
bonds to 2 cysteine residues:
Definition
Heme C
Term
The protein found in the sclera (conjunctiva)
which is first to react with bilirubin in jaundice
Definition
Elastin
Term
The first annular (ring) compound formed in the
biosynthesis of heme is _______.
Definition
porphobilinogen
Term
The enzyme which finishes the biosynthesis
of heme by adding iron in the form of ferrous ion
is known as _________.
Definition
ferrochelatase
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
phenobarbitol
Term
Which methylene bridge carbon is affected in the breakdown of heme to biliverdin
Definition
alpha
Term
The main sites of heme catabolism are the
____________ and the __________.
Definition
liver and spleen
Term
The indirect van den Bergh test measures ______________ bilirubin.
Definition
free or unconjugated
Term
In the van den Bergh assay ___________________
is coupled to bilirubin
Definition
diazotized sulfanilic acid
Term
Bilirubin encephalopathy is normally referred
to as ______________
Definition
kernicterus
Term
A drug used in photodynamic therapy for
the treatment of age-related macular degeneration
is __________.
Definition
Verteporfin or Visudyne
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
formyl kynurenine
Term
The heme structure normally contains ____________
side chains
Definition
8
Term
An inherited disorder of bilirubin metabolism
resulting from predominantly unconjugated
hyperbilirubinemia _________
Definition
Gilbert syndrome
Term
this vitamin as the phosphate cofactor is essential for the activity of ALA synthase
Definition
B-6 or pyrixoxine
Term
an orally effective iron chelator used to reduce iron overload in patients receiving long-term blood transfusions
Definition
deferasirox
or exjade
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
dehydratase, Pb
Term
Head to tail condensation of 4 molecules of porphobilinogen produces a linear tetrapyrrole intermediate known as __________________.
Definition
hydroxymethylbilane
Term
85% of heme is biosynthesized in
the ________________.
Definition
reticulocytes
Term
A deficiency of uroporphorynogen III cosynthase
produces congenital __________________.
Definition
erythropoietic porphyria
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
methanol
Term
When heme is degraded a small amount of a gas
is formed. This is _____________.
Definition
CO - carbon monoxide
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
urobilin
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
liver, reticulocytes
Term
A congenital absence of bilirubin
UDP-glucuronyl transferase activity leads
to the ____________
Definition
Criggler-Najjar syndrom
Term
In type I Crigler-Najjar syndrome serum bilirubin usually exceeds ___________ and is usually fatal within the first ___________ months of life.
Definition
20 mg/dl, 15
Term
An iron porphyrin enzyme that degrades
hydrogen peroxide is known as
Definition
catalase
Term
In the conversion of uroporphyrinogen III to protoporphyrinogen IX at total of _____ carbon dioxide molecules are lost.
Definition
6
Term
The oxidation of protoporphyrinogen IX to protoporphyrin IX by protoporphyrinogen oxidase results in the loss of _________ protons and _______ electrons.
Definition
6,6
Term
Of the two ionic forms of iron _________
is the form which is best absorb
Definition
ferrous
Term
an acid which when diazotized will react with bilirubin and produce a reddish-purple compound which can be measured spectrophotometrically
Definition
Sulfanilic acid
Term
Which of the following is NOT an intermediate in the synthesis of heme?
Definition
Uroporphyrin III
Term
The two most common forms of iron storage
are known as __________ and ________.
Definition
ferritin,hemosiderin
Supporting users have an ad free experience!