globin synthesis Flashcards
ref range of hgb for women (conventional)
12-15 g/dL
give the symptoms present in congenital erythropoietic porphyria
deformity of fingers/nails red discoloration of teeth associted with severe photosensitivity scarring chronic hemolysis excessive hair growth teeth and urine are fluorescent under uv
chromosome which dictates other proteins(beta, gamma, delta, epsilon)
chromosome 11
3 embryonic hemoglobin
gower I
gower II
portland
gower II contains
alpha
epsilon
production of globin chains takes place in
pronormoblast-reticulocyte
globin synthesis is controlled by
genetic control
hbA is composed of?
lifespan?
2 alpha
2 beta
6 mos-adulthood
other name for methemoglobin reductase
cytochrome b5 reductase
hbF is not found in adults but in?
F cells
formed by non enzymatic rxnof sugrs to amino grps of globin chain
hbA1
portland contains
zeta
gamma
hb ref range for men (SI)
2.17-2.79 mmol/L
what are the hgb present at 1st 3 mos of embryonic development
gower I
gower II
portland
hgb which is predominant in normal adult
hbA
ref range of hgb for newborns (conventional)
16.5-21.5 g/dL
hgb which is predominant at birth
hbF
inherited/acquired disorders of heme synthesis
porphyrias
chromosome which dictates alpha and zeta
chromosome 16
ref range of hgb for newborns (SI)
2.17-4.03 mmol/L
oxyhgb is seen in?
what color of blood?
arterial blood
bright red
methemoglobin is increased in (3)
presence of oxidants
decreased methemoglobin reductase
hbM disease
ref range of hgb for women (SI)
1.86-2.48 mmol/L
deoxyhgb is seen in?
what color?
venous blood
dark red
color of blood with carboxyhgb
cherry red
rbcs w/ Shb have (2)
normal survival
osmotic fragility
6 oxidizing agents
aniline derivatives acetanilide acetaphenetidin chlorates k ferricyanide k permanganate
mRNA which is translated more efficiently
beta-globin mRNA
this results when carboxyhgb is not capable of transporting o2
hypoxia
most common neurologic porphyria
acute intermittent porphyria
methemoglobin contains what form of iron
ferric
hb + ? = methemoglobin
oxidizing agents
deficiency caused by the defect in variegate porphyrias
ferrochelatese deficiency
methemoglobin is reversible, T/F
T
porphyria which appears in adults due to excessive alcohol intake
porphyria cutanea tards
these 2 can reduce the iron (methemoglobin) in its reduced form
ascorbic acid
methylene blue
treatment for methemoglobin
removal of offending substrate
administration of ascorbic acid and methylene blue
formed by irreversible oxidation of hgb by certain drugs and chemicals
sulfhemoglobin
hgb which contains 2 alpha and 2 gamma
hbF
hba2 is composed of
2 alpha
2 beta
4 drugs/chemicals which results to sulfhemoglobin
sulfinamides
acetanilide
phenazopyridine
phenacetin
primary cause of porphyrias
specific enzyme deficiency
gower I contains
zeta
epsilon
porphyrias can be extreted through (3)
urine
stool
deposited in tissue
3 symptoms of neurologic porphyrias
abdominal pain
psychotic behavior
neuromuscular difficulties
sulfhemoglobin cannot be convrted to modified hgb; it persists for the life of the cell, T/F
F, unmodified/ normal hgb
reaction with sulfhgb is reversible, T/F
F, irreversible
enzyme defect in variegate porphyria
heme synthetase
substances increased in variegate porphyrias
iron
protorporphyrin IX
commonly acquired porphyria that results from block in several heme biosynthetic enzyme
lead intoxiation
enzymes absent in lead intoxication
ferrochelatase
coproporphyrinogen oxidase
other name of d-ALA dehydrogenase
PBG synthetase
term used if the teeth is fluorescent
erythrodontia
most common cutaneous porphyria
porphyria cutanea tard
most severely affected enzyme in lead intoxication
d-ALA dehydrogenase
blue discoloartion if skin
cyanosis
hb ref range for men (conventional)
14-18 g/dL
methemoglobin gives what color of blood
chocolate brown color
methemoglobin is also known as
ferrihgb
what are the 3 abnormal hgbs
carboxyhgb
methgb
sulfhgb
carboxyhgb is reversible, T/F
F, irreversible
