(P) Week 3: RBC function Part 1 Flashcards
Enumerate the general characteristics of normal RBC
- Bi concave disc
- with central pallor
- Average volume of 90 fL(fentoliter)
- 6-8 micrometer in diameter
- 1.5-2.5 micrometer in thickness
- No cytoplasmic inclusions
abnormal shaped RBC
Poikilocytosis
depression in the center of the RBC where the nucleus was located
central pallor
normal percentage of the central pallor size
30-45% of the total diameter of the rbc
other souces say not more than 3um or 1/3 of the total diameter
RBC where the central pallor is smaller or absent
found in megaloblastic anemia
hyperchromic red cells
RBC’s central pallor is larger than the reference value
found in iron-deficiency anemia
hypochromic red cells
what is the ave diameter of a RBC
7.2-7.5 micrometers (range is 6-8 micrometers)
RBC that has a diameter of below 6 micrometers
microcytic RBC
RBC that has a diameter of more than 8 micrometers
macrocytic rbcs
RBC have varying size in general
anisocytosis
what is the average surface area of a rbc
140 micrometers
the ability of a RBC to be flexible
deformability
components of a RBC
membrane structure
Hgb stability
energy production
hemolysis that occurs outside the blood vessels, usually within the reticuloendothelial system such as the spleen and liver
extravascular hemolysis
red cell destruction in the blood vessels
intravascular hemolysis
What are the components of a RBC membrane
proteins
carbohydrates
lipids
what are the two types of proteins that make up the protein part of the RBC membrane
transmembrane (integral proteins) and the peripheral proteins
this is the point of attachment, vital to the stability of the RBC membrane structure
peripheral proteins
proteins that passes through the bi-lipid layer
transmembrane
lipids that make up the rbc membrane includes
cholesterol and phospolipid
the lipid bi-layer cosists of ______ heads and a ______ tail.
polar head
non-polar tail
The polar heads of the
phospholipid bi-layer is______
non-polar tail is ______
hydrophobic and hydrophilic
provides additional cushion in the red cell membrane, is situated within the lipid layer
cholesterol
disorder where cholesterol membrane is affected due to lack of beta-lipoprotein
abetalipoproteinemia
vital for the continuous metabolism of cholesterol
beta-lipoprotein
a kind of poikilocytosis associated with abetalipoproteinemia as there is a deviation in the shape of the RBC
acanthocytosis
Transmembrane proteins carry the blood group antigens, they are the one that dictates our blood types
Blood group Ag
what do you lack if you do not have a GPI anchor
GPI-linked protein
defective __________ is associated with an oblong / oval ellipsoidal RBC
Spectrin-Ankyrin-Protein-Band 4.1
Issues with Spectrin-Ankyrin-Protein-Band 4.1 leads to this disease characterized with oval shaped RBCs
elliptocytosis
3 diseases associated with defective Spectrin-Ankyrin-Protein-Band 4.1
Hereditary elliptocytosis, pyropoikilocytosis, and leach phenotype
defective ____________ causes the RBC to become loose and form abnormal blebs or mud-like structures.
Spectrin-Ankyrin-Protein-Band 3-4.2
what kind of abnormal shape is observed if the problem is Spectrin Ankyrin-PB3-PB4.2.
spherocytes or hereditary spherocytosis
Defective _________ may be associated with hereditary ovalocytosis / southeast asia ovalocytosis
Spectrin Ankyrin-PB3
Spectrin protein is made up of which two chains?
alpha and beta chain
responsible for the formation of Glycosylphosphatidylinositol linked proteins (GPI-linked proteins) in our RBC
PIGA gene
Paroxysmal nocturnal hemoglubinuria (PNH) is due to the deficiency of the of the following:
PIGA gene
CD 55
CD 59
also known as the Decaying Accelerating Factor
CD 55
also known as the Membrane Inhibitor Reactive Lysis Protein
CD 59
What is that condition that is associated with
problems with your GPI protein?
Paroxysmal nocturnal hemoglobinuria (PNH)
means that sometimes there are visible symptoms in the patient or sometimes there are none
paroxysmal
a condition where the red cells are destroyed and hemoglobin are liberated in the blood and is reflected on the urine
hemoglobinuria
other name for protectin
MAC inhibitor protein
prevents the complete formation of the
“membrane attack complex”, (known also as
C5b6789)
MAC inhibitor protein or protectin
how does the MAC inhibitor protein or protectin prevent the formation of the membrane attack complex
preventing the attachment of c9 protein
where are complement proteins normally found?
plasma or blood
_________ fights bacteria alongside WBCs by coating the it and punching a hole on the bacterial membrane
complement proteins
How does the RBCs protect themselves from being collateral damage from complementary proteins attacking bacteria?
CD 55 and 59 protects the RBC
What state/ form does our hemoglobin take form in to prevent being precipitated?
fluid / reduced state
how many globin chains is in a single hemoglobin
2 pairs
how many heme is found in a single hemoglobin
four
a vital part of the hemoglobin molecule that helps in the normal and efficient release of oxygen from the hemoglobin of the tissue
2,3 DPG
where is the 2,3 DPG synthesized
embden meyrehof metabolic pathway (Rapaport-Leubering pathway to be more specific)
An inclusion formed in the hemoglobin due to oxidization
Heinz bodies
In what state does heme have to be so that our body may absorb its iron content?
Ferrous state (Fe^2+)
What are the two components of heme?
protoporphyrin IX
Fe^2+
what enzyme aids the insertion of Fe^2+ into the protoporphyrin IX
ferrochelatase
T or F
Iron is absorbed in the ferric state (Fe^3+) from our diet
F (ingested only in the ferric state but it is then converted to the ferrous state to be absorbed)
State in which iron is stored
ferritin
ferritin is composed of this protein along with a ferric iron
apoferritin
apoferritin is a special kind of protein present on some human cells that received iron from the blood-ferritin transport protein known as __________
Transferrin
alpha and zeta genes are found in
chromosome 16
Beta, delta, gamma, and epsilon genes are carried by
chromosome 11
We can measure how much is our A1, A2, and F through
electrophoresis
the molecular structure of the globin chains forms a _________ appearance which forms loops and folds within it called helices
quarternary appearance
When you see this card please study heme formation
familiarize the stps:
- glycine + succinyl CoA
- Goes outside the cytoplasm, and is changed into porphobilinogen by d-Amino-levulinic acid
- transformed to hydroxymethyl bilane by PB deaminase
- changed into Uroporphyrinogen III by Uro II synthase
- changed to coproporphyrinogen III by Uro III decarboxylase (enters the mitochondria again)
- Transformed into Protoporphyrinoggen III by CP III oxidase
- end point is heme ring / Protoporphyrin IX by protoporpyrin III oxidase enzyme
T or F
Heme ring is not considered as a heme because it contains iron
T
Heme ring = no iron
heme = iron baby
The transport protein for iron
Transferrin
What is the form of iron in the blood?
ferric
the binding of the transferrin protein
transferrin ferric complex
term used when transferrin passed the iron to the apoferritin
apoferritin ferric complex
maturing red cells esp rubriblast will adhere to the _________ to ask for iron
macrophages
which phase does the formation of hemoglobin (binding of the heme and globin) in the cytoplasm occur ?
rubricyte stage
what is the last stage wherein hemoglobin is formed
reticulocyte
type of porphyria where ferrochelatase is affected, it is dominant, photosensitive, an increase in protoporphyrin is observed in the blood and feces
erythropoetic porphyria
type of porphyria that lacks uro III synthase, and is recessive
congenital erythropoietic porphyria
type of porphyria that lacks ALA-synthase, passed down as an X-linked trait
X-linked protoporphyria
T or F
porphyrins will be excreted by the body as the formation of heme is interrupted by the affected enzymes
F (it will accumulate)
a heme kinetic problem that will lead to severe anemia and photophobia, aka Vampire disorder
porphyria
porphyrins are seen and deposited to _____ and _____ in porphyria
teeth and urine
2 metabolic pathways that the red cells utilize
Embden Meyerhof Pathway (EMP)
Hexose monophosphate pathway (HMP)
Glycolitic pathway whose main responsibility is to produce energy anaerobically from antioxidants derived from drugs and toxic substances
EMP
how many ATPs are used by the EMP
2
How many ATPs are produced by the EMP?
4
What is the net ATP of EMP?
2 (4 are produced BUTTT 2 are used, therefore making the net ATP 2 lang :>)
enzymes used in the first phase of EMP glucose metabolism
hexokinase
gluconate phosphate isomerase
Phosphofructokinase
(Hey gurl posey)
enzymes used in the second phase of EMP glucose metabolism
Aldolase
Triose Phosphate isomerase
Phosphoglycerate
(ATP)
enzymes used in the third phase of EMP glucose metabolism
3-phosphoglycerate mutase
enolase
pyruvate kinase
lactate dehydrogenase
three enormous pussy lactate ehe
what is the end point of glucose metabolism
lactate
T or F
the embden myerhof pathway does not use any oxygen
T
ATP will not be produced if you have what type of deficiency, therefore stopping the rbc metabolism
Pyruvate kinase deficiency
around how many percent of the glucose enter the embden-meyerhof pathway for energy production
90-95%
pyruvate kinase deficiency changes the rbc into what abnormal shapes?
burr cells / acanthocytes
What is the main objective of the Embden-Meyerhof pathway?
produce energy
The site of synthesis for 2,3-Diphosphoglycerate (DPG)
Rapport-leubering pathway
This is a very important hemoglobin component, it pushed out the oxygen from our hemoglobin to the tissues
2,3-diphosphoglycerate (DPG)
what is the condition secondary to 2,3-diphosphoglycerate deficiency
hypoxia
A hemoglobin that carries a ferric (Fe3+) iron
methemoglobin
Iron becomes ferric due to exposure to toxic substances such as ______ and _______
nitrates and nitrites
What will you feel if your methemoglobin is at 1%
nothing, you’re still fine as methemoglobin should be around <1
what will you feel if methemoglobin levels reaches 3%
cyanosis and hypoxia
This enzyme allows the iron in its ferric state return to its ferrous state, as well as provides protection against oxidant present in the food we eat
methemoglobin reductase enzyme
this pathway is responsible for reducing the ferric iron into ferrous iron within the heme
methemoglobin reductase pathway
other name for methemoglobin reductase
cytochrome b5 reductase
you’ve reached the 105th card if you’re a concrete sequential learner !!
pray for nath’s sanity as she tries to keep the cards up to date !!!
