Squire

Question 1

Hematocrit

Answer 1

Portion of blood volume that is occupied by RBC

Question 2

Plasma concentration

Answer 2

Albumin: 55% for oncotic pressure, lipid/steroid/hormone/NO transport, antioxidant protein with electrophile cysteine

Globulins: 38% transport ions, hormones and immune functions (gamma globulin)

Fibrinogen for clotting
Regulator proteins

Question 3

a1-antitrypsin

Answer 3

Serpin= serine protease inhibitor protective during inflamation. Inactivated by cigarette smoke can result in elastase activity= emphysema and COPD

Question 4

a1 antichymotrypsin

Answer 4

(serpin): serine protease inhibitor with protective role during inflammation.

Question 5

serum amyloid A a1

Answer 5

Part of high-density lipoproteins that are secreted by liver in response to inflammatory cytokines; functions to recruit immune cells.

Question 6

lipoprotein a1

Answer 6

Part of low-density and high-density lipoproteins enabling fats to be transported to adipocytes and liver in blood stream.

Question 7

Haptoglobin a2

Answer 7

binds free hemoglobin for removal by spleen. Used to monitor for hemolytic anemia. Keeps hemoglobin

Question 8

α2-macroglobulin

Answer 8

inhibits ALL classes of proteases, including those involved in coagulation (e.g., thrombin) and fibrinolysis (e.g., plasmin, kallikren)

Question 9

ceruloplasmin

Answer 9

major copper carrying protein

Question 10

thyroxine-binding globulin

Answer 10

binds thyroid hormones T3 and T4 (along with transthyretin and albumin

Question 11

α2-antiplasmin

Answer 11

serpin responsible for inactivating plasmin (fibrinolys

Question 12

protein C

Answer 12

zymogen that upon activation (proteolysis) acts to inactivate blood clotting (FVa and FVIIIa) and plasmin (fibrinolysis

Question 13

angiotension:

Answer 13

upon proteolysis by ACE generates peptide hormone (vasoconstriction).

Question 14

β2-microglobulin

Answer 14

: regulates iron transport following binding to receptor (activation

Question 15

plasminogen

Answer 15

zymogen that upon activation (proteolysis) degrades fibrin (fibrinolysis).

Question 16

transferrin:

Answer 16

high-affinity iron binding protein (2 binding sites) that transports iron through the body

Question 17

C02 heme binding

Answer 17

Binds to amino terminus histidine and blocks O2 from binding via Bohr effect
Only 20% bound, the rest carried as carbonate

Question 18

O2 binding

Answer 18

02 binds to heme and makes it planar, this moves the FG loop to change the other SU.

Question 19

Bohr effect

Answer 19

(T) hb + 4O2 Hb(O2)4 + n H (R)
pka=7.7 pka=7.3
Salt bridge Asp-94 and His146 (stabilize deoxy form)
Deoxy has more salt bridges
More acidic the blood, the lower the saturation curve. So increasing CO2 increase H that decreases the saturation curve.
Protonation stableizes deoxy form

Question 20

BPG and heme

Answer 20

Increases in hypoxia to increase O2 delivery
Allosteric effector and stabilizes deoxy form (T)
Higher the altitude, more BPG, more right shift

Question 21

CO binding

Answer 21

Binds to the iron with higher affinity

Lose the sigmoidal curve with lowered vmax

Question 22

Fetal heme differences

Answer 22

BPG binding cleft (Ser to His) mutation

Question 23

EPO

Answer 23

Hypoxic stress enduces—more RBC
Synthesized in renal cortex
Hypoxia inducing factor always on but O2 degrades it.

Question 24

Heme synthesis

Answer 24

Begins and ends in Mitochondria

Heme blocks first step of rxn

Question 25

Hemoglobin pathologies

Answer 25

Thalassemias- Globin chain amount defect, common
Porphyrias- Porphorin ring problem, uncommon
Iron deficiency- most common

Question 26

Sickle cell

Answer 26

Normal RBC lifespan- 120 days
Sickle- 10 days
Dehydration can cause nucleation event
Glu-Val at position 6

Question 27

Heme catabolism

Answer 27

Processing and Excretion of Hydrophobic Products of Porphyrin Ring (Bilirubin)

Question 28

Iron metabolism

Answer 28

Transferrin is Critical for Iron Transport and Delivery, binds 2 irons (high affinity, releases in acidic environment, used for Fe Def. assay)

Ferritin is Major Protein for Iron Storage (Liver, Epithelial, Macrophages; NOT Significant in RBCs. Binds 4500 Fe and looks like a bag ofrust of Fe3+.

Question 29

Hepcidin

Answer 29

Peptide Hormone Made in Liver
Binds to Iron Export Channel Ferreportin to Block Uptake and Mobilization
Beta Thalassemia is Common Anemia Related to Iron Overload That May Result From Hepcidin Deficiencies