Biochemistry Flashcards
What is the type of cell that makes all types of blood cells?
Pluripotent stem cells
What are the 3 dereivatives from the pluripotent stem cells?
CFU-GEMM (mixed myeloid progenitor cell)
Lymphoid stem cell
NK-precursor
What type of cell does the NK-precursor make?
Nk-cells lol
What are the 2 types of cells that the lymphoid stem cells make?
B- and T-lymphocytes
What are the 2 eventual cells make from the BFU-E cells fom the CFU-GEMM?
RBC’s (using erythrocyte pathway)
Platelets (using megakaryocyte pathway)
What are the 3 eventual cells make from the CFU-GMEo pathway?
Neutrophils (using CFU-GM)
Monocytes (–> macrophages, using CFU-GM)
Eosinophils (using CFU-Eo)
Since RBCs dont have nuclei or mitrochondria, what energy pathway does it use exclusively?
Glycolysis
Since RBC’s cant make proteins, is depends on what enzyme that determines its lifespan?
G6PD
What is the intermediate of the glycolysis pathway that is used in the HMP shunt (PPP)?
G6P
What is the intermediate of the glycolysis pathway that is used in the Rapoport Luberin shunt?
1,3-BPG
What is the structure of heme?
Porphyrin ring with a Fe at its center
What are the 2 components in teh first step of heme synthesis?
Succinyl CoA + Glycine
What is the first enzyme in the heme synthesis pathway to make δ-ALA?
δ-ALA synthase
What is the last enzyme in the heme synthesis pathway to put a Fe++ molecule in Protoporphyrin IX?
Ferrocheletase
What is the condition where there is a defect in any enzyme in the heme synthesis?
Porphyria
Why do vampire and werewolf stories come from people with porphyrias?
Cuz the heme intermediates are toxic to the nervous system –> photosensitivity, hallucinations, and seizures
laso, pt have scarring and increased facial hair
When hemoglobin is first degredated, what is the product?
Heme
What is teh like span of a RBC?
120 days
What is the produce of degredation of heme, which gibes off Fe++ and CO as biproducts?
Bilirubin
What does bilirubin bind to in the blood once formed?
Albumin
What molecule binds to bilirubin on the albumin, thus kicking off the albumin and forming bilirubin diglucononide?
UDP-glucuronate
Where does Bilirubin diglucuronide then put?
Bile
What is the term for Hb with Fe in the ferric (3+) state?
MetHb
For congenital MetHbemia, what enzyme is missing, which normally reduces ferric Fe to the ferrous state?
Cytochrome B5 reductase
What for of Hb can pts also inherit for congential metHbemia?
Hb M
Ingestion of what things can cause acquired metHbemia?
Certian oxidants like nitrite, quinones, aniline,a nd sulfonamides
What is the treatment for acquried metHbemia?
Administering reducing agents like ascorbic acid or methylene blue
What is oxidices by ROS in the RBC so they dont dmg the cell? What pathway keeps this molecule reduced?
Glutathione
PPP/HMPshunt
What are the components of the RBC cytoskeleton?
Specrin, actin, bands 4.1 and 4.2
Which races are at risk for G6PD deficiencies?
Africans and Mediterraneans
What is the inheritance for G6PD deficiencies?
X-linked
What medications can cause problems in G6PD deficiencies?
Anti-malarials
What 2 cells might u see on blewd smares for G6PD deficiencies?
Bite cells
Heinz bodies
This is a set of disorders common from defect in cytoskeletal proteins, notably ankyrin, spectrin, and Band 3.
Hereditary spherocytosis
What happens when RBC’s pass through capallaries and the spleen in hereditary sphreocytosis?
they’re inflexible (like my time for studying public health) –> change shape to spheres –> hemolysis
In blood typing, what Ab’s will be present in my blood since I’m type O-?
Ab’s to A antigen, B antigen, and Rh antigen
What is the universal donor and acceptor?
Donor: O- (like me cuz I’m so giving)
Acceptor: AB+ (like brent cuz hes greedy)
What is the suffix to describe the size of RBC’s?
-cytic
micro and macro- are the prefixes
What is the suffix to describe the [Hb] in RBC’s?
-chromic
Iron deficiency, thalassemia mutation, and lead poisoning may lead to impaired synthesis of what molecule?
Hb
What is the RBC morphology with impaired hemoglobin synthesis?
Microcytic, hypochromic
What 3 things can cause impaired DNA synthesis?
B12 or folic acid deficiency
Erythroleukemia
Impaired DNA synthesis may lead to what RBC morphology?
Macrocytic, normochromic
Red cell loss (like from bleeding or hemolysis) may lead to what morphology of RBC’s?
NO CHANGE
Normocytic, normochromic
Why does B6 deficiency cause the synthesis of heme production to slow?
Hint: molecule
Cuz it requires puridoxal phosphate
What is the RBC morphology in B6 deficiency?
Microcytic, hypochromic
pale and small, like a little old lady that isnt getting her B6 vitamins because she was once a student of a geriatrics class and is now showing students the repercussions of elder neglect as she has lost interest in other daily activities over the years and finds that experimental testing keeps her young and happy despite the fact that this experimental testing is deleterious to her health overall.
What is the manifestation of pyruvate kinase deficiency?
hemolytic anemia
What is the problem if there is inadquate iron intake or excessive loss of iron?
Iron deficiency.
What is the problem if there is a squence of codon 6 of the B chain changed from GAG to GTG, which substitutes Val for Glu?
Sicle cell
What is the problem if there are mutations in the alpha gene?
alpha thallasemia
What is the disorder if there is decreased absorption of B12 or lack of intrinsic factor?
Megalblastic anemia
What is the problem if there are deficiencies in the amt or in the structure of alpha or beta spectrin, ankyrin, band 3 or band 4.1?
Hereditary spherocytosis
What is the disorder when there are mitations in the PIG-A gene, affecting synthesis of GPI-anchored proteins?
Paroxysmal nocturnal hemoglobinuria
How many alpha globin genes are there?
4
How many deletions of alpha globin genes do u need to when u start to see microcytic hypochrmic RBC’s?
2
In addition to severe microcytic hypochromic anemia, what is the other manifestation of 3 alpha globin genes missing?
Splenomegaly
Do u die as a hydrops fetalis with 4 missing alpha globin genes?
Um, yes.
What is the homotetramer called of excess b chains?
HbH
like HGH. WHERES THE PROTEIN, MA? I TOLD YOU TO GET ME SOME PROTEIN.