CASE STUDY #2A 2-year-old black girl is being seen by the hematologist after her pediatrician found her to beseverely anemic with splenomegaly and jaundice. Her mother gives a possible history of a “bloodproblem" in her family but doesn't know for sure. Her hemoglobin electrophoresis was normal, andthe complete blood count (CBC) revealed a normocytic anemia. The platelet and white blood cellOn the peripheral smear, there are many bizarre erythrocytes, includingcounts are normal.spiculated cells. A diagnosis of pyruvate kinase deficiency is made.5. Since in this patient pyruvate kinase is abnormal not only is less pyruvate made but intermediates abovepyruvate in the glycolytic pathway build up slowing the pathway. Which of the following products may not bemade in the appropriate amounts in the RBC because of the deficiency of pyruvateA. GlucoseB. OxaloacetateC. acetyl-CoAD. LactateExplanation for answer in no. 5:?

Pyruvate kinase is an glycolytic enzyme, which catalyzes the conversion of phosphoenolpyruvate (PEP)…

Answered: 5. Since in this patient pyruvate…

Biochemistry

9th Edition

ISBN:9781319114671

Author:Lubert Stryer, Jeremy M. Berg, John L. Tymoczko, Gregory J. Gatto Jr.

Publisher:Lubert Stryer, Jeremy M. Berg, John L. Tymoczko, Gregory J. Gatto Jr.

Chapter1: Biochemistry: An Evolving Science

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The various forms of vitamin K Function as cofactors for enzymes with a key role in blood coagulation O Function as cofactors in the blood coagulation pathway, and as chemical antioxidants Have different functions; one form is an enzyme cofactor, and another is a potent antioxidant O All serve as antioxidants
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8. In a patient with anemia, the presence of Heinz bodics in the RBCS was the result of the hemoglobin subunits aggregation due to the oxidation of -SH groups of Hb cysteine residues with active oxygen forms and the formation of disulfide bonds. What metabolic disorders in the RBC can be the cause of this clinical case? To solve the problem: a) indicate which reactions maintain cysteine residues in a reduced state; b) name the coenzyme involved in this process, write a diagram of the process in which the reduced form of this coenzyme is formed; c) indicate the enzyme which deficiency may lcad to a lack of the reduced cocnzyme and be the cause of the clinical case described above.
Check all of the following that applied for hemoglobin
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3. In your textbook the termi- nal enzyme catalyzing the ter- minal step of glycolysis is known as pyruvate kinase (PK). No indication is given that in mammalian tissues there are four different iso- forms of this enzyme. The four mammalian isoforms are known as PKL, PKR, PKM1 and PKM2. PKL is expressed in the liver, and PKR is ex- pressed in red blood cells. C PKM1 and PKM2 are derived from altemative splicing of the PKM gene. PKM1 is a constitu- tively active isoform expressed Active Site A PKM2 Monomer B Fructose-1,6-Bisphosphate N Phe, Ala, Ser Binding Site PKM2 Tetramer "Activator" Binding Site in differentiated cells from various tissues. In contrast, PKM2 has low basal activity and is activated by an effector molecule fructose 1,6-bisphosphate (FBP). PKM2 is expressed in most proliferating cells. In addition, the regulation of PKM2 pyruvate kinase activity plays an essential role in cancer metabolism and is crucial for the growth and survival of cancer cells. Recently, PKM2 has…
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5. Since in this patient pyruvate kinase is abnormal not only is less pyruvate made but intermediates above pyruvate in the glycolytic pathway build up slowing the pathway. Which of the following products may not be made in the appropriate amounts in the RBC because of the deficiency of pyruvate ?