Question

Need help with questions 1,3 and 5 please

C I NE CLINICALLY: Be S.M.A.R.T. About Cases w throughout the book edd levels Current SD car d comotion for Gu y and cardiovascular probleThe main of the pisto blood to the b lood that the patient will suffer from ang l ic h by the to w y , chcel and triglycerides Cornstarch is a dietary supplement for regulating Wood cose levels in OSD patients in a polysaccharide that like yco- gen, is a large polymer of sucos e se constarch is slowly broken down it help blood glucose levels. A patient with GSD Inspector 301045 percent of his or her daily calories from wwwcostarch. The cornstarch is mored with a ter; it should not be estado mid with highly acide juices, as the chemical composition of the match can be alred making it al leive treatment Henry's Blood Wors 0-105 mg 13 L Frost Troyorides 200 mg 150 mg tiedot Below 2.3 20-150 mg 50-120 mod three-month-old Henry washed to the next M en's hospital by ab ce Upon arriving at the hospital was unconscious exhibited behind had low body temperature. Once Henry was the phys en spoke with the parents. They said it was the first sight of rying to limit nighttime dinge for Henry since birth hat been eating on a two- h r ound the clock schedule that was hausting for his mother. Since the baby oder e hour troches between ding by the same is felt it was time to try anf ing schedule The mother reported that Hy wee t , but at she did not feed him immediately high he would back . The mother checked on my during his crying spell to make sure the war . A bout 5 minutes of nonstop crying the mother decided to feed him walking toward Henry's room she heard his cries suddenly stop. and upon entering the room found Henry cousing in his crib The physician examined the baby and reported that Henry had an enlarged liver (hepatomegaly. His blood work was also concerning (select data table). The parents shared that when Henry was born, his blood sugar was extremely low hypogly- cemia), but he was readily stabilired with glucose. It was deter- mined that Henry's low blood glucose level likely triggered that night's seizure. Henry was eventually released and his parents were told to follow up on his condition with a specialist. After pemingly endless tests, Henry was finally diagnosed with von Gierke's disease, or type I glycogen storage disease (GSD). The parents learned that GSDs are rare genetic disorders with an overall incidence of about 1 in every 43.000 live births There are over 12 classes of GSDs, but about so percent of all cases are characterized as type L In GSD I, the patient can't break down glycogen due to a deficiency of the enzyme glucose 6-phosphatase. This enzyme catalyzes the last chemical reaction before the release of glucose into the bloodstream the hydrolysis of glucose-6 phosphate (C,H,O,P) into glucose and inorganic phosphate (PJ. This enzyme is made of 357 amino acids, and is a transmembrane protein (spans a lipid bilayer). GSD I patients have trouble stabilizing their blood glucose levels when fasting because they cannot make use of their glycogen energy stores. CASE-BASED QUESTIONS 1. Is gycogen an o o ring compound? Explain your 2. Write the chilotion for the action catalyzed by gucone ghorphase be sure to unlocular formas, but don't worry about wing a cader Afring the reaction ou na Thements that make the reactants and specify how many tons of each comentare present in the react . Explan what the ons mg/dL and momen in the blood work Tests 4. How would you describe Henry's blood prin comparison with normal anges in relation to his observed blood ph, what would he be described as man testing? 5. How would you describe the polarity of the enzyme glucose- phosphatase, knowing is a transmembrane protein? Think Clinically: Be SMART About Cases 63

Answer 1

Ans1 glycogen is a polysaccharide of glucose so it is an organic compound.

Ans 3 mg/dl or milligram per litre is the amount of a perticular substance ( such as glucose) in specific amount of blood.

mmol/L or millimol per litre. A mole is amount of a substance that contains molecule equal to the Avogadro number. A millimol is one thousands part of a mole.

Ans 5 The enzyme is madeup of three subunits

G6PT1, G6PT2, G6PT3 . glucose 6 phosphatase is madeup of 357 amino acids and is anchored in endoplasmic reticulum.

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