Match the each enzyme deficiency with their corresponding disease β-hexosaminidase A α -galactosidase Sphigomyelinase β-glucosidaseA.Fabry diseaseB.Tay-Sachs diseaseC.Niemann-Pick diseaseD.Gaucher disease

Different enzymes are required for synthesis of spingolipids. If these enzymes are not…

Answered: Match the each enzyme deficiency with…

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

Section: Chapter Questions

Problem 1P

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Which of the following is a purely glucogenic amino acid? A. Leucine B. Tyrosine C. Tryptophan D. Alanine
Answer the following questions about the inhibition of acetylcholine esterase by neostigmine. 5a. Does neostigmine have a structure that enables it to bind reversibly to the ACbE active site? 5b. Is neostigmine catalytically processed by ACHE? 5c. How does the stability of the carbamolyl-enzyme intermediate produced by treatment of ACbE with neostigmine compare to the stability of the acetyl- enzyme intermediate produce when ACb, is hydrolyzed?
Statin drugs are used to treat hypercholesterolemia because they a. prevent cholesterol from inserting in plasma membranes b. prevent cholesterol ester formation c. inhibit HMG-CoA reductase d. stimulate HMG-CoA synthase
Saponification is a reaction in which a. triacylglycerols are incorporated into lipoproteins b. triacylglycerols bind to the serum protein albumin c. fatty acid salts react to form triacylglycerols d. triacylglycerols undergo a base-catalyzed ester hydrolysis
The source of the nitrogen during serine synthesis is A. glutamate B. NH4+ C. NO2 D. α-ketoglutarate
A child with coarse facies, corneal clouding, joint stiffness and mental retardation is found to have a Damage to this enzyme directly affects which of partial defect in N-acetylglucosaminotransferase. the following biochemical functions? A. Formation of polysomes B. Microtubule attachment to centrioles C. Synthesis of ribosomal RNA D. Targeting of enzymes for lysosomes E. Transport of sugars into mitochondria
The immediate donors of the nitrogen atoms of urea are: a. Aspartate and glutamate b. Glutamate and carbamoyl phosphate c. Aspartate and carbamoyl phosphate d. Glutamine and aspartate
Choose which enzyme is likely deficient based on the symptoms described below: Elevated blood lactate, alanine and pyruvate a) lactate dehydrogenase d) citrate synthase c) pyruvate dehydrogenase d) pyruvate kinase
Von Gierke’s disease is a rare genetic disorder that results in a deficiency of glucose-6-phosphatase. Symptoms ketoacidosis, a buildup of glycogen in the liver, and lactate buildup. Why does this glycogen storage disease not affect the brain? a) The brain is able to still perform glycolysis effectively b) None of the above c) In the event of hypoglycemia, the brain is able to utilize other sources of energy to produce ATP d) The brain is able to metabolize lactate, which is present in high concetrations.
Which of the following is a degradation product of both cytidine and deoxycytidine? a. β-alanine b. β-aminoisobutyrate c. succinyl-CoA d. uric acid
Novolog differs from "normal" insulin in that: Select one: a. An aspartic acid has been substituted for proline at B28 b. The proline at B28 and the lysine at B29 have been reversed c. A glycine has been substituten in at A21, and two new arginines have been added as B31 and B32 d. A "C-peptide" chain has been added e. The usual insulin molecule has been combined with zinc isophane

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Match the each enzyme deficiency with their corresponding disease β-hexosaminidase A α -galactosidase Sphigomyelinase β-glucosidase A. Fabry disease B. Tay-Sachs disease C. Niemann-Pick disease D. Gaucher disease