BRS 5TH EDITION - BIOCHEM SHELF EXAM QUESTIONS AND
ANSWERS WITH COMPLETE SOLUTIONS VERIFIED
Deficiences of glycolytic enzyme have profound effect on ____ function.
RBC. RBCs lack mitochondria are completely reliant on glycolysis as a source of
energy.
Hereditary deficiency of GLUT-1, and insulin dependent transporter, results in
decreased glucose in the _____ _____. Patients manifest w/ intractable seizures
in infancy and developmental delay.
cerebrospinal fluid
Absence of A isoform of aldolase (found in RBCs and muscle)
The disorder presents w/ hemolytic anemia. Patients also have episodes of
rhabdomyolysis (destruction of muscle cells) following febrile illness.
Triose phosphate isomerase (TPI) deficiency
Neonatal-onset hemolytic anemia as well as progressive neurologic involvement.
Children have progressive hypotodiaphragm paralysis that requires ventilation, as well
as cardiomyopathy
What is enolase inhibited by?
Fluoride. Done to prevent ongoing glycolysis in a patient's blood samples collected
sensitive glucose tolerance tests, blood is collected in tubes containing fluoride.
MODY type 2.
,Maturity onset diabetes of the young type 2 is an autosomal dominant disorder involving
mutations in glucokinase gene (GCK). Patients have nonprogressive hyperglycemia that
is usually asymptotomatic at diagnosis and is usually managed with diet alone.
PFK deficiency
Glycogen storage disease (type VII) in which glycogen accumulates in muscle. Results
in inefficient use of glucose stores by RBCs and muscles. Patients experience hemolytic
anemia as well as muscle cramping.
Pyruvate kinase deficiency
causes decreased production of ATP from glycolysis. RBCS have insufficient ATP for
their membrane pumps, and a hemolytic anemia results.
Arsenic/rat poisoning
Inhibits enzymes of glycolysis, specifically pyruvate dehyrdrogenase. The lack of ATP
leads to dec in glutathione production and blocks gluconeogenesis.
GLUT-4
muscle and adipose glucose transporter.
GLUT-1
in cerebrospinal fluid. glucose transporter.
F-2,6-BP
Indicates glucose is abundant. Potent activator of PFK-1. It's levels increase in the liver
when insulin is released (after a meal).
What traps labeled glucose in the cell?
conversion of glucose to G6P by glucokinase and hexokinase.
Lactate formation occurs when the ____/____ ratio is high.
, NADH/NAD+
Glucose inhibits ____ formation and is also used in the treatment of ___-blocker
overdose.
pyruvate, beta
The basis of elevated lactate is an increase in ____ due to the lack of oxygen in
the muscle.
NADH
In triose phosphate isomerase deficiency, a component of the ____ ____ ____ is
in elevated amounts in the muscle.
glycerol phosphate shuttle (this disease would lead to an accumulation of DHAP that is
involved in this shuttle)
TCA cycle provides carbon skeletons for three processes. What are they?
1. gluconeogenesis
2. FA synthesis
3. Interconversion of amino acids
PDHC deficiency
Most common neurodegenerative disorders associated w/ abnormal mitochondrial
metabolism. Severe forms of disease are lethal. Mild forms exhibit ataxia and mild
psychomotor delay related to lactate buildup, especially during times of illness, stress,
or high carb intake.
Order of TCA enzymes
1. citrate synthase
2. aconitase
ANSWERS WITH COMPLETE SOLUTIONS VERIFIED
Deficiences of glycolytic enzyme have profound effect on ____ function.
RBC. RBCs lack mitochondria are completely reliant on glycolysis as a source of
energy.
Hereditary deficiency of GLUT-1, and insulin dependent transporter, results in
decreased glucose in the _____ _____. Patients manifest w/ intractable seizures
in infancy and developmental delay.
cerebrospinal fluid
Absence of A isoform of aldolase (found in RBCs and muscle)
The disorder presents w/ hemolytic anemia. Patients also have episodes of
rhabdomyolysis (destruction of muscle cells) following febrile illness.
Triose phosphate isomerase (TPI) deficiency
Neonatal-onset hemolytic anemia as well as progressive neurologic involvement.
Children have progressive hypotodiaphragm paralysis that requires ventilation, as well
as cardiomyopathy
What is enolase inhibited by?
Fluoride. Done to prevent ongoing glycolysis in a patient's blood samples collected
sensitive glucose tolerance tests, blood is collected in tubes containing fluoride.
MODY type 2.
,Maturity onset diabetes of the young type 2 is an autosomal dominant disorder involving
mutations in glucokinase gene (GCK). Patients have nonprogressive hyperglycemia that
is usually asymptotomatic at diagnosis and is usually managed with diet alone.
PFK deficiency
Glycogen storage disease (type VII) in which glycogen accumulates in muscle. Results
in inefficient use of glucose stores by RBCs and muscles. Patients experience hemolytic
anemia as well as muscle cramping.
Pyruvate kinase deficiency
causes decreased production of ATP from glycolysis. RBCS have insufficient ATP for
their membrane pumps, and a hemolytic anemia results.
Arsenic/rat poisoning
Inhibits enzymes of glycolysis, specifically pyruvate dehyrdrogenase. The lack of ATP
leads to dec in glutathione production and blocks gluconeogenesis.
GLUT-4
muscle and adipose glucose transporter.
GLUT-1
in cerebrospinal fluid. glucose transporter.
F-2,6-BP
Indicates glucose is abundant. Potent activator of PFK-1. It's levels increase in the liver
when insulin is released (after a meal).
What traps labeled glucose in the cell?
conversion of glucose to G6P by glucokinase and hexokinase.
Lactate formation occurs when the ____/____ ratio is high.
, NADH/NAD+
Glucose inhibits ____ formation and is also used in the treatment of ___-blocker
overdose.
pyruvate, beta
The basis of elevated lactate is an increase in ____ due to the lack of oxygen in
the muscle.
NADH
In triose phosphate isomerase deficiency, a component of the ____ ____ ____ is
in elevated amounts in the muscle.
glycerol phosphate shuttle (this disease would lead to an accumulation of DHAP that is
involved in this shuttle)
TCA cycle provides carbon skeletons for three processes. What are they?
1. gluconeogenesis
2. FA synthesis
3. Interconversion of amino acids
PDHC deficiency
Most common neurodegenerative disorders associated w/ abnormal mitochondrial
metabolism. Severe forms of disease are lethal. Mild forms exhibit ataxia and mild
psychomotor delay related to lactate buildup, especially during times of illness, stress,
or high carb intake.
Order of TCA enzymes
1. citrate synthase
2. aconitase
