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13 Dec 2019

The question belong to Metabolic pathway in Biochemistry. Consider the following scenarios. Provide a description of the metabolic adaptations that would accompany each of the situations. Your answer should contain a well thought of analysis of the metabolic pathways that would be active, or inactive, during the given scenario.

a. when an individual is on a high carb diet (assume low fat, low protein)

b. when an individual is on a low carb, low fat diet (assume high protein)

c.when an individual is on a low carb, high fat diet (assume high protein)

d. after an individual has had a well-balanced meal.

e. after an individual has been fasting for 2-3 hours.

f. after an individual has been fasting for more than 24 hours (or more).

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17 Dec 2019
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Related questions

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2A). Why are people on high protein diets (such as the Adkins diet) instructed to drink lots of water?

2B). Explain why the symptoms resulting from a partial deficiency of an enzyme in the urea cycle can be attenuated by a low-protein diet?

3) In a famous UC Davis study, cats were fasted overnight, and then fed a synthetic diet containing all amino acids except Arg. The cats’ blood ammonia levels rose rapidly, with clinical symptoms of ammonia toxicity. A control group, whose meal was supplemented with ornithine showed no clinical symptoms.

If the cats fasted, catabolic metabolism of what would be enhanced? Comparing the experiment and control, what are cats deficient in synthesizing?

4) Several mutant E. coli strains have been identified that, after mutation, required require Ile for growth. Further characterization shows that these strains also require Val for growth, and some require Leu as well. Explain this observation. ​

Based on “Morgan: A case of Diabetes” by Lisa Rubin and Clyde Freeman Herreid (National Center for Case Study Teaching in Science)

The Patient:

Morgan Water is a 27-year old Native American that lives on a reservation in Oklahoma. Nearly ¼ of Morgan’s tribe has diabetes but she has no direct family history of diabetes, heart disease, or other serious conditions. She is however obese (BMI = 32) and leads a sedentary lifestyle. Morgan has been suffering from an increasing persistence of symptoms including unusual thirst, dizziness, blurred vision, and numbness in her right foot. The symptoms have been developing over several months but as she is planning her wedding she attributed them to nerves. She is finally persuaded to see her family doctor. Her non-fasting blood glucose level was elevated so the doctor has her return the next morning for a fasting blood sample so they could more accurately measure her blood glucose and insulin. Her blood results are below:

Test

Morgan’s values

Normal range

Pre-Diabetic range

Diabetic range

Plasma glucose, mg/dl

260

70 -139

140 - 199

≥ 200

Fasting glucose, mg/dl

140

≤ 99

100 - 125

≥ 126

A1C, %

7.6

~ 5

5.7 – 6.4

≥ 6.5

Fasting Insulin, mIU/ml

34

2 - 10

10 - 20

≥ 20

The recommendation of Morgan’s doctor was to improve her exercise, good nutrition, and weight loss. He recommends meeting with a nutritionist to assist Morgan in her weight loss goals. When Morgan got home and told her family the diagnosis they however had LOTS of suggestions on how she can lose weight. Below is a description of the advice given to her by three family members.

Best friend Savannah:

“You know how I’ve been losing weight? Well, I’ve been on the Atkins’ Diet. I heard about it on TV. It’s great! You get to eat steak, chicken wings, and all the good stuff. All you have to do is limit carbohydrate intake. You should definitely give it a try. This diet has been around for years and it has worked for lots of people, including me.”

*Note: Atkin’s Diet recommends: 10% carbohydrates, 55% fats, 35% protein

Brother Alan:

“I think the best thing for you to do is exercise and stop eating all of that pizza and candy and food filled with fat. Our people never used to eat that junk. I say eat meals that are nutritionally balanced and high in fiber and low in refined sugars and saturated fats. And run a lot. Stop sitting around the house.”

Grandfather:

“Morgan, it is true that it is best to live in harmony and balance – to eat a nutritionally balanced diet that includes carbohydrates, proteins, and ‘good’ fats to meet your body’s needs. That is good. But look to your heritage. Our people were never overweight before we started eating ‘civilized’ food. Look to our native diet. I think you should look to traditional herbal remedies for help. What Alan says is true. Exercise is good. But it is sometimes not enough. Our people have always looked to natural medicine for cures. They will lift your spirits and energize you. Then you will lose weight and you will be healthy again. I hear the herbs from the diet shop are safe and good. But you must take them like the medicine label says. It can’t hurt to try them.”

*Note: these herbal supplements Morgan’s grandfather is recommending contain the herb

ephedra (ephedrine).

Question 5:

Given Morgan’s metabolic state, what is the primary fuel source for her cells currently?

A. Carbohydrates (glucose)

B. Fats

C. Proteins

D. It is a balance of all three

Question 6:

Metabolic ketoacidosis is a common problem with diabetics, which is caused by which of the following?

A. Excessive oxidation of fatty acids, leading to an accumulation of ketone bodies in the blood.

B. Excessive oxidation of glucose, leading to an accumulation of ketone bodies in the blood.

C. Excessive oxidation of proteins, leading to an accumulation of ammonia in the blood.

D. Hyperglycemia.

Question 7:

Which weight loss suggestion is designed to achieve some level of ketosis?

A. Atkins Diet

B. Exercise

C. High fiber diet

D. Herbal treatments

Question 8:

One major side effect of the herbal supplement can be hot flashes and increased perspiration due to increased thermogenesis, what is the likely cause of the heat production?

A. Increasing the activity of phosphfructokinase-1.

B. Increasing the activity of the pyruvate dehydrogenase complex.

C. Increasing the activity of citrate synthase.

D. Increasing the activity of uncoupling proteins.

Question 9:

Would substituting fiber and complex carbohydrates (larger % cellulose) for the simple sugars and starches (high sucrose and amylose/amylopectin) help Morgan lose weight, why?

A. Yes, because complex carbohydrates like cellulose are largely indigestible so they pass through

the digestive tract without being absorbed.

B. Yes, cellulose contains less glucose residues than amylose or amylopectin.

C. Yes, because the fructose in sucrose is much more responsible for weight gain than the glucose.

D. No, because cellulose, amylose, and amylopectin are all composed of glucose so it doesn’t

matter.

Question 10:

Which weight loss suggestion will lead to an increased need for gluconeogenesis?

A. Atkins Diet

B. Exercise

C. High fiber diet

D. Herbal treatments

Case Study Section 4 – Nitrogen Metabolism Based on “A Sickeningly Sweet Baby Boy: A Case Study on Autosomal Recessive Inheritance” by Jacqueline Washington and Anne Zayaitz (National Center for Case Study Teaching in Science) The Patient: Mathew Miller is a 7-day-old infant that is rushed to the emergency room by his parents Emma and Jacob Miller, Mennonites from Lancaster County, PA. Emma’s pregnancy and delivery with Mathew was normal but he started having trouble nursing and now has completely stopped feeding. By the time they reach the emergency room Mathew’s limbs were rigid and he had had a seizure. The initial examination showed no infection and his x-rays were normal. The family history collected by the Doctor revealed that Emma and Jacob had had a previous son, Samuel that died at 9 days-of-age. There also is a history of unexplained childhood mortality on both sides of the family; Emma’s mother had two sisters who died in the first year of their lives and Jacob’s father had a sister who died at 7 months of age. Emma also points out to the Doctor that Mathew’s diaper has a funny smell to it. Blood and urine samples were taken for testing and skin biopsies from Mathew, Emma, and Jacob were taken and tested for enzyme activity. The tests results showed that:

• Mathew’s urine had elevated levels of the branched chain amino acids and their α-keto acid derivatives (which caused the sweet smell in his diapers)

• Both Emma and Jacob’s skin biopsies had nearly normal levels of branched chain amino acid metabolism/enzyme activity

• Mathew’s enzyme activity was less than 2% of normal

Question 1: What is the most likely diagnosis for Mathew?

A. Argininemia

B. Diabetes mellitus '

C. Maple syrup urine disease

D. Phenylketonuria

Question2: What enzyme did the skin biopsy test measure?

A. Carbamoyl phosphate synthetase I

B. Alanine aminotransferase

C. Phenylalanine hydroxylase

D. Branched-chain α-keto acid dehydrogenase complex

Question 3: This enzyme functions VERY similarly to two other enzymes that we have discussed, what are they?

A. Phosphofructokinase-1 and fructose 1,6-bisphosphatase (FBPAse-1)

B. Pyruvate dehydrogenase complex and α-ketoglutarate dehydrogenase complex

C. Urease and arginase

D. Isocitrate dehydrogenase and malate dehydrogenase

Question 4: How did Mathew (and presumably Samuel) inherit the disease if both Emma and Jacob have normal enzyme activity levels?

A. Emma and Jacob must both be carriers for the disease but their one normal gene is enough to maintain enzyme activity levels.

B. The disease can happen spontaneously, there is no genetic link.

C. Jacob is not really the father of the boys.

D. The disease can skip generations.

Question 5: What would be the best diet for Mathew to follow to control his disease?

A. Atkin’s diet (low carbohydrates, high fat, and high protein)

B. Low protein

C. Low fat

D. No special diet will control the disease

Over the next 4 years Mathew’s metabolic disease is controlled by his special diet but he still suffers approximately 3 metabolic crises a year. These occur when amino acids accumulate in his blood leading to the swelling of his brain. Even something as simple as a cold or the flu can affect his amino acid levels and send his metabolism into crisis. Jacob and Emma decide that “We cannot continue to live in constant fear that a minor infection or a simple cold or ear infection could kill our son. Even though we are doing everything we are supposed to, he is still getting sick and we are afraid we may lose Mathew.” They learn that some children with Mathew’s disease have undergone liver transplants and are effectively cured.

Question 6: How/why would a liver transplant effectively cure Mathew’s condition?

A. A new liver would be able to spread it’s genes throughout the body and correct the genetic disorder.

B. A new liver would more effectively respond to metabolic hormonal control.

C. A new liver would express the functional enzyme needed to metabolize the amino acids causing Mathew’s condition.

D. A new liver would produce more hemoglobin to transport oxygen more effectively.

The family is directed to Dr. Morton of Children’s Hospital of Pittsburgh, where transplant experts agree to list Mathew for a liver transplant. Jacob and Emma learn that children who receive a liver transplant have to take strong immunosuppressive drugs for the rest of their lives. It was also clear that there was a 40% possibility that Mathew could reject the liver and need a second transplant (which also might be rejected) or he could die from surgical complications.

Question 7: Assuming the liver transplant was successful and Mathew did not reject the new liver, what type of diet would Mathew be able to/need to eat?

A. He would still need to eat his special diet but he would be at less risk of a metabolic crisis

B. He could eat a normal diet

C. He would need to change to a high protein diet like Atkin’s

D. He would be on an all liquid diet

Question 8: Emma and Jacob decide they want to try to have another child. What would be the chance that this child would also have the disorder that Mathew and Samuel have/had?

A. 0 %

B. 25%

C. 50%

D. 75%

E. 100%

Question 9: If Mathew grew up, got married, and decided to have children, what would be the chance that his children would have the disorder? A. 0%, the liver transplant would have cured him

B. 25%

C. 50%

D. 100%

E. It would depend on his wife, if she was normal than there would be a 0% chance of his children having the disorder but 100% would be carriers, if she was a carrier than there would be a 50% chance of his children having the disorder and a 50% chance that they would be a carrier.

Question 10: The disorder is found in 1 in 200,000 infants throughout the US but 1 in 200 in the Amish and Mennonites of Lancaster County, PA. Why is there such a difference in the prevalence of the disease?

A. There are environmental factors associated with being Amish or Mennonites that increase the prevalence.

B. There is a large amount of genetic similarity within these populations because they tend to marry within the population so recessive disorders such as this are more likely.

C. The gene mutation is preferentially carried in Caucasians so when you average the prevalence across all races in the US the prevalence drops.

D. It is unknown why the prevalence is so much greater in Amish and Mennonites.

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