1. Ketosis:-

Normal concentration of ketone body is very low in the blood (0.2 mmol/L)

Hepatic cell: responsible for the synthesis of ketone bodies

Non-hepatic cell: utilization of ketone bodies

So when the rate of formation of ketone bodies  exceeds there utilization, there is increase in the blood concentration of blood

Rise in ketone bodies in blood:- ketonemia

Rise in ketone body in urine:- ketonuria

Ketonemia + ketonuria = ketosis

Causes:- low level of ketone and carbs

Treatment:– a special ketogenic diet

2. Ketoacidosis:-

Acetoacetate and beta hydroxybutyrate  behave as moderate acids. So if their concentration increases in the blood, the bicarbonate (HCO3-) compensate this. After performing this function, the concentration of bicarbonate ion decreases In the blood and deplete the alkali reserve. This leads to ketoacidosis (blood Ph decreases).

This condition is mainly found in Type-1 diabetes and rarely found in type-2 diabetes.

Causes:- dehydration, heart attack, drug or alcohol abuse, inadequate insulin therapy etc

Treatment– fluid intake, insulin therapy, electrolytes, regular checking of sugar level

3. Carnitine Transport system Disorders:-

This disorder is due to the impaired flow of fatty acids from cytosol to the mitochondria and cause pathological changes. No of disease occurs due to the deficiency of

• Carnitine
• Carnitine acyl transferase
• Translocase of the system

Carnitine deficiency does not allow the long chain fatty acids to enter into the inner membrane of mitochondria.

The fatty acids which cannot transport are accumulated within the cytosol of the muscle cell; this leads to reduced muscular contraction.

Medium chain fatty acids which do not require carnitine to enter the mitochondria are oxidized normally in the patient.

4. Sphingolipidoses:-

Sphingopholipidoses is the group of inherited lysosomal diseases. In a normal individual, the synthesis and degradation of sphingolipids are balanced so that the amount of these compounds present in the membrane is constant.

When the hydrolytic enzyme liberated by the lysosomes is genetically defective, it incompletely degrades the sphingolipids. This leads to the accumulation of insoluble sphingolipids in the lysosomes. The lysosomes then become swollen to such a extent, that it disturbs the cellular functions

Since brain is excessively rich of sphingolipids, so if its degradation is interrupted; It disturbs the normal brain function and can lead to mental retardation.

Causes- genetic disorder, metabolic abnormalities

Treatment:- enzyme replacement therapy, substrate reduction therapy, pharmacological chaperones

5. Atherosclerosis:-

It is a cholesterol metabolism disorder. Atherosclerosis is a general tern for hardening of the arteries. It is characterized by accumulation of cholesterol and lipids on the walls of arteries. This leads to the formation of plaque and constriction of the arteries. Following are some factors which are responsible for atherosclerosis:-

• Age:- age brings changes in the wall of the blood vessel as metabolism of cholesterol decreases by age.
• Sex:- males are more affected than females
• Genetic factors:- hereditary derangement of lipoprotein metabolism leads to high blood lipid level and also due to genetic hypercholesterolemia (high blood cholesterol level)
• Hypertension:- It is the major risk factor after the age of 45 years. Increased blood pressure can lead to the mechanical injury of the wall of blood vessel; which ultimately leads to atherosclerosis.
• Diabetes Mellitus:- the risk is due to co-existing other risk factors such as obesity, hypertension and hyperlipidemia.

Causes;- high cholesterol, high BP, smocking, alcohol consumption, diabetes etc. 

Treatment:- ACE inhibitors, calcium channel blockers, beta blockers, anti-platelet medications

Multiple choice questions (MCQs)

1. ketoacidosis is a disorder of which metabolism?
A. phospholipid metabolism B. ketone body metabolism
C. cholesterol metabolism B. fatty acid metabolism

2. Atherosclerosis Is the disorder of which metabolism?
A. phospholipid metabolism B. ketone body metabolism
C. cholesterol metabolism B. fatty acid metabolism

3. sphingolipidoses is the disorder of?
A. phospholipid metabolism B. ketone body metabolism
C. cholesterol metabolism B. fatty acid metabolism

4. What happens during ketosis?
A. rate of formation exceeds utilization
B. rate of utilization is more than formation
C. both
D. none

5. Match the following causes and their disorders-
a. atherosclerosis 1. Decreased concentration of HCO3
b. sphingolipidoses 2. When the concentration of ketone body rises in blood
c. ketosis 3. Incomplete degradation of sphingolipids
d. ketoacidosis 4. hyperlipidemia

6. Sphingolipidoses leads to the improper functioning of which organ?
A. liver B. stomach
C. brain D. heart

7. Atherosclerosis is hardening of —–?
A. arterial wall B. muscle cell wall
C. nerve cell wall D. brain cell wall
8. Which of the following statement is NOT true?
A. ketosis is associated with ketone bodies
B. in ketoacidosis, blood Ph increases
C. sphingolipidoses is a disorders of sphingolipids
D. atherosclerosis is caused due to hyperlipidemia
9. Carnitine Transport system Disorder is associated with which metabolism?
A. phospholipid metabolism B. ketone body metabolism
C. cholesterol metabolism B. fatty acid metabolism
10. Which of the following factor is responsible for atherosclerosis?
A. age B. genetic factor
C. smocking D. all of the above
11. In sphingolipids, where is insoluble sphingolipids accumulated?
A. mitochondria B. lysosomes
C. ribosomes D. cytoplasm
12. Which of the following is the causes for ketoacidosis?
A. heart attack B. renal failure
C. high cholesterol D. High BP
13. What are the effects due to Carnitine Transport system Disorder?
A. long chain fatty acid cannot transfer B. heart disease
C. accumulation of fatty acid in muscle D. both A and C
14. What is the concentration of ketone bodies in the blood?
A. 2mmol/L B. 0.2mmol/L
C. 0.02MMOL/L D. 20mmol/L
15. Ketosis is mainly found in which disease?
A. rheumatic fever B. diabetes mellitus
C. hypertension D. lung diseases

ANSWERS:-

1. ketone body metabolism
2. cholesterol metabolism
3. phospholipid metabolism
4. rate of formation exceeds utilization
5. a – 4 b – 3 c – 2 d – 1
6. brain
7. arterial wall
8. in ketoacidosis, blood Ph increases
9. fatty acid metabolism
10. all of the above
11. lysosomes
12. heart attack
13. both A and C
14. 0.2mmol/L
15. diabetes mellitus

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REFERENCE:- Pankaja Naik- Biochemistry; 4^th edition; page no:- 222, 223, 224, 236, 255