Classification of Proteins and their related disorders

Classification of
Proteins
Based on Composition and solubility

I. Simple proteins
II. Conjugated proteins
III. Derived proteins

Classification based on function

Classification based on Nutritional value

Simple Proteins:

These upon hydrolysis yield only amino acid or their derivatives.

Albumins
Globulins
Globins
Protamines
Prolamines
Lectins
Scleroproteins
 ALBUMINS
• Soluble in water and Easily Coagulated by Heat
• Mol. Weight about 70,000
• Deficient in glycine
• Synthesised by hepatocytes 25% of total hepatic protein
synthesis(12gm/day) Synthesis controlled by colloidal osmotic
pressure (COP) & protein intake
Half life is about 20 days
Important Liver Function Test
Human serum albumin: 69,000
Egg albumin
Lactalbumin
 Important functions:
Functions:
1. Effective Osmotic Pressure
Colloidal Osmotic Pressure
Total Osmotic Pressure : 5000 mm Hg
80% by albumin; 20% globulins
2. Transport of hydrophobic substances
Specific carriage
Bilirubin
Non-esterified fatty acids (NEFA)
Non-specific carriage
Steroids, Thyroxin, Calcium, Copper, Aspirin, Sulpha drugs
Dicoumarol, Phenytoin
3. Buffering (histidine residues)
4. Nutritional-- carries essential amino acids from liver to extrahepatic tissue.
5. Important role to maintain blood volume & body fluid distribution hence low
albumin concentration leads edema
Edema when Albumin < 2 g / dl
 Clinical
Significance

Normal Albumin level in blood:

3.6 – 5.4 g /dl
Hypoalbuminemia (< 2g/dl)
• Reduced synthesis:
Cirrhosis, liver failure
Malnutrition (generalised edema)
Malabsorption syndromes
• Abnormal distribution:
Ascites
Cirrhosis of liver where synthesis of albumin is less & it
Generalized Edema
escapes
into ascetic fluid
• Increased Catabolism
Injury ex. Major surgery or trauma
Infection
Fever
Malignant diseases
• Overhydration
 Protein-Energy Malnutrition
The World Health Organization (WHO) defines malnutrition as “the cellular
imbalance between the supply of nutrients and energy and the body's demand
for them to ensure growth, maintenance, and specific functions.”

The term protein-energy malnutrition (PEM) applies to a group of

related disorders that include Marasmus and kwashiorkor
 Malnutrition and Malabsorption
Malnutrition is a condition that results from eating a diet in which one or more
nutrients are either not enough or are too much such that the diet causes health
problems. It may involve calories, protein, carbohydrates, vitamins or minerals.
821 million undernourished i.e.11% of the population (2017)

Malabsorption is a disorder that occurs when people are unable to absorb

nutrients from their diets, such as carbohydrates, fats, minerals, proteins, or
vitamins. Some commonly known disorders related to malabsorption are
lactose intolerance and celiac disease
More than 1 million cases per year (India)
 Kwashiorkor :
Insufficient protein consumption –
Child weaned: mother’s milk to starchy vegetable
Symptoms:
• Weight loss
• arms and legs -decrease of muscle mass
• Swollen abdomen
• Ascites: increase of capillary permeability
• Enlarged liver: fatty liver
• Peripheral oedema: decrease of oncotic pressure
• Anaemia: lethargy
• Hair and skin changes
• Moon face
 Marasmus
Marasmus -Energy deficiency
Marasmus is a severe form of protein-energy malnutrition that results when a person
does not consume enough protein and calories. Without these vital nutrients, energy
levels become dangerously low and vital functions begin to stop.
Symptoms:
Weight loss: “skin and bones”
•Prominent of ribs
•Drastic loss of adipose tissue
•Growth retardation
•Chronic diarrhea
•Muscle atrophy
•Skin folds
•“old man”face
Complications of PEM
•Hypoglycemia
•Hypothermia
•Dehydration and shock
•Electrolyte imbalance –hypokalemia (low K) and
hyponatremia (low Na)
•Infections (bacterial, viral and thrush)
•Micronutrient deficiencies
 Treatment and prevention:
• Correction of water and electrolyte balance
• Treat infection and worm infestations
• Dietary support 3-4g protein and 200 cal/ kg
body weight/day -Vitamins and minerals
• Prevention of hypothermia
• Counsel parents and plan future including
immunization and diet supplements
 Initial “refeeding”

Continue breast feeding

•Add frequent small feeds
•Use liquid diet
•Give vitamin A and folic acid on admission
•With diarrhea use lactose free or soya bean
formula
Take home message
•Protein-energy malnutrition = undernutrition
•+++ in developing countries
•2 types of PEM
-Kwashiorkor: protein deficiency + oedema
-Marasmus: Protein + calories deficiency
• Infection major cause of death
•Treatment:
-progressive “refeeding”
-treatment of infections
 Nephrotic syndrome
• Nephrotic syndrome is a kidney disorder that causes body to excrete too much
protein in your urine.
• Nephrotic syndrome is often caused by damage to small blood vessels (glomeruli)
in the kidneys that filter waste and excess water from the blood.
Signs and symptoms of nephrotic syndrome:
• Too much protein in urine (proteinuria)
• Low levels of protein in blood (hypoalbuminemia)
• High levels of fat and cholesterol in blood.
• Severe swelling (edema), particularly around eyes, and in
ankles and feet
• Foamy urine, which may be caused by excess protein in urine
• Weight gain due to excess fluid retention
• Fatigue
• Loss of appetite
 Diagnosis
Tests used to diagnose nephrotic syndrome include:
1. Estimation of serum proteins
2. Detection of proteins in urine
3. A sample of kidney tissue for testing.

Treatment

4. Blood pressure medications.

5. Water pills (diuretics) help control swelling by increasing
kidneys' fluid output.
6. Cholesterol-reducing medications
7. Blood thinners
8. Immune system-suppressing medications.
GLOBULINS
Soluble in mild acid / alkali / salt
Mol. Wt. 150,000 D
Coagulation
Human serum globulins
Legumin of peas
Ovaglobulin of egg
Bigger in size than albumin
Globulins are Precipitated by Half Saturation with Salts
M.Wt 90,000 - 1,30,000

α1 GLOBULIN
Synthesized in Liver
α2 GLOBULIN

 GLOBULIN Reticulo Endothelial System

 Globulin
 α1 Globulin
1. Retinol Binding Proteins(RBP)

2. α1 protease inhibitor (API)/ α1 antitrypsin

3. α1 fetoprotein (AFP)
4. α1 acid glycoprotein (AAG)
5. Prothrombin
α2 Globulins

1. Ceruloplasmin ( ferroxidase)
2. Corticosteroid binding globin
3. Hepatoglobin

4. Thyroxin binding globulin (TBG)

 β Globulin
1. Transferrin
2. C-reactive proteins (CRP)
3. Hemopexin
4. β microglobins (BMG)

Other important plasma proteins

1. γ Globulins – (immunoglobulins)
2. Bence Jone proteins
 α1 protease inhibitor (API)/ α1 antitrypsin
Emphysema
It is a lung condition that causes shortness of breath. In people with
emphysema, the air sacs in the lungs (alveoli) are damaged. Over time,
the inner walls of the air sacs weaken and rupture creating larger air
spaces instead of many small ones

• Alpha-1-antitrypsin (also known as alpha-1-

antiprotease) is a substance that fights a
destructive enzyme in the lungs called trypsin (or
protease).
• Emphysema is a form of chronic (long-term) lung disease.
• This is called “obstructive” because it’s as though something is blocking
the smooth flow of air in and out of the lungs.

What Causes Emphysema?

 Deficiency of an enzyme called alpha-1-antitrypsin it is a heredity

disease.
 Cigarette smoking:

 Cigarette smoke directly affects the cells in the airway responsible for
clearing mucus and other secretions.
 Secondly it looses proteins responsible for keeping the lungs elastic.
 In addition, the tissue separating the air cells (alveoli) from one another
also destroyed.
 Symptoms and Signs:

• Shortness of breath is the most common symptom of emphysema.

• Cough, sometimes caused by the production of mucus, and
wheezing may also be symptoms of emphysema.
• Tolerance for exercise decreases over time.
One of the hallmark signs of emphysema is "pursed-lipbreathing." The person
with emphysema struggles to exhale completely, in an attempt to empty trapped
air.
 Wilson's disease (Low Ceruloplasmin)

Wilson's disease is a genetic disorder in which copper builds up

in the body. Symptoms are typically related to the brain and liver.

• Autosomal recessive disease

• Defective biliary excretion of copper
• Majority diagnosed 15-35 years of age
• Children present with liver disease, adults with neurologic
disease
Sign and symptoms

• In a healthy body, the liver filters out excess copper and releases it
through urine. With Wilson’s disease, the liver cannot remove the
extra copper properly. The extra copper then builds up in organs
such as the brain, liver, and eyes.
Liver-related
• weakness,
Neurological
• feeling tired, •memory, speech loss
• weight loss, •abnormal walking
• nausea, •insomnia
• vomiting, •personality changes
• loss of appetite, •changes in mood
• itching, •depression
• jaundice, or yellowing of the skin,
• edema, or the swelling of legs and
abdomen
• pain or bloating in the abdomen,
• muscle cramps
Kayser-Fleischer rings and sunflower cataract

• K-F rings are abnormal golden-brown

discolorations in the eyes that are caused by
deposits of excess copper.
• K-F rings show up in about 97 percent of people
with Wilson’s disease.
• Sunflower cataracts show up in 1 out of 5
people with Wilson’s disease.
GLOBINS
Not soluble in salt solutions
Hemoglobin

PROTAMINES
Soluble in water
Not coagulated
Arg / Lys; strongly basic
Nucleoprotein of sperm

PROLAMINES
soluble in 70 - 80% alcohol
Rich in proline & deficient in lysine
Zein in corn
Gliadin of wheat
Hordein of barley
GLUTELINS
Soluble in dilute acids & alkalies
Plant proteins
Glutelin of wheat
Oryzenin of rice

HISTONES
Soluble in water
Not Coagulable
Rich in basic A A
Nucleoproteins

SCLEROPROTEINS
Insoluble in water / mild acid / organic solvents
Soluble only in hot strong acid
Gelatin: it is derived from collagen.
• Used in food industries
• In treatment of osteoarthritis, rheumatoid
arthritis, osteoporosis to strengthening
bones, joints and fingernails.
• Typically constituent shell of drugs

Collagen ( rich in glycine, hydroxy proline & hydroxy lysine)

X-Y-G
bone, cartilage, eyes

Keratin
Hair, nail, horn & hoof Collagen and elastin together
Protect epithelial cell from damage gives strength and shape to
tissue
Elastin
In elastic tissue like tendons and arteries
Provides softness and elasticity
Conjugated proteins

Glycoproteins
Lipoproteins
Nucleoproteins
Chromoproteins
Phosphoproteins
Metalloproteins
 Conjugated
Proteins
Protein + Prosthetic group
GLYCOPROTEINS

Cell surface antigens

Blood group antigens

More than 10% :

Mucoproteins or proteoglycans
Glycosaminoglycan

Lesser than 4% :
Glycoproteins
Mucin of saliva, Ig, FSH, LH, TSH etc.
LIPOPROTEINS
Cell membranes; serum lipoproteins

NUCLEOPROTEINS
Histones (Lysine)

CHROMOPROTEINS
Heme + Globin Hemoglobin (red)
Riboflavin + Protein Flavoprotein (yellow)

PHOSPHOPROTEINS
Serine / Threonine / Tyrosine
Casein of milk; Vitellin of Egg yolk
Enzyme activation
METALLO PROTEINS
Hemoglobin : Iron
Myoglobin : Iron
Cytochromes : Iron

Ceruloplasmin : Copper
Tyrosinase : Copper
Carbonic anhydrase : Zinc
 DERIVED PROTEINS

A.Primary derived :

Protein Proteans Metaloproteins

B. Secondary derived :

Metaloproteins Peptones
Peptides Amino acids
Classification Based on Functions of Proteins

1. Catalytic proteins
Enzymes
Glucokinase, Hexokinase, Dehydrogenase, Transaminase
etc.
2. Structural proteins
Collagen, a fibrous protein of tendons
Elastin, elastin of ligaments
Keratin of hair and nails
3. Contractile proteins
Actin, Myosin ability to contract and function in the
contractile system of skeletal muscle
4. Transport proteins
Hemoglobin, myoglobin, transferrin, Ceruloplasmin,
Apolipoproteins etc.
5. Regulatory proteins or hormones
Insulin and glucagon
Growth hormone like
platelet derived growth factors (PGDF) and epidermal growth factor
(EGF) controls cell division and differentiation

7. Protective/ Defence proteins

Defend against invasion of foreign substances, such as
bacteria, viruses and other organisms
Immunoglobulins or antibodies
Interferons
Clotting factors ex. Fibrinogen and thrombin
6. Genetic proteins
Histones
7. Stress Response proteins
A capacity of living organisms to survive a variety of abiotic stress
is mediated by such proteins

Cytochrome P450 an enzyme converts toxic compounds to non toxic

derivatives

Metallothionein, cysteine rich protein binds to toxic metals like cadmium,

mercury and silver to reduce their ill effect

Heat shock proteins a family of proteins that produced by cells in response

to exposure to stressful conditions like heat, cold UV light etc.