Proteins

Question 1

Albumin

Answer 1

Albumin band

• Largest protein component of human serum
• migrates anodally (left)
• produced by Liver
• Maintains fluid balance (colloid osmotic pressure)
• Transport (hormones, fatty acids, vitamins, enzymes, and drugs)
• Stabilizing blood volume
• Detoxifies by binding, such as bilirubin and heavy metals
• Antioxidant properties (protects against free radicals)
• Regulating Blood Ph
• source of amino acids
• normal range: 3.6 - 4.8 g/dl

Hypoalbuminemia: liver disease, malnutrition, protein loss (urine, Gi tract, burns, neoplasia0

Hyperalbuminemia: normal healthy variation, dehydration is the only pathological association

Bisalbuminemia: congenital, induced by drugs (bound drugs cause second peak), in acute pancreatitis

Question 2

Alpha1 Antitrypsin

Answer 2

Alpha 1

• produced in liver
• also called alpha-1 protease inhibitor
• protects the lungs and liver from damage, particularly from inflmmation and infection by inhibiting the action of neutrophil elastase, an enzyme release by white blood cells (emphysema)
• is a protease inhibitor
• deficiency can lead to lung and liver disease (emphysema and COPD)
• liver dz caused by accumulation of abnormal AAT proteins in the liver
• genetic variants may present with multiple peaks

Question 3

Thyroid-binding globulin

Answer 3

Alpha 1

• produced in liver
• binds thyroid hormones T4 and T3, transporting them in blood and regulating their availability to tissue
• T4 (thyroxine)
• T3 - triiodothyronine
• can be influeced by pregnancy, genetic factores, medications like estrogen or androgenic steroids), and certain medical conditions (liver disease, kidney disease)

Question 4

Transcortin

Answer 4

Alpha 1

• also known as corticosteroid-binding globulin (CBG)
• produced in liver
• transports steroid hormones, particularly cortisol, regulating their availability to target tissues
• vital for circadian rhythm, glucose metabolism, arousal, and cognitive functions
• can be elevated by increased estrogen levels

Question 5

Ceruloplasmin

Answer 5

Alpha 2

• produced in the liver
• copper transport
• plays a role in iron metabolism by oxidizing ferrous iron to ferric iron
• this is necessary for iron to be incorporated into transferrin, a protein that carries iron in the blood
• an acute-phase protein
• antioxidant and bactericidal activities
• associated with Wilson’s disease, a genetic disorder that causes copper to accumulate in the body
• regulation of iron levels in the CNS and may prevent free radical injury

Question 6

Alpha 2 Macroglobulin

Answer 6

Alpha 2

• broad-spectrum protease inhibitor that acts as a molecular trap to bind and inactivate various proteases
• regulating extracellular proteolysis, immune function, and potentially in conditions like Alzheimer’s disease
• can bind variouos cytokines and growth factors, potentially influencing cell signaling and immune response
• some research suggests my be involved in clearance and degradation of beta-amyloid, a protein that accumulates in the brains of people with Alzheimer’s disease
• normal range: 0.15 - 0.35 g/dl
• Elevated: is not eliminated through kidney because of its huge molecule size, it may increase 10 fold in nephrosis
• no specific disease states are associated with low levels
• mobility may change with storage

Question 7

Haptoglobin

Answer 7

Alpha 2

• binds free hemoglobin released during red blood cell breakdown (hemolysis), preventing iron loss, kidney damage, and oxidative stress
• an acute-phase reactant
• is used to help diagnose and monitor hemolytic anemia
• can be low in presence of hemolytic anemia as it binds the excess free hemoglobin
• increased in hypoproliferative anemia, including iron deficiency and in hemolysis in vitro
• decreaed immediately following intravascular hemolysis, hemolytic anemia, transfusion reaction, malaria
• mey present with different phenotypes leading to a confusing pattern (wrong fraction)
• place overlay of normal pattern over this pattern to determine with belongs to alpha and beta

Question 8

Transferrin

Answer 8

Beta 1 (almost all of it)

• transport iron throughout the body
• produced in liver
• by binding to iron, transferrin makes it soluble and prevents iron from oxidizing, which can lead to the formation of harmful free radicals
• increased in iron deficiency
• decreased in iron overload, acute or chronic inflammation

Question 9

Beta-lipoprotein

Answer 9

Beta 2

• transporting cholesterol and fats in the bloodstream
• high levels can lead to plaque buildup in arteries, increasing the risk of heart disease

Question 10

Complement proteins

Answer 10

Beta 2
- almost all of it
- C3 is only one at sufficient levels to be detected
- decreased in >3 day old sample
- increased: late stage of acute phase response or comigrating monoclonal band
- normal range: 2.0 - 5.4% of TP

Question 11

C-Reactive protein

Answer 11

between beta and gamma

• produced by liver
• serves as a marker for inflammation in the body
• normally undetectable, but when elevated, may distort this region
• detect inflammation
• monitor infections
• elevated in high-sensitivity CRP (hs-CRPO) can indicate an increased risk of cardiovascular disease
• monitoring chronic inflammatory diseases
• may be mistaken for a monoclonal band

Question 12

Prealbumin

Answer 12

• also called transthyretin
• positioned anodally (to the left)
• frequently not at detectable levels
  usually just included in Albumin quantitation
• transports serum thyroxine, and vitamin A (retinol)
• low levels indicate malnutrition
• not accurately measurable in electrophoresis
  -normal range: 0.02 - 0.04 g/dl

Question 13

Alpha-1 Fraction proteins

Answer 13

• just cathodal (ro the right of of) Albumin
• major proteins: alpha 1 acid glycoprotein, alpha-1 antitrypsin
• minor bands: alpha 1 lipoprotein (HDL) at the Albumin-alpha1 junction), alpha 1 antichymotrypsin (alpha1-alpha 2 junction)
• different phenotypes of these proteins may complicate the pattern, present as multiple bands
  -normal range: 0.2 - 0.5 g/dl

Question 14

Alpha-1 acid glycoprotein

Answer 14

• also called orosomucoid (ORM)
• major acute phase protein
• not readily measured due to co-migration with other proteins
• low stain affinity: not visible on gel (generally higher peak on alpha 1 in CEP)

Question 15

Alpha-2 Fraction proteins

Answer 15

• normally a single peak cathodic to alpha-1 fraction
• major: Haptoglobin, alpha-2 macroglobulin
• minor: ceruloplasmin and Gc globulin
• decreased alpha-2 values are due to intravascular hemolysis
• increased alpha-2 values are due to: acute inflammation, nephrotic syndrome, hemolysed samples (hemolysis of sample after drawing)
• normal range: 0.5 to 0.9 g/dl

Question 16

BETA - GAMMA region

Answer 16

• frequent area of difficult interpretation due to proteins migrating atop or in broad regions including this border
• position of: C-reactive protein, IgA, and the IgG4 subtype: of interest in screening for multiple myeloma

Question 17

Gamma Fraction

Answer 17

• immunoglobulins migrate here (except IgA)
• decreased: elderly, congentital immunodeficiency, immunosuppressive treatment (HIV, Transplant, etc)
• increased in: lymphoplasmocytic syndromes such as multiple myeloma, hyper immunization, acute infections, chronic, liver disease, chronic inflammatory disease