Sources of Heme
Enzymes of Pathway
Bilirubin Transport
Liver & Conjugation
100

The majority of heme for degradation comes from these cells.

What are red blood cells?

100

The enzyme that converts heme into biliverdin.

What is heme oxygenase?

100

Unconjugated bilirubin is hydrophobic, so it travels in blood bound to this protein.

What is albumin?

100

Bilirubin enters hepatocytes and is conjugated with this sugar.

What is glucuronic acid?

200

When hemoglobin is degraded, the globin chains are broken down into these building blocks, which can be recycled.

What are amino acids?

200

Biliverdin is converted to bilirubin by this enzyme.

What is biliverdin reductase?

200

Why can’t unconjugated bilirubin appear in urine?

Because it is tightly bound to albumin and water-insoluble.

200

The enzyme that conjugates bilirubin.

What is UDP-glucuronosyltransferase (UGT1A1)?

300

This organ is the primary site where aged RBCs are broken down.

What is the spleen?

300

The cofactor required by biliverdin reductase in this reaction.

What is NADPH?

300

Albumin binding keeps unconjugated bilirubin in circulation until it reaches this specific organ for uptake and conjugation.

What is the liver?

300

Conjugated bilirubin is secreted into this organ system.

What is the gallbladder/biliary system?

400

Why does ineffective erythropoiesis (ex: thalassemias) increase bilirubin production?

Issue with globin chain synthesis, but heme synthesis is fine. You will have proportionally more heme compared to functional hemoglobin to put it in. This excess heme will be degraded into bilirubin.

400

These color changes reflect sequential enzyme action: heme gives blood its red color, biliverdin appears green, and bilirubin imparts this color as it accumulates.

What is yellow?

400

What condition can arise if bilirubin builds up and is displaced from albumin in neonates?

Kernicterus

400

The “15% rule” states that if direct bilirubin is greater than 15% of total (when total is >3mg/dL), the problem is with excretion or obstruction. In contrast, if direct bilirubin is less than 15% of total, the cause may be due to ____ or ____.

Ineffective erythropoiesis or hemolytic disease

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