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Subunit A of factor XIII regulates subunit B plasma concentration
Thrombosis and Haemostasis
|October 1, 1981
Summary
Factor XIII subunit a deficiency is linked to reduced subunit b levels. Supplementing subunit a in deficient individuals increased subunit b, indicating subunit a regulates subunit b plasma concentration.
Area of Science:
- Biochemistry
- Hematology
- Genetics
Background:
- Factor XIII is a plasma transglutaminase essential for hemostasis.
- Factor XIII is composed of two catalytic (a2) and two non-catalytic (b2) subunits.
- Deficiency in Factor XIII subunits can lead to bleeding disorders.
Observation:
- Homozygotes for Factor XIII subunit a deficiency lack the subunit entirely.
- Heterozygotes exhibit reduced levels of Factor XIII subunit a.
- A concurrent reduction in Factor XIII subunit b was observed in individuals with subunit a deficiency.
Findings:
- Administration of Factor XIII subunit a to individuals lacking the subunit led to a significant increase in subunit b levels.
- Subunit b levels peaked five days post-infusion of subunit a.
- This demonstrates a regulatory relationship where subunit a influences subunit b plasma levels.
Implications:
- The plasma concentration of Factor XIII subunit b is regulated by the amount of Factor XIII subunit a.
- This finding challenges previous assumptions of independent genetic control for subunits a and b.
- Understanding this regulatory mechanism is crucial for managing Factor XIII deficiency disorders.