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  CATEGORIES ABOUT FACTOR XIII
   
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ec 2.3.2
   
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FXIII is known also as Laki-Lorand factor.


GENETICS


Zymogene factor XIII is a 320000 Mr glycoprotein consisting of twice two subunits (2 A and 2 B), the Gene s for which are on different Chromosome s:
  • A subunit (6p25-p24). The Transglutaminase part; this adds an alkyl group to the nitrogen on a Glutamine residue, which binds in turn with a Lysine on the other chain. The molecular weight of the A chain is approximately 83000.

  • B subunit (1q31-q32.1). This has no clear enzymatic activity, and may serve as a carrier for the A subunit. The molecular weight of the B chain is approximately 76500.



PHYSIOLOGY

Typical concentrations of FXIII in plasma is 10 μg/ml (2A2B heterodimer), while the concentration of free B chain is 22 μg/ml. FXIII has a long half life, ranging from 5-9 days.


ROLE IN DISEASE

Factor XIII deficiency may occur very rarely, and can cause a severe bleeding tendency.


SEE ALSO



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