Purified radiolabeled antithrombin III metabolism in three families with hereditary AT III deficiency: application of a three-compartment model
Purified human radioiodinated antithrombin III (125I-AT III) was used to study its metabolism in six members from three different families with a known hereditary AT III deficiency. Six healthy volunteers served as a control group. Sodium dodecyl sulfate-polyacrylamide gel electrophoresis (SDS-PAGE) and crossed immunoelectrophoresis (CIE) showed the purified AT III to be homogeneous. Amino acid analysis of the protein revealed a composition identical to a highly purified internal standard. The specific activity was 5.6 U/mg. Analysis of plasma radioactivity data was performed, using a three-compartment model. Neither plasma disappearance half-times nor fractional catabolic rate constants differed significantly between patients and control subjects. The mean absolute catabolic rate in the patient group was significantly lower than that of the control group at 2.57 +/- 0.44 and 4.46 +/- 0.80 mg/kg/day, respectively. In addition, the mean patient alpha 1-phase, flux ratio (k1,2 and k2,1) of the second compartment alpha 2-phase and influx (k3,1) of the third compartment were significantly reduced as compared with control values. It has been tentatively concluded that the observed reduction in the second compartment may be caused by a decrease in endothelial cell surface binding.
- Research Organization:
- Univ. of Amsterdam, The Netherlands
- OSTI ID:
- 5881313
- Journal Information:
- Blood; (United States), Vol. 1
- Country of Publication:
- United States
- Language:
- English
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Related Subjects
ANTICOAGULANTS
METABOLISM
AMINO ACID SEQUENCE
BLOOD COAGULATION FACTORS
BLOOD PLASMA
HEMIC DISEASES
HEREDITARY DISEASES
IODINE 125
LABELLED COMPOUNDS
PROTEINS
BETA DECAY RADIOISOTOPES
BIOLOGICAL MATERIALS
BLOOD
BODY FLUIDS
COAGULANTS
DAYS LIVING RADIOISOTOPES
DISEASES
DRUGS
ELECTRON CAPTURE RADIOISOTOPES
HEMATOLOGIC AGENTS
INTERMEDIATE MASS NUCLEI
IODINE ISOTOPES
ISOTOPES
MATERIALS
MOLECULAR STRUCTURE
NUCLEI
ODD-EVEN NUCLEI
ORGANIC COMPOUNDS
RADIOISOTOPES
550601* - Medicine- Unsealed Radionuclides in Diagnostics