Although susceptibility to infections in Kabuki make-up syndrome (KMS) has frequently been reported, there have been few immunological studies. We describe a 14 year old girl with KMS exhibiting chronic idiopathic thrombocytopenic purpura (chronic ITP), including immunological studies.Corticosteroid therapy was not effective therefore splenecfomy was performed. The patient's platelet count increased transiently. Immunological studies revealed normal T cell functions and low serum immunoglobulin A (1gA) levels.Because of the abnormalities of B cell functions in chronic ITP and low serum IgA levels in our patient, we speculate that there may be some abnormalities of humoral immunity in KMS.

Kinetic studies of the reduction of plastocyanin from spinach chloroplasts by ascorbate were carried out using a stopped flow apparatus. The reduction of spinach plastocyanin by ascorbate followed first-order kinetics in the reductant with a rate constant k = 415 M-1.S-1 [20 degrees C, 0.1 M NaCl, pH 8.0 (10 mM Tris-HCl)]. The second-order rate constants for the reduction of plastocyanin by ascorbate increased dramatically with increasing pH and ionic strength. The cationic micelles of cetyltrimethylammonium bromide also accelerated this electron-transfer reaction. The importance of electrostatic interactions between plastocyanin and ascorbate is discussed in relation to the photosynthetic electron-transport reactions.

The rates of the reduction of Nitroblue Tetrazolium by O2− during the autoxidation of pyrogallol, epinephrine, and catechol were increased 110, 55, and 37-fold respectively in the presence of hexadecyltrimethylammonium bromide. A nonionic micelle of polyoxyethylene dodecyl ether also accelerated these reactions. The microenvironment effects on the electron-transfer from O2− to Nitroblue Tetrazolium were discussed.

2-p-Toluidino-naphthalene-6-sulfonate is a sensitive fluorescent reporter group which can be used for the detection of the conformation of fructose 1,6-diphosphatase from spinach chloroplasts. When fructose 1,6-diphosphatase was added to a dilute solution of 2-p-toluidino-naphthalene-6-sulfonate at pH 9.0, the fluorescence intensity gradually increased. At this pH, the enzyme activity decreased at the same rate. However, at neutral pH (7.5), this time-dependent fluorescence change was not observed. In the presence of Mg2+, which is an activator of the enzyme, the fluorescence intensity was increased instantly and did not change for 30 min in the pH range 8.0--9.0. From the concentration dependence of the fluorescence intensity, the dissociation constant for Mg2+ was determined, Kdis = 3 mM. The effects of pH and Mg2+ on the conformation and activity of chloroplast fructose 1,6-diphosphatase are discussed.

In the absence of EDTA, the rate of autoxidation of dithiothreitol (2•~10-5M) increased with an increase of the concentration of Cu2+, and reached to the maximum at 5•~10-8 M Cu2+. And then the rate declined and the autoxidation of dithiothreitol ceased at 1.6•~10-8 M Cu2+ In the presence of EDTA, both the rate and the optimum concentration of Cul+ increased. A model which involves Cu2+-EDTA or Cu2+-(dithiothreitol)2 and 02 complexes for the catalysis of the autoxidation of dithiothreitol was proposed.