Proper SUMO-1 conjugation is essential to DJ-1 to exert its full activities.

DJ-1 is a multifunctional protein that plays roles in transcriptional regulation and antioxidative stress, and loss of its function is thought to result in the onset of Parkinson's disease (PD). Here, we report that DJ-1 was sumoylated on a lysine residue at amino-acid number 130 (K130) by PIASxalpha or PIASy. The K130 mutation abrogated all of the functions of DJ-1, including ras-dependent transformation, cell growth promotion and anti-UV-induced apoptosis activities. Sumoylation of DJ-1 was increased after UV irradiation concomitant with a pI shift to an acidic point of DJ-1. Furthermore, L166P, a mutant DJ-1 found in PD patients, and K130RX, an artificial mutant containing four mutations in DJ-1, were improperly sumoylated, and they became insoluble, partly localized in the mitochondria and degraded by the proteasome system. Both L166P-expressing cells and DJ-1-knockdown cells were found to be highly susceptible to UV-induced cell apoptosis.

[Short-latency somatosensory evoked potentials in patients with hypertensive intracerebral hemorrhage: clinical availability of N20 component].

Short-latency Somatosensory Evoked Potentials (SSEPs) were studied in 14 patients with putaminal hemorrhage and 17 patients with thalamic hemorrhage. After median nerve stimulation SSEPs were recorded from the electrodes placed on C3', C4' (2cm behind C3 or C4 of the internal 10-20 system respectively), and Cv7. (Reference was the linked ears.) Erb's potential was also recorded from the Erb's point using the contralateral one as the reference. The patients were classified into three groups according to central conduction time laterality index (CCT LI). CCT LI = (CCT on the affected side which is defined as the interpeak latency between N13 and N20)--(CCT on the non affected side). Group 1: CCT LI < or = 0.73 (it means within normal limit); Group 2: CCT LI > 0.73 (it means the significant latency delay of the N20 on the affected side); Group 3: CCT LI is not available (because the N20 was abolished on the affected side). We analyzed retrospectively the localization of the hematoma on CT scans, the degree of motor and sensory disturbance of the upper extremity in each group. Of the 14 patients with putaminal hemorrhage, 5 were in group 1; 3 were in group 2; 6 were in group 3. Patients in group 1 had a localized hematoma within the pallido-putamen complex. Patients in group 2 had a hematoma compressing the posterior limb of the internal capsule. Patients in group 3 had a hematoma involving the posterior limb of the internal capsule and had severer motor disturbance than those in group 1 or group 2.(ABSTRACT TRUNCATED AT 250 WORDS)

[A study of pathogenesis and symptoms of Tourette's syndrome--mainly on the importance of "startle reflex" through Latah reaction].

There is no established theory on pathogenesis of Tourette's syndrome, but recently, a theory of the British school of behaviorism; 'transient tics' and the more serious case, Tourette's syndrome which ensues on the fixation of the startle reflex, have received attention. So we determined, whether pathogenesis and symptoms of Tourette's syndrome could be systematically explained by "the startle reflex theory". Authors assumed that the startle reflex was composed of two divided actions, a series of muscular movements which started with eye blinks and terminated in the flexion of lower limbs (the primary startle reflex system), and more purposeful and complicated actions which occasionally arose from these movements (the secondary startle reflex system). On the basis of this supposition, we considered simple tics which are usually called "tics", those are the manifestation of astonishment by the primary startle reflex system, and complex tics; echo phenomena, coprolalia, complex movements, and so on, which are said to be pathognomonic of Tourette's syndrome, are the expression of amazement by the secondary startle reflex system. Furthermore, we formulated a hypothesis that in the latter case, the secondary startle reflex system contains the senses of orientation and defense, and the defense mechanism which is innately imprinted in common throughout human race, and covered through individual development, is released and fixed in the form of complex movements or echo phenomena in an emergency, such as astonishment. To discuss further, we determined Latah reaction minutely (especially in "Imu" of the Ainu race in Japan) in which intricate symptoms had been suggested as one of the senses of defense, were closely related to Tourette's syndrome. For more concrete study, we presented two severe cases of Tourette's syndrome. Considering the circumstances mentioned above, pathogenesis and symptoms of Tourette's syndrome could be explained by the startle reflex theory. Finally, we analyzed several psychiatrical syndromes which were provoked by astonishment, we concluded that it was necessary to emphasize Tourette's syndrome was only a part of those more comprehensive syndromes which could be called "startle syndromes".

[One possible classification of the delusional misidentification syndromes and its developmental, regressive understandings].

In recent years, misidentification syndromes have attracted much attention, and various types of these syndromes have been recognized. However, misidentification syndromes tend to be discussed as case reports or as a whole, and yet there is no established classification. This is primarily due to a small number of cases encountered by each researcher. We propose a new classification of misidentification syndrome based on more than 70 patients with these syndromes, who have been treated at one psychiatric hospital during 20 years. First, delusional misidentification syndromes were classified into two types: misidentification of human relationships represented by descent delusion (relational misidentification), and misidentification of identity itself represented by Capgras symptom and Fregoli symptom (identical misidentification). Next, identical misidentification was subclassified into 3 types: division of identity (divided type), union of identity (unionized type), and transformation of identity (transformed type). Various misidentification syndromes and associated signs and symptoms were evaluated in detail from the aspect of descriptive psychiatry and were compared with our new classification. In addition, this typological study on misidentification was analyzed based on the regressive theory and Jacksonism, and we proposed a hypothesis that there is correspondence between misidentification syndromes and the development of person identification in infants.

[The present states and considerations of orthognathic surgery in Japan. Questionnaire in department of orthodontics of university dental hospital, and department of oral surgery of university medical hospital].

To investigate the present state of orthognathic surgery, questionnaires were sent to 30 departments of orthodontics of university dental hospital and 60 departments of oral surgery of university medical hospital. 1. Orthognathic surgery was carried out in 100% of the departments of orthodontics and 91.8% of the departments of oral surgery examined. 2. There was a difference in the number of patients between individual hospitals. 3. Analyses of cephalogram were used in 96% of the departments of orthodontics and 93% of the departments of oral surgery. 4. Pre- and post-surgical orthodontic treatments were carried out in all of the departments of orthodontics. 5. Orthodontic appliances (83.9%) were used in the departments of orthodontics for intermaxillary fixation, while orthodontic appliances (47.8%) and wire splints (49.2%) were used in the departments of oral surgery. 6. It is pointed out that the criterion of orthognathic surgery, treatment objectives on soft and hard tissue, selection of surgical method, adaptability of soft tissue after surgery, relapse, treatment planning of two jaw surgery, and postoperative stability remain to be solved in future.