Hematopoietic stem cells (HSCs) are regarded as one of essential cell sources for treating regenerative diseases. Among many stem cells, the feasibility of using adult-derived hematopoietic stem cells in therapeutic approaches is very diverse, and is unarguably regarded as an important cell source in stem cell biology. So far, many investigators are exploring HSCs and modified HSCs for use in clinical and basic science. In the present review, we briefly summarized HSCs and their application in pathophysiologic conditions, including non-hematopoietic tissue regeneration as well as blood disorders. HSCs and HSCs-derived progenitors are promising cell sources in regenerative medicine and their contributions can be properly applied to treat pathophysiologic conditions. Among many adult stem cells, HSCs are a powerful tool to treat patients with diseases such as hematologic malignancies and liver disease. Since HSCs can be differentiated into diverse progenitors including endothelial progenitors, they may be useful for constructing strategies for effective therapy.

Clinical presentation of supplementary motor area (SMA) syndrome includes complete akinesia of the contralateral side of the body and mutism, with secondary recovery of neurologic deficit. Multi-joint coordination is frequently impaired following the development of a brain lesion and is generally restricted by abnormal patterns of muscle activation within the hemiparetic limb, clinically termed muscle synergies. However, no work to date has confirmed this observation with the aid of objective methods, such as gait analysis, and the development of reflex pattern has not been suggested as a possible cause. We describe two unusual cases of flexor synergy after tumor resection of SMA lesions.
Brain ; Brain Neoplasms ; Extremities ; Gait ; Lower Extremity* ; Motor Cortex ; Muscles ; Mutism ; Neurologic Manifestations ; Reflex

BACKGROUND: The renin angiotensin syaimstem plays an important role in hypertension. Therefore, the purpose of this study was to investigate the comparison of responsiveness to angiotensin II (ANG II) in isolated renal proximal convoluted tubules of spontaneously hypertensive (SHR) and Wistar Kyoto (WKY) rats. METHODS: Intracellualr calcium concentration ([Ca2+i) was measured using Fura- 2/AM, inositol trisphosphate (IP3) accumulation was determined by radioimmuno assay and cellular ATP content measured using the microchemilunescene method in renal proximal tubule suspension or isolated renal proximal tubules. RESULTS: When measured the ANG II-induced [Ca2+i, the young rats showed a greater response to ANG II than adult rats in both strains. The ANG II (10-7 M)-induced [Ca2+i transient in the cortical tubule suspension from adult SHR was significantly lower than that in age-matched WKY. In isolated proximal tubule segments, ANG II-induced [Ca2+i increment was only observed in S1 segments. Comparing responsiveness to ANG II in SHR and WKY, similar phenomenon was observed as experiment using tubule suspension. IP3 accumulation by ANG II also attenuated in adult SHR. The 20-minutes incubation without any exogenous substrate in proximal convoluted tubule (S1) significantly decreased cellular ATP content and ANG II (10-7 M) inhibited decrement of cellular ATP level. The effect of ANG II on cellular ATP restoration was disappeared by the treatment with losartan. CONCLUSION: From these results, the responsiveness of ANG II to AT1A receptor is attenuated in the proximal convoluted tubules of adult SHR comparing the age- mached WKY.
Adenosine Triphosphate ; Adult* ; Angiotensin II ; Angiotensins ; Animals ; Calcium ; Humans ; Hypertension ; Inositol ; Losartan ; Rats ; Renin

Septo-optic dysplasia, as first described by de Morsier, is a rare developmental anomaly of absent or hypoplastic septum pellucidum, a primitive optic vesicle, and dysplasia of the optic nerve, chiasm, and optic tracts. It is a common cause of hypopituitarism in children and has variable hypothalamic-pituitary dysfunction. We experienced a case of septo-optic dysplasia in 5 month old female baby who presented with developmental delay and seizure. A magnetic resonance imaging of the brain showed absence of the septum pellucidum and the fundoscopic examination disclosed the absence of left optic disc and atrophy of right optic disc.
Atrophy ; Brain ; Child ; Female ; Humans ; Hypopituitarism ; Infant ; Magnetic Resonance Imaging ; Optic Nerve ; Seizures ; Septo-Optic Dysplasia* ; Septum Pellucidum ; Visual Pathways

No abstract available.
Lung*

OBJECTIVE: To evaluate the accuracy of combined transvaginal ultrasound and beta-hCG discriminatory zone for diagnosing intrauterine pregnancy, abortion, and ectopic pregnancy in early abnormal pregnancy. METHODS: Initial ultrasound findings and beta-hCG level were compared with final pregnancy outcome in 164 early pregnant women who visit our hospital with vaginal bleeding or abdominal pain. The sensitivity, specificity, and predictive value by the combination of two diagnostic tools were calculated. Statistic analysis of collected data used x2 of SPSS(9.0). RESULTS: Of 90 women with normal outcome, 64(71.1%) had a gestational sac 5mm, and in 47 cases, the hCG level was above 1,800 mIU/ml. Transvaginal ultrasound was non-diagnostic in 47(28.6%) of 164 women, and especially, 43(53.1%) of 81 cases with beta-hCG levels below 1,800 mIU/ml. The portion of accurate ultrasound diagnosis was significantly higher in women above 1,800 mIU/ml (85.5%, 71 of 83 cases) compard with levels below 1,800 mIU/ml (37.0%, 30 of 81 cases) : P < 0.001; Relative Risk(RR) 2.31; CI 95%. Sensitivity of transvaginal ultrasound diagnosis of intrauterine pregnancy, abortion, and ectopic pregnancy was 90.2%, 79.3%, and 66.7% in women who presented with beta-hCG levels above 1,800 mIU/ml, and 41.0%, 23.5%, and 75% below 1,800 mIU/ml, respectively. And, negative predictive value was 83.9%, 89.7%, and 98.8% in each of intrauterine pregnancy, abortion, and ectopic pregnancy above 1,800 mIU/ml, and 64.6%, 60%, and 97.3% below 1,800 mIU/ml, respectively. CONCLUSIONS: The sensitivity, specificity, and predictive value of transvaginal ultrasound for diagnosing abnormal early pregnancy were poor except cases of ectopic pregnancy when beta-hCG levels were low than discriminatory zone. Ultrasound impressions were well related with beta-hCG levels.
Abdominal Pain ; Diagnosis ; Female ; Gestational Sac ; Humans ; Pregnancy Outcome ; Pregnancy* ; Pregnancy, Ectopic ; Pregnant Women ; Sensitivity and Specificity ; Ultrasonography* ; Uterine Hemorrhage

Paget's disease of bone (osteitis deformans), a chronic osseous disease of unknown etiology, is extremely rarein Asia although well known in western countries. This disease is characterized by the simultaneous absorption ofbone and the new bone formation of abnormal character affecting middle and later age groups. The authorsexperienced a case of monostotic Paget's disease involving left pelvic bone in 56-years old female. To ourknowledge, this is the first cas report of monostotic Paget's in Korea.
Absorption ; Asia ; Female ; Humans ; Korea ; Osteitis Deformans ; Osteogenesis ; Pelvic Bones

To investigate the effect of endothelin on intracellular free calcium ([Ca(2+)]i) mobilization and the existence of the endothelin receptor in cultured bovine corneal endothelial cells(BCEC), endothelin-1(ET-1) -induced [Ca(2+)]i increase was measuredby using calcium sensitive indicator, fura-2/AM, and the studies on its desensitizaton and receptor antagonists were also performed. ET-1 increased [Ca(2+)]i in a concentration-dependent manner(10(-9)M-10(-5)M) and ET-1 -unduced [Ca(2+)]i transient increase was significantly ingibited (~50%) by the pretreatment with EGTA for 1 min. Similarly, neomycin also attenuated ET-1 -unduced [Ca(2+)]i transient increase in a concentration-dependent mannet. Desensitizatin study with successive treatment of ET-1 and ET-3, and the experiments of antagonists(BQ-610 for the ET(A) receptor and BQ-788 for the ET(B) receptor) showed the presence of ET(B) receptor in BCEC. In addition, ET-1(10(-6)M) accumulated inositol trisphosphate about two folds (310+/-6.8 cpm) comparing to control(154+/-11.6 cpm) and this accumulation was significantly inhibited by the treatment with neomycin (187+/-28 cpm). These results suggest that endothelin-induced calcium mobilization is receptor-mediated response and TE(B) receptor exists in BCEC.
Calcium* ; Egtazic Acid ; Endothelins ; Endothelium, Corneal* ; Inositol ; Neomycin ; Receptors, Endothelin

BACKGROUND: Ichthyosis is the name of a group of disorders characterized by a generalized, persistent, non-inflammatory scaling disorder of the skin. Filaggrin is an intermediate filament associated protein which functions to aggregate keratin intermediate filaments in the stratum corneum of mamrnalian epidermis. It is synthesized as a large precursor protein, profilaggrin, that consists of multiple filaggrin units and is localized in keratohyalin granules(KHG). Some reports elucidated the pathogenetic role of filaggrin in ichthyosis, especially autosomal-dominant ichthyosis(ADI). OBJECTIVE: We conducted an immunohistochemical study in order to observe the relationship between clinical phenotypes of various ichthyosiform dermatoses and the exprassion of filaggrin. MATERIALS AND METHODS: Clinically and histopathologically proven ADI, lamellar exfoliation of the newborn, lamellar ichthyosis, epidermolytic hyperkeratosis, and acquired ichthyosis cases were included in this study. Using monoclonal mouse anti-human filaggrin antibodies, an immunohisto-chemical study was performed with formalin-fixed, parraffin-embedded involved skin tissues via the ABC technique. RESULTS: Filaggrin was staind linearly or in a punctuate pattern along the granular cell layer of the normal epidermis. The expression of the filaggrin was decreased or partly absent in the skin tissue of ADI in accordance with the changes of KHGs. Similar immunoreactivities with filaggrin antibodies were noted in the skin of acquired ichthyosis. In lamellar exfoliation of the newborn, filaggrin was staineil from the stratum granulosum to the mid-layer of the stratum corneum with basket-weave byperkeratosis. Filaggrin was intensely and focally labelled in the normal acrosyringium of the lamellar ichthyosis from the granular layer to the mid horney layer. A decreased positive immunorcactivity was observed in the interfollicular stratum granulosum. A strong immunoreactivity was noted in the area with relatively normal KHGs of epidermolytic hyperker atosis, and a decreased l membranous staning pattern was observed in the layer with degenerated vacuolar KHGs. CONCLUSION: It can be concluded that decreased expression of filaggin may play a causative role in the pathogenesis of ADI. However in lamellar exfoliation of the newborn, lamellar ichthyosis, epidermolytic hyperkeratosis, and acquired ichhtyosis, the altered expression of filaggrin must reflect the changes of KHGs in the stratum granulosum.
Animals ; Antibodies ; Epidermis ; Humans ; Hyperkeratosis, Epidermolytic ; Ichthyosis ; Ichthyosis, Lamellar ; Immunohistochemistry ; Infant, Newborn ; Intermediate Filaments ; Mice ; Phenotype ; Skin ; Skin Diseases*

The human organic anion transporter 4 (hOAT4) has been identified as the fourth isoform of OAT family. hOAT4 contributes to move several negatively charged organic compounds between cells and their extracellular milieu. The functional characteristics and regulatory mechanisms of hOAT4 remain to be elucidated. It is well known that caveolin plays a role in modulating proteins having some biological functions. To address this issue, we investigated the co-localization and interaction between hOAT4 and caveolin-1. hOAT4 and caveolin-1 (mRNA and protein expression) were observed in cultured human placental trophoblasts isolated from placenta. The confocal microscopy of immuno-cytochemistry using primary cultured human trophoblasts showed hOAT4 and caveolin-1 were co-localized at the plasma membrane of the cell. This finding was confirmed by Western blot analysis using isolated caveolae-enriched membrane fractions and immune-precipitates from the trophoblasts. When synthesized cRNA of hOAT4 along with scrambled- or antisense-oligodeoxynucleotide (ODN) of Xenopus caveolin-1 were co-injected to Xenopus oocytes, the [3H]estrone sulfate uptake was significantly decreased by the co-injection of antisense ODN but not by scrambled ODN. These findings suggest that hOAT4 and caveolin-1 share a cellular expression in the plasma membrane and caveolin-1 up-regulates the organic anionic compound uptake by hOAT4 under the normal physiological condition.
Animals ; Caveolin 1/genetics/*metabolism ; Cells, Cultured ; Female ; Humans ; Immunohistochemistry ; Immunoprecipitation ; Microscopy, Confocal ; Models, Biological ; Oocytes/metabolism ; Organic Anion Transporters/genetics/*metabolism ; Placenta/cytology ; Protein Binding ; Reverse Transcriptase Polymerase Chain Reaction ; Trophoblasts/cytology/*metabolism ; Xenopus