The roles of glycosphingolipids in the proliferation and neural differentiation of mouse embryonic stem cells

Glycosphingolipids including gangliosides play important regulatory roles in cell proliferation and differentiation. UDP-glucose:ceramide glucosyltransferase (Ugcg) catalyze the initial step in glycosphingolipids biosynthesis pathway. In this study, Ugcg expression was reduced to approximately 80% by short hairpin RNAs (shRNAs) to evaluate the roles of glycosphingolipids in proliferation and neural differentiation of mouse embryonic stem cells (mESCs). HPTLC/immunofluorescence analyses of shRNA-transfected mESCs revealed that treatment with Ugcg-shRNA decreased expression of major gangliosides, GM3 and GD3. Furthermore, MTT and Western blot/immunofluorescence analyses demonstrated that inhibition of the Ugcg expression in mESCs resulted in decrease of cell proliferation (P < 0.05) and decrease of activation of the ERK1/2 (P < 0.05), respectively. To further investigate the role of glycosphingolipids in neural differentiation, the embryoid bodies formed from Ugcg-shRNA transfected mESCs were differentiated into neural cells by treatment with retinoic acid. We found that inhibition of Ugcg expression did not affect embryoid body (EB) differentiation, as judged by morphological comparison and expression of early neural precursor cell marker, nestin, in differentiated EBs. However, RT-PCR/immunofluorescence analyses showed that expression of microtubule- associated protein 2 (MAP-2) for neurons and glial fibrillary acidic protein (GFAP) for glial cells was decreased in neural cells differentiated from the shRNA-transfected mESCs. These results suggest that glycosphingolipids are involved in the proliferation of mESCs through ERK1/2 activation, and that glycosphingolipids play roles in differentiation of neural precursor cells derived from mESCs.

Diagnostic testing for Duchenne/Becker Muscular dystrophy using Dual Priming Oligonucleotide (DPO) system

PURPOSE: Large exon deletions in the DMD gene are found in about 60% of DMD/BMD patients. Multiplex PCR has been employed to detect the deletion mutation, which frequently generates noise PCR products due to the presence of multiple primers in a single reaction as well as the stringency of PCR conditions. This often leads to a false-negative or false-positive result. To address this problematic issue, we introduced the dual primer oligonucleotide (DPO) system. DPO contains two separate priming regions joined by a polydeoxyinosine linker that results in high PCR specificity even under suboptimal PCR conditions. METHODS: We tested 50 healthy male controls, 50 patients with deletion mutation as deletion-positive patient controls, and 20 patients with no deletions as deletion-negative patient controls using DPO- multiplex PCR. Both the presence and extent of deletion were verified by simplex PCR spanning the promoter region (PM) and 18 exons including exons 3, 4, 6, 8, 12, 13, 17, 19, 43-48, 50-52, and 60 in all 120 controls. RESULTS: DPO-multiplex PCR showed 100% sensitivity and specificity for the detection a deletion. However, it showed 97.1% sensitivity and 100% specificity for determining the extent of deletions. CONCLUSION: The DPO-multiplex PCR method is a useful molecular test to detect large deletions of DMD for the diagnosis of patients with DMD/BMD because it is easy to perform, fast, and cost-effective and has excellent sensitivity and specificity.

A Case of Treatment of Serum EB Virus Antibody Positive Patient Presented with Evans Syndrome / 대한혈액학회지

Evans syndrome is defined as the simultaneous or sequential occurrence of Coombs- positive hemolytic anemia and idiopathic thrombocytopenia. The clinical course is characterized by periods of remission and exacerbation with variable, and often disappointing responses to therapy. We experienced a case of serum Epstein-Barr virus antibody positive patient presented with Evans syndrome in a 31-year-old woman whose chief complaints were dyspnea and general weakness and whose disease responded to the multimodality therapy including prednisolone, plasmapheresis, intravenous immunoglobulin (IVIG), and alternate-day cyclosporine A and prednisolone. This is the encouraging report of the use of multimodality treatment with prednisolone, plasmapheresis, IVIG, and cyclosporine A and prednisolone in a serum EV virus antibody positive patient presented with Evans syndrome.

Acute Myeloid Leukemia with Trisomy 9 Following Chemotherapy of Non-Hodgkin's Lymphoma / 대한암학회지

A 56-year-old male patient with non-Hodgkin's lymphoma achieved complete remission in July 1994 after receiving MACOP-B chemotherapy. 29 months after treatment, acute myeloid leukemia (AML, FAB subtypes M4) with trisomy 9 was developed. To our knowledge this is the first report of therapy-related AML with trisomy 9.

Immediate reconstruction of thumb tip injury

No abstract available.

A Case of Intramuscular Lipoma in the Malar Area / 영남의대학술지

Lipomas are common soft tissue that are usually located in the subcutaneous tissue. And intramuscular lipomas commonly arise in the upper and lower extremities, where they usually involve the large muscles. Intramuscular lipoma, also referred to as an infiltrating lipoma, is an unusual benign slow growing tumor composed of mature fat cell interdigitating with skeletal muscle. However, intramuscular lipomas are exceedingly rare in the face. We have been experienced a case intramuscular lipoma that located in the malar area. Because of the rarity of these tumors and their propensity to recur without adequate surgery, the case report is presented here. Achievement of surgical margin is essential as the recurrent rate may be as high as 15% to 62.5% without complete excision.