Botulinum Toxin A Ameliorates Neuroinflammation in the MPTP and 6-OHDA-Induced Parkinson’s Disease Models

Recently, increasing evidence suggests that neuroinflammation may be a critical factor in the development of Parkinson’s disease (PD) in addition to the ratio of acetylcholine/dopamine because dopaminergic neurons are particularly vulnerable to inflammatory attack. In this study, we investigated whether botulinum neurotoxin A (BoNT-A) was effective for the treatment of PD through its anti-neuroinflammatory effects and the modulation of acetylcholine and dopamine release. We found that BoNT-A ameliorated MPTP and 6-OHDA-induced PD progression, reduced acetylcholine release, levels of IL-1β, IL-6 and TNF-α as well as GFAP expression, but enhanced dopamine release and tyrosine hydroxylase expression. These results indicated that BoNT-A had beneficial effects on MPTP or 6-OHDA-induced PD-like behavior impairments via its anti-neuroinflammation properties, recovering dopamine, and reducing acetylcholine release.

Effects of Carnitine on Peripheral Blood Mitochondrial DNA Copy Number and Liver Function in Non-Alcoholic Fatty Liver Disease / 대한소화기학회지

BACKGROUND/AIMS: Functional and anatomical abnormalities of mitochondria play an important role in developing steatohepatitis. Carnitine is essential for enhanced mitochondrial beta oxidation through the transfer of long-chain fatty acids into the mitochondria. We examined the impact of carnitine complex on liver function and peripheral blood mitochondria copy number in NAFLD patients. METHODS: Forty-five NAFLD patients were enrolled. Patients were categorized into the carnitine complex-administered group and control group. Before and 3 months after drug administration, a liver function test and peripheral blood mitochondrial DNA and 8-oxo-dG quantitive analysis were conducted. RESULTS: In carnitine treatment group, ALT, AST, and total bilirubin were reduced after medication. There was no difference in AST, ALT, and total bilirubin between before and after treatment in control group. In carnitine group, peripheral mitochondrial DNA copy number was significantly increased from 158.8+/-69.5 copy to 241.6+/-180.6 copy (p=0.025). While in control group the mitochondrial copy number was slightly reduced from 205.5+/-142.3 to 150.0+/-109.7. 8-oxo-dG level was also tended to decrease in carnitine group (p=0.23) and tended to increase in control group (p=0.07). CONCLUSIONS: In NAFLD, the carnitine improved liver profile and peripheral blood mitochondrial DNA copy number. This results suggest that carnitine activate the mitochondria, thereby contributing to the improvement of NAFLD.

A Case of Promyelocytic Leukemia with Basophil-like Granules / 대한혈액학회지

According to the FAB (French-American-British) classification, two main cytological subtypes are recognized: the classical hypergranular and the microgranular variant. However, other morphological variants have been reported in the literature. Therefore, careful examination is needed to diagnose acute promyelocytic leukemia (APL). Herein, the case of an APL variant, with basophil-like granules, occurring in a 65 year-old woman admitted due to high fever is reported. The peripheral blood showed blasts and increased basophils. A bone marrow smear showed leukemic blasts, with irregular nuclei and coarse basophil-like granules, in the cytoplasm. Cytoplasmic vacuoles were also noted, but no Auer rod was found. The blasts showed positivity toward MPO, tryptase and toluidine blue staining. The immunophenotypes revealed a myeloid lineage, with aberrant expression of CD2 and CD7. The karyotype was t(15;17)(q22;q12) in 17 out of 20 metaphases. The RT-PCR was positive for long form PML/RARalpha transcripts. Four weeks after chemotherapy, her bone marrow status findings were in complete remission, with the karyotype converted to normal. Basophil-like granules in the blasts and promyelocytes became decreased during the course of all-transretinoic acid (ATRA) treatment. The chimeric transcript of PML/RARalpha converted to negative after consolidation chemotherapy.

A Case of Hereditary Sideroblastic Anemia / 대한혈액학회지

We experienced a case of pyridoxine refractory hereditary sideroblastic anemia (HSA) in a 4 year-old girl and; therefore, conducted a study of her family. She was admitted to hospital for anemia, which was uncorrected by iron treatment. The peripheral blood smears showed hypochromic microcytic anemia. The results of the biochemical study indicated serum iron of 80 microgram/dL, TIBC of 275 microgram/dL and serum ferritin of 67ng/dL. The bone marrow smears showed 80% cellularity, with mild dyserythropoiesis. Many ringed sideroblasts, 45% of normoblasts and an increased amount of hemosiderin particles were observed with iron staining. Despite high-dose pyridoxine therapy, the anemia was not corrected. In the peripheral blood and iron studies conducted on her family members, the mother, maternal aunt and aunt's son showed microcytic hypochromic anemia and normal iron metabolism. Her mother's brother had died of acute myeloid leukemia that had transformed from myelodysplastic syndrome. From a search of the Korean literature, this is the first reported case of HSA with pedigree.