Enzyme studies in GM2 gangliosidiosis,and their application in prenatal diagnosis

Assay of hexosaminidase A and B enzymes in four cases with developmental regression and cherry red spot on fundus examination confirmed that three cases had Tay-Sachs disease, and one case had Sandhoff disease. Prenatal diagnosis was carried out by hexosaminidase enzyme assay in amniotic fluid and cells in one family, and chorionic villus sample in the second family. The fetus was diagnosed to be unaffected in one, and affected in the other family. Assay of hexosaminidase A and B is useful for specific diagnosis of GM₂ gangliosidosis, and for prenatal diagnosis to reduce the burden of these disorders.     

Rabbit as an animal model for experimental research

Animal experimentation is carried out in consultation with the veterinary wing but it is essential that be familiar with experimental protocols of animal model to be able to design an approriate study. This is more so in place where the veterinary facilities are not easily available.Span Rabbits are commonly used as subjects for screening implant material. They have gained favour for their numerous advantages even though they should be ideally used prior to testing in a larger animal model. Though experimentation on rabbits seems to be easy there are many pitfalls. Our endeavor in this article is to integrate all the data about maintaining rabbits as a model and to critically analyze it on the basis of our experimentation.     

PELP1 signaling contributes to medulloblastoma progression by regulating the NF-κB pathway

Medulloblastoma (MB) is the most common and deadliest brain tumor in children. Proline-, glutamic acid-, and leucine-rich protein 1 (PELP1) is a scaffolding protein and its oncogenic signaling is implicated in the progression of several cancers. However, the role of PELP1 in the progression of MB remains unknown. The objective of this study is to examine the role of PELP1 in the progression of MB. Immunohistochemical analysis of MB tissue microarrays revealed that PELP1 is overexpressed in the MB specimens compared to normal brain. Knockdown of PELP1 reduced cell proliferation, cell survival, and cell invasion of MB cell lines. The RNA-sequencing analysis revealed that PELP1 knockdown significantly downregulated the pathways related to inflammation and extracellular matrix. Gene set enrichment analysis confirmed that the PELP1-regulated genes were negatively correlated with nuclear factor-κB (NF-κB), extracellular matrix, and angiogenesis gene sets. Interestingly, PELP1 knockdown reduced the expression of NF-κB target genes, NF-κB reporter activity, and inhibited the nuclear translocation of p65. Importantly, the knockdown of PELP1 significantly reduced in vivo MB progression in orthotopic models and improved the overall mice survival. Collectively, these results suggest that PELP1 could be a novel target for therapeutic intervention in MB.     

Gynecologic brachytherapy-II: Intravaginal brachytherapy for carcinoma of the endometrium

Brachytherapy plays a significant role in the management of endometrial cancer. In the adjuvant setting, based on pathologic risk factors, intravaginal brachytherapy alone, external radiation therapy alone, or a combination of the two is recommended. For patients who are medically inoperable, brachytherapy with or without external beam therapy is the mainstay of treatment. In recurrent disease, to achieve improved local regional control interstitial and/or intravaginal brachytherapy is used as a boost. This article will highlight the indications and technical aspects of postoperative intravaginal brachytherapy, which is the most common application of brachytherapy in endometrial cancer.     

Amniotic fluid derived stem cells ameliorate focal cerebral ischaemia-reperfusion injury induced behavioural deficits in mice

The present study has been designed to investigate the effect of amniotic fluid derived stem cells on focal cerebral ischaemia-reperfusion injury induced behavioural deficits in mice. Middle cerebral artery occlusion of 60 min followed by reperfusion for 7 days was employed in present study to produce ischaemia and reperfusion induced cerebral injury in mice. Assessment of cognitive behaviour was done using elevated plus maze. Assessment of neurological severity score was employed to assess motor, sensory, reflex, and balance tests in a composite manner. Adhesive-removal somatosensory test was employed to evaluate somatosensory deficit. Partial occlusion of middle cerebral artery markedly impaired memory, motor coordination, sensorimotor ability and somatosensory functions as inferred from the results of elevated plus-maze test, adhesive-removal test and neurological severity score test. Intracerebroventricular administration of amniotic fluid derived stem cells/embryonic neuronal stem cells significantly reversed the focal cerebral ischaemia-reperfusion induced behavioural deficit measured in terms of loss of short-term memory, motor coordination, sensorimotor ability and somatosensory functions. Therefore, it may be concluded that stem cells derived from amniotic fluid exert beneficial effect on the ischaemic brain to an extent comparable to the neuroprotective effect of embryonic neuronal stem cells.     

Neuroprotective effect of Momordica charantia in global cerebral ischemia and reperfusion induced neuronal damage in diabetic mice

Ethnopharmacological relevance

Momordica charantia L. (Cucurbitaceae) fruits have been used traditionally for centuries, especially for treating diabetes and associated complications.

Aim of the study

The present study was performed to evaluate neuroprotective effect of lyophilized M. charantia fruit juice against global cerebral ischemia and reperfusion induced neuronal injury in diabetic mice.

Materials and methods

Global cerebral ischemia induced by occluding both common carotid arteries for 10 min followed by 24 h reperfusion was used to induce neuronal injury. Ischemia-reperfusion induced neuronal injury was evaluated in terms of cerebral infarct size, generation of free radicals measured as thiobarbaturic acid reactive substances (TBARS), and neurological functions measured as short term memory and motor activity.

Results

The cerebral oxidative stress and damage, and neurological deficits were dose dependently attenuated by pre-treatment with the lyophilized M. charantia juice (200-800 mg/kg, p.o., o.d.). Moreover, M. charantia also exhibited dose dependent antihyperglycemic activity in diabetic mice.

Conclusions

These results suggest that M. charantia has potent neuroprotective activity against global cerebral ischemia-reperfusion induced neuronal injury and consequent neurological deficits in diabetic mice.