Unusual Large Sporadic Angiomyolipoma Co-existing with Huge Simple Renal Cyst

Renal Angiomyolipoma (AML) is an unusual benign mesenchymal tumor with no malignant potential. It is composed of adipose tissue, smooth muscle and abnormal thick walled blood vessels. It can occur sporadically or may be associated with tuberous sclerosis. Sporadic angiomyolipoma (AML) coexisting with simple renal cyst is extremely rare and only one case report is available in the literature. In our case, unique combination of sporadic AML along with simple renal cyst with huge size and weight was noted. To the best of our knowledge, ours is the second such case and first case from India. Due to its large size, complete nephrectomy was performed to avoid chances of rupture and retroperitoneal hemorrhage. Post-operative period was uneventful and the patient ahs been on regular follow-up

Bioenergetic status modulates motor neuron vulnerability and pathogenesis in a zebrafish model of spinal muscular atrophy

Degeneration and loss of lower motor neurons is the major pathological hallmark of spinal muscular atrophy (SMA), resulting from low levels of ubiquitously-expressed survival motor neuron (SMN) protein. One remarkable, yet unresolved, feature of SMA is that not all motor neurons are equally affected, with some populations displaying a robust resistance to the disease. Here, we demonstrate that selective vulnerability of distinct motor neuron pools arises from fundamental modifications to their basal molecular profiles. Comparative gene expression profiling of motor neurons innervating the extensor digitorum longus (disease-resistant), gastrocnemius (intermediate vulnerability), and tibialis anterior (vulnerable) muscles in mice revealed that disease susceptibility correlates strongly with a modified bioenergetic profile. Targeting of identified bioenergetic pathways by enhancing mitochondrial biogenesis rescued motor axon defects in SMA zebrafish. Moreover, targeting of a single bioenergetic protein, phosphoglycerate kinase 1 (Pgk1), was found to modulate motor neuron vulnerability in vivo. Knockdown of pgk1 alone was sufficient to partially mimic the SMA phenotype in wild-type zebrafish. Conversely, Pgk1 overexpression, or treatment with terazosin (an FDA-approved small molecule that binds and activates Pgk1), rescued motor axon phenotypes in SMA zebrafish. We conclude that global bioenergetics pathways can be therapeutically manipulated to ameliorate SMA motor neuron phenotypes in vivo

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Not AvailableA seed rearing experiment was attempted to evaluate the effects of stocking densities in the biofilm-based system reared with pearlspot, Etroplus suratensis on different aquaculture parameters. Pearlspot advanced fry of size (4.67 ± 0.04 cm/1.71 ± 0.03 g) stocked at four different stocking densities viz 50 fish (T1); 100 fish (T2); 150 fish (T3) and 200 fish (T4) m−3 in the biofilm-based rearing system and at the rate of 150 fish m−3 in the clear water fed (CWF) system tanks. The total ammonia nitrogen and dissolved oxygen levels in biofilm tanks with different stocking density were significantly lower than CWF. Diatom was found as dominant plankton in all biofilm treatment tanks in water as well as on substrate. The growth parameters were found to significantly decrease with increasing stocking density in the biofilm-based rearing system. The significant highest individual growth parameters and significantly lowest fish biomass were found in T1. Final fish biomass was found to increase with increasing stocking density up to 150 advanced fry m−3 and then reduced due to decreased survival. Body composition analysis of E. suratensis showed increased protein and decreased lipid levels in all biofilm-based rearing system treatments at different stocking densities compared with clear water fed system. The present investigation findings on plankton abundance, growth, body composition and blood parameters at different stocking densities will provide guidelines for selecting appropriate stocking density for pearlspot advanced fry to fingerling rearing in a low saline biofilm-based system without providing a supplementary dietNot Availabl