D-galactonate metabolism in enteric bacteria: a molecular and physiological perspective.

D-galactonate, a widely prevalent sugar acid, was first reported as a nutrient source for enteric bacteria in the 1970s. Since then, decades of research enabled a description of the modified Entner-Doudoroff pathway involved in its degradation and reported the structural and biochemical features of its metabolic enzymes, primarily in Escherichia coli K-12. However, only in the last few years, the D-galactonate transporter has been characterized, and the regulation of the dgo operon, encoding the structural genes for the transporter and enzymes of D-galactonate metabolism, has been detailed.

Working during the COVID-19 pandemic: Understanding employee mental health.

Background: The coronavirus disease 2019 (COVID-19) pandemic has radically transformed workplaces, bearing an adverse impact on the mental health of employees.

Aim: The current study attempts to gain an understanding of the mental health of employees while working from home (WFH) during the COVID-19 pandemic.

Setting And Design: The research followed a mixed-methods design and was conducted across two phases, with participants divided into two subgroups - the WFH subgroup (currently engaging in WFH) and the not working from home (NWFH) subgroup (unable to engage in vocational tasks due to the pandemic).

Identification of potential inhibitors of brain-specific CYP46A1 from phytoconstituents in Indian traditional medicinal plants.

Unlabelled: Cytochrome P450 46A1 (CYP46A1) is a crucial enzyme in brain that converts cholesterol to 24 (S) hydroxy cholesterol thereby increasing its polarity to facilitate removal of excess cholesterol from the CNS. The inhibition of CYP46A1 with several synthetic molecules has been investigated extensively for treatment of Alzheimer's disease, Huntington's disease, glaucoma, and in hippocampal neurons from aged mice. However, phytochemicals have received far little attention in studies involving development of potential CYP46A1 inhibitors.

Intracerebroventricular dosing of N-sulfoglucosamine sulfohydrolase in mucopolysaccharidosis IIIA mice reduces markers of brain lysosomal dysfunction.

Mucopolysaccharidosis type IIIA (MPS IIIA) is a lysosomal storage disorder caused by N-sulfoglucosamine sulfohydrolase (SGSH) deficiency. SGSH removes the sulfate from N-sulfoglucosamine residues on the nonreducing end of heparan sulfate (HS-NRE) within lysosomes. Enzyme deficiency results in accumulation of partially degraded HS within lysosomes throughout the body, leading to a progressive severe neurological disease.

Management and surgical outcomes of pediatric retinal detachment associated with familial exudative vitreoretinopathy - Our experience at a tertiary care ophthalmic center in North India.

Purpose: To report the clinical profile, management, and long-term anatomical and visual acuity (VA) outcomes of pediatric macula-off rhegmatogenous retinal detachment (RRD) secondary to familial exudative vitreoretinopathy (FEVR).

Methods: This was a prospective, interventional study of 14 eyes of 13 children aged ≤18 years with macula-off FEVR-RRD. The primary outcomes were anatomical reattachment and VA changes.

Application of --cultured primary hepatocytes to evaluate species translatability and AAV transduction mechanisms of action.

Recombinant adeno-associated virus (AAV) is an effective platform for therapeutic gene transfer; however, tissue-tropism differences between species are a challenge for successful translation of preclinical results to humans. We evaluated the use of primary hepatocyte cultures to predict liver-directed AAV expression in different species. We assessed whether AAV transduction assays in cultured primary hepatocytes from mice, nonhuman primates (NHPs), and humans could model liver-directed AAV expression of valoctocogene roxaparvovec (AAV5-hFVIII-SQ), an experimental gene therapy for hemophilia A with a hepatocyte-selective promoter.

Multilayered inverted internal limiting membrane flap technique versus standard internal limiting membrane peeling for large macular holes: A comparative study.

Purpose: To evaluate the outcome of vitrectomy with multilayered inverted internal limiting membrane flap technique (ML-IILM) versus vitrectomy with standard ILM peeling for large macular holes in terms of visual acuity and anatomical closure.

Methods: A hospital-based, prospective, randomized, interventional study was conducted during three calendar years with a total 150 eyes (75 in each group) in two groups-vitrectomy with ILM peeling (Group A) and vitrectomy with ML-IILM flap technique (Group B) after informed consent of study participants who met the inclusion criteria.

Results: The mean minimum and maximum diameter of macular hole did not differ statistically in both the groups.

Concomitant pulmonary valve replacement with intracardiac repair for adult tetralogy of Fallot.

Objectives: Adult patients undergoing tetralogy of Fallot (TOF) repair have a higher risk of mortality compared to pediatric patients. Pulmonary regurgitation (PR) further predisposes these patients to heart failure, arrhythmias, and sudden death. Pulmonary valve replacement (PVR) may improve the symptoms in these patients but, fails to reverse the other deleterious effects.

Molecular insights into effector binding by DgoR, a GntR/FadR family transcriptional repressor of D-galactonate metabolism in Escherichia coli.

Several GntR/FadR transcriptional regulators govern sugar acid metabolism in bacteria. Although effectors have been identified for a few sugar acid regulators, the mode of effector binding is unknown. Even in the overall FadR subfamily, there are limited details on effector-regulator interactions.

A rare case of total anomalous pulmonary venous connection with intact interatrial septum.

The presence of interatrial communication is considered obligatory in total anomalous pulmonary venous connection (TAPVC). Even a restriction in this communication leads to obstructive TAPVC. We report a rare case of obstructed supracardiac TAPVC with the absence of interatrial communication and with multiple ventricle septal defects (VSDs) in a 3-month-old child.

Intermittent enzyme replacement therapy with recombinant human β-galactosidase prevents neuraminidase 1 deficiency.

Mutations in the β () gene cause lysosomal β-galactosidase (β-Gal) deficiency and clinical onset of the neurodegenerative lysosomal storage disease, GM1 gangliosidosis. β-Gal and neuraminidase 1 (NEU1) form a multienzyme complex in lysosomes along with the molecular chaperone, protective protein cathepsin A (PPCA). NEU1 is deficient in the neurodegenerative lysosomal storage disease sialidosis, and its targeting to and stability in lysosomes strictly depend on PPCA.

Truncus Arteriosus With Double Aortic Arch.

Truncus arteriosus (TA) or common arterial trunk is a congenital cardiac anomaly having high association with arch anomalies such as right aortic arch or aortic arch interruption. However, TA with double aortic arch (DAA) is a rare occurrence. We report a case of TA with DAA where the diagnosis of DAA was missed initially.

Type II Aortopulmonary Window with Isolated Left Subclavian Artery from Left Pulmonary Artery.

Type II Aortopulmonary window (APW) accounts for only 10% of total cases of APW, which by itself is a rare congenital anomaly. Various cardiac malformations have been reported to be associated with this rare anomaly. We report one such association of origin of left subclavian artery (LSCA) from left pulmonary artery (LPA) via ductus arteriosus that was surgically repaired.

Does addition of bi-directional cavo-pulmonary shunt to tricuspid repair in advanced cases of Ebstein anomaly result in better outcomes?

Objectives: Ebstein anomaly can be managed by single ventricular, bi-ventricular and one and a half ventricular repairs. We present midterm results of Comprehensive Tricuspid Valve repair (CTVR) with bi-directional cavo-pulmonary shunt (BCPS).

Methods: In this prospective observational study (Jan2012-July2018), 69 patients underwent surgery for Ebstein anomaly.

Inhibition of endogenous miR-23a/miR-377 in CHO cells enhances difficult-to-express recombinant lysosomal sulfatase activity.

Difficult-to-express (DTE) recombinant proteins such as multi-specific proteins, DTE monoclonal antibodies, and lysosomal enzymes have seen difficulties in manufacturability using Chinese hamster ovary (CHO) cells or other mammalian cells as production platforms. CHO cells are preferably used for recombinant protein production for their ability to secrete human-like recombinant proteins with posttranslational modification, resistance to viral infection, and familiarity with drug regulators. However, despite huge progress made in engineering CHO cells for high volumetric productivity, DTE proteins like recombinant lysosomal sulfatase represent one of the poorly understood proteins.

Intracerebroventricular enzyme replacement therapy with β-galactosidase reverses brain pathologies due to GM1 gangliosidosis in mice.

Autosomal recessive mutations in the galactosidase β1 () gene cause lysosomal β-gal deficiency, resulting in accumulation of galactose-containing substrates and onset of the progressive and fatal neurodegenerative lysosomal storage disease, GM1 gangliosidosis. Here, an enzyme replacement therapy (ERT) approach in fibroblasts from GM1 gangliosidosis patients with recombinant human β-gal (rhβ-gal) produced in Chinese hamster ovary cells enabled direct and precise rhβ-gal delivery to acidified lysosomes. A single, low dose (3 nm) of rhβ-gal was sufficient for normalizing β-gal activity and mediating substrate clearance for several weeks.

Anomalous origin of a pulmonary artery in a teenager with tetralogy of Fallot with associated double aortic arch and coronary arteriovenous fistula.

We report the case of a 14-year-old female who had tetralogy of Fallot along with anomalous origin of the left pulmonary artery from the ascending aorta with co-dominant double aortic arch forming a complete vascular ring compressing the oesophagus along with a left main coronary artery to right ventricular outflow tract fistula. She underwent surgical correction without conduit placement.

Intramural Aortic Course Should Always Be Considered for Anomalous Origin of the Left Coronary Artery From the Right Pulmonary Artery.

Anomalous origin of the left coronary artery (LCA) from the right pulmonary artery (ALCARPA) is an extremely rare subset of an already rare entity, anomalous origin of the LCA from the pulmonary artery. Whenever it is diagnosed preoperatively, one should be extremely vigilant about the potential intramural course of the descending part of the LCA in the aorta. Preoperative imaging frequently fails to delineate this intramural course.

Ebstein's Anomaly: "The One and a Half Ventricle Heart".

Objective: Ebstein's anomaly remains a relatively ignored disease. Lying in the 'No Man's land' between congenital and valve surgeons, it largely remains inadequately studied. We report our short-term results of treating it as a 'one and a half ventricle heart' and propose that the true tricuspid annulus (TTA) 'Z' score be used as an objective criterion for estimation of 'functional' right ventricle (RV).

Mesenchymal Stem Cell Therapy in Ischemic Stroke: A Meta-analysis of Preclinical Studies.

Numerous preclinical studies have been carried out using mesenchymal stem cells (MSCs) therapy for ischemic stroke. The purpose of the present meta-analysis is to review the quality of preclinical studies. In all, 4,361 articles were identified, out of which 64 studies were included (excluding in vitro studies).

Btn2a2, a T cell immunomodulatory molecule coregulated with MHC class II genes.

Evidence has recently emerged that butyrophilins, which are members of the extended B7 family of co-stimulatory molecules, have diverse functions in the immune system. We found that the human and mouse genes encoding butyrophilin-2A2 (BTN2A2) are regulated by the class II trans-activator and regulatory factor X, two transcription factors dedicated to major histocompatibility complex class II expression, suggesting a role in T cell immunity. To address this, we generated Btn2a2-deficient mice.