A Novel Mouse Model for Cerebral Inflammatory Demyelination in X-Linked Adrenoleukodystrophy: Insights into Pathogenesis and Potential Therapeutic Targets.

Objective: X-linked adrenoleukodystrophy (ALD) is caused by mutations in ABCD1, a peroxisomal gene. More than half of males with an ABCD1 mutation develop inflammatory cerebral demyelination (cALD), but underlying mechanisms remain unknown and therapies are limited. We sought to develop and characterize a mouse model of cALD to facilitate study of disease mechanisms and therapy development.

Consensus guidelines for the monitoring and management of metachromatic leukodystrophy in the United States.

Metachromatic leukodystrophy (MLD) is a fatal, progressive neurodegenerative disorder caused by biallelic pathogenic mutations in the ARSA (Arylsulfatase A) gene. With the advent of presymptomatic diagnosis and the availability of therapies with a narrow window for intervention, it is critical to define a standardized approach to diagnosis, presymptomatic monitoring, and clinical care. To meet the needs of the MLD community, a panel of MLD experts was established to develop disease-specific guidelines based on healthcare resources in the United States.

Comparative Evaluation of Efficacy of Different Irrigating Needles and Devices in Removal of Debris from Apical Third of Root Canal: An SEM Study.

Background: Irrigants were required to eliminate the microbes and debris from the intraradicular space and must have direct contact with the entire root canal wall. Therefore, different irrigation methods have been proposed to deliver the irrigant as close as possible to the remote areas of the root canal.

Aim: The aim of the present study is to evaluate the cleaning efficacy of single-beveled needle, side-vented needle, endovac, and endo-irrigator plus in the removal of debris from apical third of root canal by Scanning Electron Microscope.

Case series of adenomatoid odontogenic tumour of jaws with variations in presentation.

Adenomatoid odontogenic tumour (AOT) is a rare tumour of odontogenic origin with distinct clinicopathological appearance but is often clinically misdiagnosed as a cyst. The most common site is the anterior maxilla in the canine region. We present here two cases, one at its commonest location in the maxillary canine while the other is at the uncommon location of the anterior mandible.

PTEN negatively regulates the cell lineage progression from NG2 glial progenitor to oligodendrocyte via mTOR-independent signaling.

Oligodendrocytes (OLs), the myelin-forming CNS glia, are highly vulnerable to cellular stresses, and a severe myelin loss underlies numerous CNS disorders. Expedited OL regeneration may prevent further axonal damage and facilitate functional CNS repair. Although adult OL progenitors (OPCs) are the primary players for OL regeneration, targetable OPC-specific intracellular signaling mechanisms for facilitated OL regeneration remain elusive.

An integrative system biology approach to unravel potential drug candidates for multiple age related disorders.

Aging, though an inevitable part of life, is becoming a worldwide social and economic problem. Healthy aging is usually marked by low probability of age related disorders. Good therapeutic approaches are still in need to cure age related disorders.

dbAARD & AGP: A computational pipeline for the prediction of genes associated with age related disorders.

The atrocious behavioral and physiological shift with aging accelerate occurrence of deleterious disorders. Contemporary research is focused at uncovering the role of genetic associations in age-related disorders (ARDs). While the completion of the Human Genome Project and the HapMap project has generated huge amount of data on genetic variations; Genome-Wide Association Studies (GWAS) have identified genetic variations, essentially SNPs associated with several disorders including ARDs.

mTOR pathway inhibition prevents neuroinflammation and neuronal death in a mouse model of cerebral palsy.

Background And Purpose: Mammalian target of rapamycin (mTOR) pathway signaling governs cellular responses to hypoxia and inflammation including induction of autophagy and cell survival. Cerebral palsy (CP) is a neurodevelopmental disorder linked to hypoxic and inflammatory brain injury however, a role for mTOR modulation in CP has not been investigated. We hypothesized that mTOR pathway inhibition would diminish inflammation and prevent neuronal death in a mouse model of CP.