TDP43 autoregulation gives rise to dominant negative isoforms that are tightly controlled by transcriptional and post-translational mechanisms.

The nuclear RNA-binding protein TDP43 is integrally involved in the pathogenesis of amyotrophic lateral sclerosis (ALS) and frontotemporal lobar degeneration (FTLD). Previous studies uncovered N-terminal TDP43 isoforms that are predominantly cytosolic in localization, prone to aggregation, and enriched in susceptible spinal motor neurons. In healthy cells, however, these shortened (s)TDP43 isoforms are difficult to detect in comparison to full-length (fl)TDP43, raising questions regarding their origin and selective regulation.

TDP43 autoregulation gives rise to shortened isoforms that are tightly controlled by both transcriptional and post-translational mechanisms.

The nuclear RNA-binding protein TDP43 is integrally involved in the pathogenesis of amyotrophic lateral sclerosis (ALS) and frontotemporal lobar degeneration (FTLD). Previous studies uncovered N-terminal TDP43 isoforms that are predominantly cytosolic in localization, highly prone to aggregation, and enriched in susceptible spinal motor neurons. In healthy cells, however, these shortened (s)TDP43 isoforms are difficult to detect in comparison to full-length (fl)TDP43, raising questions regarding their origin and selective regulation.

Long-Term Survival Rates and Treatment Trends of Burkitt Lymphoma in Patients with HIV-A National Cancer Database (NCDB) Study.

Background: Burkitt lymphoma (BL) accounts for 10-35% of AIDS-defining lymphoma in people with HIV (PWH). Previous research consisting of smaller cohorts has shown decreased survival for HIV-associated BL. This study aims to compare overall mortality in BL patients with and without HIV, while investigating impact of treatment modalities in HIV-associated BL.

Readmission rates in HIV-associated burkitt lymphoma patients in the USA: a nationwide readmission database (NRD) analysis.

Background: People with human immunodeficiency virus have an increased risk of developing AIDS-defining malignancies including Burkitt lymphoma. Survival outcomes in HIV-associated Burkitt lymphoma remain worse than non-HIV-associated Burkitt lymphoma, despite widespread implementation of antiretroviral therapy. We aimed to determine the association between HIV status and risk for 30-day and 90-day readmission in the US after index hospitalization for Burkitt lymphoma.

Dissecting the roles of EIF4G homologs reveals DAP5 as a modifier of CGG repeat-associated toxicity in a Drosophila model of FXTAS.

Neurodegeneration in Fragile X-associated tremor/ataxia syndrome (FXTAS) is caused by a CGG trinucleotide repeat expansion in the 5' UTR of FMR1. Expanded CGG repeat RNAs form stable secondary structures, which in turn support repeat-associated non-AUG (RAN) translation to produce toxic peptides. The parameters that impact RAN translation initiation efficiency are not well understood.

Sports Participation and Neurological Injuries in Pediatric Patients With Chiari I Malformation.

Background: Currently, there is no consensus recommendation regarding the safety of sports participation for pediatric patients with Chiari I malformation (CM-I).

Objective: To prospectively survey the treated and untreated patients with CM-I to define the risk of sports-associated neurological injury.

Methods: A prospective survey was administered to 744 pediatric patients at one neurosurgery clinic between 2010 and 2021.

The impact of misplaced percutaneous iliac dynamic reference frame pins used during navigated spine surgery: incidence and outcomes.

Objective: Image guidance requires placement of a dynamic reference frame (DRF), often either onto local spinous process or by freehand intraosseous DRF placement into the ilium via the posterior superior iliac spine (PSIS). There is a paucity of studies in the literature that describe the complications of intraosseous DRF placement. The aim of this study was to describe the radiographic location, prevalence and nature of complications, and long-term clinical outcomes of attempted DRF placement into the PSIS.

Subdural hematoma prevalence and long-term developmental outcomes in patients with benign expansion of the subarachnoid spaces.

Objective: Benign expansion of the subarachnoid spaces (BESS) is a condition seen in macrocephalic infants. BESS is associated with mild developmental delays which tend to resolve within a few years. It is accepted that patients with BESS are at increased risk of spontaneous subdural hematomas (SDHs), although the exact pathophysiology is not well understood.

Patients who meet electrodiagnostic criteria for CIDP rarely present with a sensory predominant DSP phenotype.

Introduction: It is unknown how often patients with electrodiagnostic evidence of chronic inflammatory demyelinating polyradiculoneuropathy (CIDP), a potentially treatable condition, present with a distal symmetric polyneuropathy (DSP) phenotype.

Methods: We reviewed the records of patients who presented to our electrodiagnostic laboratory between January 1, 2011, to December 31, 2019, and fulfilled electrodiagnostic criteria for CIDP to identify those who presented with a sensory predominant DSP phenotype.

Results: One hundred sixty-two patients had a chronic acquired demyelinating neuropathy, of whom 138 met criteria for typical or atypical CIDP.