Characterization of neurons from immortalized dental pulp stem cells for the study of neurogenetic disorders.

A major challenge to the study and treatment of neurogenetic syndromes is accessing live neurons for study from affected individuals. Although several sources of stem cells are currently available, acquiring these involve invasive procedures, may be difficult or expensive to generate and are limited in number. Dental pulp stem cells (DPSCs) are multipotent stem cells that reside deep the pulp of shed teeth.

Variation in Dube3a expression affects neurotransmission at the Drosophila neuromuscular junction.

Changes in UBE3A expression levels in neurons can cause neurogenetic disorders ranging from Angelman syndrome (AS) (decreased levels) to autism (increased levels). Here we investigated the effects on neuronal function of varying UBE3A levels using the Drosophila neuromuscular junction as a model for both of these neurogenetic disorders. Stimulations that evoked excitatory junction potentials (EJPs) at 1 Hz intermittently failed to evoke EJPs at 15 Hz in a significantly higher proportion of Dube3a over-expressors using the pan neuronal GAL4 driver C155-GAL4 (C155-GAL4>UAS-Dube3a) relative to controls (C155>+ alone).

Variation in sodium current amplitude between vasopressin and oxytocin hypothalamic supraoptic neurons.

Biophysical characteristics of tetrodotoxin-sensitive sodium (Na(+)) currents were studied in vasopressin (VP) and oxytocin (OT) supraoptic neurons acutely isolated from rat hypothalamus. Na(+) current density (pA/pF) was significantly greater in VP neurons than in OT neurons. No significant difference between VP and OT neurons was detected regarding the voltage dependence of activation and steady-state inactivation, or rate of recovery from inactivation of Na(+) currents.

The distribution of low-threshold TTX-resistant Na⁺ currents in rat trigeminal ganglion cells.

The distribution of low-threshold tetrodotoxin-resistant (TTX-r) Na(+) current and its co-expression with high-threshold TTX-r Na(+) current were studied in randomly selected acutely dissociated rat trigeminal ganglion (non-identified TG cells) and TG cells serving the temporomandibular joint (TMJ-TG cells). Conditions previously shown to enhance Na(V)1.9 channel-mediated currents (holding potential (HP) -80 mV, 130-mM fluoride internally) were employed to amplify the low-threshold Na(+) current.

Up-regulation of low-threshold tetrodotoxin-resistant Na+ current via activation of a cyclic AMP/protein kinase A pathway in nociceptor-like rat dorsal root ganglion cells.

The effects of forskolin on low-threshold tetrodotoxin-resistant (TTX-r) Na(+) currents was studied in small diameter (average ≈ 25 μm) dorsal root ganglion (DRG) cells. All DRG cells included in the study were categorized as type-2 or non-type-2 based on the expression of a low-threshold A-current. In all type-2 and some non-type-2 DRG cells held at -80 mV, the adenylyl cyclase (AC) activator forskolin (10 μM) up-regulated TTX-r Na(+) currents evoked with steps to -55 mV through -35 mV (low-threshold current).