45 research outputs found

    Assessing Rat Forelimb and Hindlimb Motor Unit Connectivity as Objective and Robust Biomarkers of Spinal Motor Neuron Function

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    Sensitive and objective biomarkers of neuronal injury, degeneration, and regeneration can help facilitate translation of experimental findings into clinical testing. Whereas measures of upper motor neuron connectivity have been readily established, functional assessments of lower motor neuron (LMN) innervation of forelimb muscles are lacking. Compound muscle action potential (CMAP) and motor unit (MU) number estimation (MUNE) are well-established methods that allow longitudinal MU integrity monitoring in patients. In analogy we refined CMAP and MUNE methods for assessing spinal MU input in the rat forelimb and hindlimb. Repeated CMAP and MUNE recordings are robust (coefficients of variability: 4.5–11.3%), and MUNE measurements from forelimb wrist flexor muscles (415 ± 8 [SEM]) align with back-traced anatomical LMN counts (336 ± 16 [SEM]). For disease validation, cross-sectional blinded electrophysiological and muscle contractility measurements were obtained in a cohort of G93A SOD1 mutant overexpressing rats and compared with controls. Longitudinal assessment of mutant animals demonstrated progressive motor unit decline in the hindlimb to a greater extent than the forelimb. Hindlimb CMAP and MUNE demonstrated strong correlations with plantarflexion muscle contractility. Cross-species assessment of upper/fore- limb and lower/hind- limb motor units using objective electrophysiological CMAP and MUNE values as biomarkers will guide and improve bi-directional translation

    Substantial impacts of subsistence fishing on the population status of an Endangered reef predator at a remote coral atoll

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    Napoleon wrasse Cheilinus undulatus has declined drastically throughout most of its range, owing, in large part, to overexploitation. In Anaa, French Polynesia, the species is harvested as part of the subsistence catch by fishers using rockpile traps, spearguns, handmade harpoons, and baited handlines. We sampled 70 Napoleon wrasse captured by artisanal fishers of Anaa between 2015 and 2018 to assess the status of this population, and we applied data-poor fisheries models to assess the stock status of this iconic reef predator. The species was determined to be overexploited at a rate of 0.82 based on values of natural (0.14; Hoenig method) and fishing (0.58; difference of total and natural mortality) mortality as components of total mortality (0.72; Beverton-Holt estimation). The left-skewed length distribution (mean = 36 ± 13 cm SL) suggested an under-representation of large adults in the population, which would predominantly be terminal males in this sequentially hermaphroditic protogynous fish. This was not considered to be reflective of poor sel

    Novel disease-causing variants and phenotypic features of X-linked megalocornea

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    Purpose: The aim of the study was to describe the phenotype and molecular genetic causes of X-linked megalocornea (MGC1). We recruited four British, one New Zealand, one Vietnamese and four Czech families. // Methods: All probands and three female carriers underwent ocular examination and Sanger sequencing of the CHRDL1 gene. Two of the probands also had magnetic resonance imaging (MRI) of the brain. // Results: We identified nine pathogenic or likely pathogenic and one variant of uncertain significance in CHRDL1, of which eight are novel. Three probands had ocular findings that have not previously been associated with MGC1, namely pigmentary glaucoma, unilateral posterior corneal vesicles, unilateral keratoconus and unilateral Fuchs heterochromic iridocyclitis. The corneal diameters of the three heterozygous carriers were normal, but two had abnormally thin corneas, and one of these was also diagnosed with unilateral keratoconus. Brain MRI identified arachnoid cysts in both probands, one also had a neuroepithelial cyst, while the second had a midsagittal neurodevelopmental abnormality (cavum septum pellucidum et vergae). // Conclusion: The study expands the spectrum of pathogenic variants and the ocular and brain abnormalities that have been identified in individuals with MGC1. Reduced corneal thickness may represent a mild phenotypic feature in some heterozygous female carriers of CHRDL1 pathogenic variants

    An Assessment of Mobile Predator Populations along Shallow and Mesophotic Depth Gradients in the Hawaiian Archipelago.

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    Large-bodied coral reef roving predators (sharks, jacks, snappers) are largely considered to be depleted around human population centers. In the Hawaiian Archipelago, supporting evidence is primarily derived from underwater visual censuses in shallow waters (=30?m). However, while many roving predators are present or potentially more abundant in deeper strata (30-100?m+), distributional information remains sparse. To partially fill that knowledge gap, we conducted surveys in the remote Northwestern Hawaiian Islands (NWHI) and populated Main Hawaiian Islands (MHI) from 2012-2014 using baited remote underwater stereo-video. Surveys between 0-100?m found considerable roving predator community dissimilarities between regions, marked conspicuous changes in species abundances with increasing depth, and largely corroborated patterns documented during shallow water underwater visual censuses, with up to an order of magnitude more jacks and five times more sharks sampled in the NWHI compared to the MHI. Additionally, several species were significantly more abundant and larger in mesophotic versus shallow depths, which remains particularly suggestive of deep-water refugia effects in the MHI. Stereo-video extends the depth range of current roving predator surveys in a more robust manner than was previously available, and appears to be well-suited for large-scale roving predator work in the Hawaiian Archipelago

    Data from: Effects of gear restriction on the abundance of juvenile fishes along sandy beaches in Hawai'i

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    In 2007, due to growing concerns of declines in nearshore fisheries in Hawai‘i, a ban on gillnets was implemented in designated areas around the island of O‘ahu in the main Hawaiian Islands. Utilizing a 17 year time-series of juvenile fish abundance beginning prior to the implementation of the gillnet ban, we examined the effects of the ban on the abundance of juveniles of soft-bottom associated fish species. Using a Before-After-Control-Impact (BACI) sampling design, we compared the abundance of targeted fishery species in a bay where gillnet fishing was banned (Kailua, O‘ahu), and an adjacent bay where fishing is still permitted (Waimānalo, O‘ahu). Our results show that when multiple juvenile fish species were combined, abundance declined over time in both locations, but the pattern varied for each of the four species groups examined. Bonefishes were the only species group with a significant BACI effect, with higher abundance in Kailua in the period after the gillnet ban. This study addressed a need for scientific assessment of a fisheries regulation that is rarely possible due to lack of quality data before enactment of such restrictions. Thus, we developed a baseline status of juveniles of an important fishery species, and found effects of a fishery management regulation in Hawai‘i

    Population dynamics of roundjaw bonefish Albula glossodonta at a remote coralline Atoll inform community-based management in an artisanal fishery

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    Fisheries management requires knowledge on the population dynamics of exploited stocks. To that end, the present study used a mark–recapture approach to characterise the population demographics of roundjaw bonefish Albula glossodonta (Forsskål) and their interaction with a data-limite
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