Combined temperature and salinity effects on the passive sampling of PAHs with an assessment of impacts to petroleum toxicity.

In equilibrium-based passive sampling applications, the accuracy of estimating freely dissolved concentration () of hydrophobic organic compounds (HOCs) relies on the passive sampler-water partition coefficient () values applied. The vast majority of are generated under standard conditions: 20 °C in deionized or freshwater. Few empirically derived values are available for non-standard conditions.

Application of low-density polyethylene (LDPE) passive samplers for monitoring PAHs in groundwater.

Equilibrium passive sampling continues to find increasing use for performing in situ assessments and monitoring of hydrophobic organic compounds (HOCs). Although this method has been successfully used in several field studies including open surface waters and sediments, comparatively, their use in groundwater has been very limited. In this study, low-density polyethylene (LDPE) passive samplers were deployed for 80 days in three groundwater wells contaminated with polycyclic aromatic hydrocarbons (PAHs).

A C-terminal motif containing a PKC phosphorylation site regulates γ-Protocadherin-mediated dendrite arborization in the cerebral cortex in vivo.

The Pcdhg gene cluster encodes 22 γ-Protocadherin (γ-Pcdh) cell adhesion molecules that critically regulate multiple aspects of neural development, including neuronal survival, dendritic and axonal arborization, and synapse formation and maturation. Each γ-Pcdh isoform has unique protein domains-a homophilically interacting extracellular domain and a juxtamembrane cytoplasmic domain-as well as a C-terminal cytoplasmic domain shared by all isoforms. The extent to which isoform-specific versus shared domains regulate distinct γ-Pcdh functions remains incompletely understood.

Assessing petroleum contamination in parts of the Niger Delta based on a sub-catchment delineated field assessment.

The Niger Delta in Nigeria is a complex and heavily contaminated area with over 150,000 interconnected contaminated sites. This intricate issue is compounded by the region's strong hydrological processes and high-energy environment, necessitating a science-based approach for effective contamination assessment and management. This study introduces the concept of sub-catchment contamination assessment and management, providing an overarching perspective rather than addressing each site individually.

The distribution of sediment microplastics assemblages is driven by location and hydrodynamics, not sediment characteristics, in the Gulf of Maine, USA.

Microplastics (MP) are found in marine sediments across the globe, but we are just beginning to understand their spatial distribution and assemblages. In this study, we quantified MP in Gulf of Maine, USA sediments. MP were extracted from 20 sediment samples, followed by polymer identification using Raman spectroscopy.

Sediment Toxicity Tests: A Critical Review of Their use in Environmental Regulations.

Sediments are an integral component of aquatic systems, linking multiple water uses, functions, and services. Contamination of sediments by chemicals is a worldwide problem, with many jurisdictions trying to prevent future pollution (prospective) and manage existing contamination (retrospective). The present review assesses the implementation of sediment toxicity testing in environmental regulations globally.

Boosting BDNF in muscle rescues impaired axonal transport in a mouse model of DI-CMTC peripheral neuropathy.

Charcot-Marie-Tooth disease (CMT) is a genetic peripheral neuropathy caused by mutations in many functionally diverse genes. The aminoacyl-tRNA synthetase (ARS) enzymes, which transfer amino acids to partner tRNAs for protein synthesis, represent the largest protein family genetically linked to CMT aetiology, suggesting pathomechanistic commonalities. Dominant intermediate CMT type C (DI-CMTC) is caused by YARS1 mutations driving a toxic gain-of-function in the encoded tyrosyl-tRNA synthetase (TyrRS), which is mediated by exposure of consensus neomorphic surfaces through conformational changes of the mutant protein.

Boosting BDNF in muscle rescues impaired axonal transport in a mouse model of DI-CMTC peripheral neuropathy.

Charcot-Marie-Tooth disease (CMT) is a genetic peripheral neuropathy caused by mutations in many functionally diverse genes. The aminoacyl-tRNA synthetase (ARS) enzymes, which transfer amino acids to partner tRNAs for protein synthesis, represent the largest protein family genetically linked to CMT aetiology, suggesting pathomechanistic commonalities. Dominant intermediate CMT type C (DI-CMTC) is caused by mutations driving a toxic gain-of-function in the encoded tyrosyl-tRNA synthetase (TyrRS), which is mediated by exposure of consensus neomorphic surfaces through conformational changes of the mutant protein.

Lack of effect from genetic deletion of Hdac6 in a humanized mouse model of CMT2D.

Background: Inhibition of HDAC6 has been proposed as a broadly applicable therapeutic strategy for Charcot-Marie-Tooth disease (CMT). Inhibition of HDAC6 increases the acetylation of proteins important in axonal trafficking, such as α-tubulin and Miro, and has been shown to be efficacious in several preclinical studies using mouse models of CMT.

Aims: Here, we sought to expand on previous preclinical studies by testing the effect of genetic deletion of Hdac6 on mice carrying a humanized knockin allele of Gars1, a model of CMT-type 2D.

Dominant mutations causing axonal Charcot-Marie-Tooth disease expand -associated diseases.

Pathogenic variants in six aminoacyl-tRNA synthetase (ARS) genes are implicated in neurological disorders, most notably inherited peripheral neuropathies. ARSs are enzymes that charge tRNA molecules with cognate amino acids. Pathogenic variants in asparaginyl-tRNA synthetase () cause a neurological phenotype combining developmental delay, ataxia and demyelinating peripheral neuropathy.

Testing SIPA1L2 as a modifier of CMT1A using mouse models.

Charcot-Marie-Tooth disease type 1A (CMT1A) is a demyelinating peripheral neuropathy caused by the duplication of peripheral myelin protein 22 (PMP22), leading to muscle weakness and loss of sensation in the hands and feet. A recent case-only genome-wide association study of CMT1A patients conducted by the Inherited Neuropathy Consortium identified a strong association between strength of foot dorsiflexion and variants in signal induced proliferation associated 1 like 2 (SIPA1L2), indicating that it may be a genetic modifier of disease. To validate SIPA1L2 as a candidate modifier and to assess its potential as a therapeutic target, we engineered mice with deletion of exon 1 (including the start codon) of the Sipa1l2 gene and crossed them to the C3-PMP22 mouse model of CMT1A.

Where the rubber meets the road: Emerging environmental impacts of tire wear particles and their chemical cocktails.

About 3 billion new tires are produced each year and about 800 million tires become waste annually. Global dependence upon tires produced from natural rubber and petroleum-based compounds represents a persistent and complex environmental problem with only partial and often-times, ineffective solutions. Tire emissions may be in the form of whole tires, tire particles, and chemical compounds, each of which is transported through various atmospheric, terrestrial, and aquatic routes in the natural and built environments.

Quantitative thermodynamic exposure assessment of PCBs available to sandworms () in activated carbon remediated sediment during ongoing sediment deposition.

Marine mesoscale studies with sandworms () were conducted to isolate important processes governing the exposure and bioaccumulation of polychlorinated biphenyls (PCBs) at contaminated sediment sites. equilibrium sampling with silicone-coated jars, and passive sampling with low-density polyethylene (LDPE) were used to determine the performance of an activated carbon (AC) amendment remedy applied to the bed sediment. A quantitative thermodynamic exposure assessment ('QTEA') was performed, showing that PCB concentrations in polymers at equilibrium with the surficial sediment were suited to measure and assess the remedy effectiveness with regard to PCB bioaccumulation in worms.

A C-terminal motif containing a PKC phosphorylation site regulates γ-Protocadherin-mediated dendrite arborization in the cerebral cortex .

The gene cluster encodes 22 γ-Protocadherin (γ-Pcdh) cell adhesion molecules that critically regulate multiple aspects of neural development, including neuronal survival, dendritic and axonal arborization, and synapse formation and maturation. Each γ-Pcdh isoform has unique protein domains-a homophilically-interacting extracellular domain and a juxtamembrane cytoplasmic domain-as well as a C-terminal cytoplasmic domain shared by all isoforms. The extent to which isoform-specific shared domains regulate distinct γ-Pcdh functions remains incompletely understood.

A Review of Mechanistic Models for Predicting Adverse Effects in Sediment Toxicity Testing.

Since recognizing the importance of bioavailability for understanding the toxicity of chemicals in sediments, mechanistic modeling has advanced over the last 40 years by building better tools for estimating exposure and making predictions of probable adverse effects. Our review provides an up-to-date survey of the status of mechanistic modeling in contaminated sediment toxicity assessments. Relative to exposure, advances have been most substantial for non-ionic organic contaminants (NOCs) and divalent cationic metals, with several equilibrium partitioning-based (Eq-P) models having been developed.

Misregulation of mitochondria-lysosome contact dynamics in Charcot-Marie-Tooth Type 2B disease Rab7 mutant sensory peripheral neurons.

Inter-organelle contact sites between mitochondria and lysosomes mediate the crosstalk and bidirectional regulation of their dynamics in health and disease. However, mitochondria-lysosome contact sites and their misregulation have not been investigated in peripheral sensory neurons. Charcot-Marie-Tooth type 2B disease is an autosomal dominant axonal neuropathy affecting peripheral sensory neurons caused by mutations in the GTPase Rab7.

Using eRNA/eDNA metabarcoding to detect community-level impacts of nanoplastic exposure to benthic estuarine ecosystems.

Plastic particles are ubiquitous in marine systems and fragment into smaller pieces, such as nanoplastics (NPs). The effects of NPs on marine organisms are of growing concern but are not well understood. Marine sediments act as a sink for many contaminants, like microplastics, and are rich habitats for benthic micro- and meiofauna which are ecologically-important components of marine food webs; however, little is known about the sensitivities of specific organisms to NPs or the effects on community diversity and composition.

Two new mouse models of Gjb1-associated Charcot-Marie-Tooth disease type 1X.

Background: Charcot-Marie-Tooth disease type 1X is caused by mutations in GJB1, which is the second most common gene associated with inherited peripheral neuropathy. The GJB1 gene encodes connexin 32 (CX32), a gap junction protein expressed in myelinating glial cells. The gene is X-linked, and the mutations cause a loss of function.

A Novel ENU-Induced Mutation Causes Motor Deficits in Mice without Causing Peripheral Neuropathy.

Mitochondrial fission and fusion are required for maintaining functional mitochondria. The mitofusins (MFN1 and MFN2) are known for their roles in mediating mitochondrial fusion. Recently, MFN2 has been implicated in other important cellular functions, such as mitophagy, mitochondrial motility, and coordinating endoplasmic reticulum-mitochondria communication.

Passive Sampling-Based versus Conventional-Based Metrics for Evaluating Remediation Efficacy at Contaminated Sediment Sites: A Review.

Passive sampling devices (PSDs) are increasingly used at contaminated sites to improve the characterization of contaminant transport and assessment of ecological and human health risk at sediment sites and to evaluate the effectiveness of remedial actions. The use of PSDs after full-scale remediation remains limited, however, in favor of evaluation based on conventional metrics, such as bulk sediment concentrations or bioaccumulation. This review has three overall aims: (1) identify sites where PSDs have been used to support cleanup efforts, (2) assess how PSD-derived remedial end points compare to conventional metrics, and (3) perform broad semiquantitative and selective quantitative concurrence analyses to evaluate the magnitude of agreement between metrics.