A study published in eLife discovered that loss of muscle stem cells is the main driving force behind muscle decline in old age in mice. By depleting muscle stem cells, researchers found that muscle decline accelerated, while mice with preserved stem cells maintained healthier muscles at older ages.
Scientists have discovered how certain forms of motor neuron disease begin and progress, revealing potential new ways to slow down or even stop the process. Healthy astrocyte-supporting cells may play a role in combating neurodegenerative diseases.
A study in mice suggests that abnormalities in sensory synapses may contribute to the disease, and increasing their activity can alleviate symptoms. The findings also indicate that targeting these synapses could be a new approach for treating spinal muscular atrophy.
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Researchers have made progress in understanding how genetic mutations cause spinal muscular atrophy (SMA), a disease that cripples motor neuron function and leads to muscle weakness. A new discovery identifies the Gemin3 protein as a key player in the mechanism, and suggests targeting its receptors may be beneficial for treating SMA.
Researchers have identified a protein in urine samples as a potential biomarker for ALS, allowing for monitoring disease progression and assessing therapy effectiveness. Studies found patients with higher levels of the protein survived longer, suggesting it could be used to predict disease outcome.
Researchers found that the brain prepares both possible movements before making a decision, allowing for quick switching between plans. This 'backup plan' helps individuals react faster to changing situations, such as dodging an opponent's block in hockey.
Researchers found that the SMN protein acts as a 'molecular chaperone,' helping RNA transport into cells and promoting interactions with processing proteins. This discovery sheds light on SMA's causes and could inform optimization of treatments like nusinersen.
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Researchers identified a genetic signature in nerve cells supplying hands and feet, which is distinct from those in nearby muscles. This signature involves the coordinated activity of multiple genes and suggests that the evolution of extremities may be related to fine motor control, such as grasping.
A team of biomedical scientists has identified a molecule that targets the EphA4 receptor, a gene associated with rapid ALS progression. The research aims to delay motor neuron death and explore potential applications in spinal cord injury, Alzheimer's disease, and some cancers.
Researchers have created sensor technology for a robotic prosthetic arm that detects signals from nerves in the spinal cord, enabling users to control the device with phantom limb movements. This breakthrough could lead to more functional and user-friendly prosthetics, potentially revolutionizing the lives of amputees.
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Researchers at Nagoya University have uncovered a novel brain circuit for hunger responses during starvation, revealing the role of neuropeptide Y signals in controlling heat production by brown adipose tissue. The study found that inhibitory GABAergic inputs from medullary reticular neurons inhibit BAT thermogenesis.
Researchers found a dynamic, multi-step process in which multiple independent changes converge to transform stem cells into motor neurons. The study outlines challenges facing current cell-replacement technology but also highlights potential pathways for enhanced gene-therapy methods.
Researchers have identified a small neural circuit in male fruit flies that controls the complex mating ritual, with specific groups of cells controlling distinct steps. The findings suggest a mechanism for separating sex from reproductive function and provide insight into universal principles of nervous system coordination.
The Cedars-Sinai Board of Governors Regenerative Medicine Institute is conducting the first human trial of a novel combined stem cell and gene therapy for ALS patients. The therapy aims to preserve leg mobility by providing supportive cells that produce glial cell line derived neurotrophic factor (GDNF).
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Researchers discovered damage to RNA-binding protein hnRNP A2/B1 contributes to ALS by scrambling cellular messaging systems. The study provides a new therapeutic target for treating the disease.
Researchers have identified novel expression sites in the hippocampus and olfactory bulb of mouse brains where the C9orf72 gene is strongly expressed. This discovery provides an important resource for studying animal models of ALS and FTD, with hopes of developing new treatments and eventually cures.
A new study by the University of Malta and CNRS/Université de Montpellier reveals a faulty cellular machine cutting and pasting genetic instructions for motor neuron disease. The research suggests that ALS and SMA are related disorders with a shared mechanism that could lead to common treatments.
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Researchers have identified three new risk genes for amyotrophic lateral sclerosis (ALS) using genetic data from Project MinE. One of these genes, C21orf2, increases the risk of developing ALS by 65%. The study's findings could aid the development of personalized treatments using gene therapy.
Researchers have identified three new genes that increase the risk of motor neuron disease (MND), a debilitating condition with no effective treatments. The discovery provides new opportunities for targeted research and potentially improved outcomes for Australian patients, who are expected to benefit from increased genetic discoveries.
Researchers at Cedars-Sinai Medical Center have discovered that current engineered stem cells are too immature to accurately model ALS. To improve this, they suggest 'aging' the motor neurons in a laboratory dish to better represent the disease's progression.
Researchers at Umeå University have discovered that aggregated SOD1 protein in motor neurons causes rapid spread of ALS in mice. The study suggests a domino effect that spreads the disease up the spinal cord, mirroring human cases with hereditary traits for ALS.
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Long-term exercise has been shown to improve muscle resistance and aerobic performance in mice with Spinal Muscular Atrophy. Swimming and running both had beneficial effects on motor neurons affected by the disease, suggesting specific types of exercise may limit neurodegeneration.
Scientists have developed a method to systematically identify individual classes of brain cells, or neurons, in the spinal cord. By analyzing genetic characteristics and applying statistical approaches, researchers were able to distinguish 50 distinct types of V1 interneurons.
Loss of major histocompatibility complex I (MHCI) expression in motor neurons leads to vulnerability to ALS astrocyte toxicity. Studies show that increased MHCI expression is protective against astrocyte-induced death, providing a potential translational target for ALS treatment.
Researchers have discovered a specific enzyme, JNK3, that plays a critical role in spinal muscular atrophy and suppressing its activity could reduce disease severity. Mice with spinal muscular atrophy showed significant improvement when the JNK3 enzyme was genetically inhibited.
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A small peptide called TAxI has been shown to effectively deliver functional proteins into the spinal cord after a muscle injection. This breakthrough delivery method holds promise for carrying biologic drugs into the brain and spinal cord, potentially leading to new treatments for motor neuron disorders.
Researchers demonstrate selective gene delivery to modified upper motor neurons, showing promise for future gene replacement therapies. The study provides evidence that targets diseased cells with high specificity, laying the groundwork for effective treatment strategies.
A team of researchers discovered a protein called Pur-alpha that can protect against toxic degeneration in cells, which may lead to a treatment for ALS. The study suggests that Pur-alpha could serve as a novel therapeutic target for developing a treatment for ALS patients.
Researchers at Karolinska Institutet have discovered a new role for motor neurons in influencing rhythmic movements. Motor neurons directly control the recruitment of upstream excitatory interneurons via gap junctions, indicating they are not passive recipients of signals from interneuronal circuits.
A study in California has identified nearly 60 cases of acute flaccid myelitis, a rare syndrome similar to polio, between 2012 and 2015. The condition affects mostly children and young adults, with symptoms including respiratory or gastrointestinal illness, fever, and muscle pain.
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Research reveals tiny genetic molecules called microRNAs play crucial role in controlling muscles, with dysfunction linked to devastating neurodegenerative diseases such as ALS and SMA. The findings open new avenues for treating these disorders by correcting dysfunctional microRNAs.
A zebrafish study has discovered that serotonin boosts the growth of new motor neurons after a spinal cord injury, a finding that could lead to new therapies for neurodegenerative conditions. Researchers hope that understanding this repair mechanism in zebrafish may eventually trigger similar processes in human stem cells.
Harvard Stem Cell Institute researchers discovered molecular changes in SMA that explain why motor neurons are affected, diverging from other neurodegenerative diseases. The findings suggest a stress response pathway convergence between SMA and ALS, potentially leading to a single treatment.
Researchers have created a method to observe motor neuron activity in real-time, revealing how spinal cord cells connect with motor neurons. The findings suggest that the genetic identity of each subtype of cells is also important in forging connections.
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Researchers at NIH's NINDS have discovered a critical transport defect in motor neurons with SOD1 mutations, which causes cells to accumulate damaged materials. Increasing snapin levels during early stages of the disease can correct the problem and improve motor neuron survival.
Researchers developed a fly model to study age-dependent neurodegeneration at single cell resolution, identifying three genes involved in the process. The findings have relevance for understanding ALS progression and could lead to therapies for neurodegenerative diseases.
Researchers studying marine snails found that aging impairs sensory and motor neurons' performance in short-term memory. This results in an inability of old animals to learn, providing insights into the underlying mechanisms of age-related memory loss.
Scientists uncover fragile alliance between SMN protein and Gemins that leads to SMA. Disrupting this balance causes catastrophic consequences, including death in flies and muscle weakness.
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Researchers at Northwestern University have discovered that increased stress in the endoplasmic reticulum is a key factor in the degeneration of upper motor neurons in ALS. This finding could lead to the development of new therapies aimed at improving the survival of these critical cells.
Researchers have discovered that electric eels use a Taser-like system to locate and incapacitate their prey. The eel emits high-frequency pulses that cause muscle contractions, temporarily paralyzing the prey within three to four milliseconds.
UCL scientists have discovered a new pathway to deliver therapies to the nervous system, offering a potential treatment for tetanus and neurological disorders such as motor neuron disease. By blocking the entry of tetanus neurotoxin into nerve cells, researchers hope to develop targeted treatments with fewer side effects.
Researchers found that blocking the ALS mutation in the brain slowed disease onset and progression, suggesting that early dysfunction of brain motor neurons may contribute to later disease development. The study used rats with ALS, showing delayed disease onset and extended survival when suppressing the mutation in the brain.
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Researchers at INSERM have developed an innovative approach to produce human motor neurons from stem cells in just 14 days, nearly twice as fast as before. This breakthrough could lead to rapid progress in understanding and treating diseases like infantile spinal muscular amyotrophy and ALS.
Researchers found that aging astrocytes lose ability to protect motor neurons, but replacing old cells with engineered protein improves neuron survival. A specially engineered protein called GDNF increases motor neuron survival when cultured with aging astrocytes.
A study published in Cell Reports found that female fruit flies exhibit a preference for acetic acid, or vinegar, when carrying eggs due to sensory neurons detecting stretch in the reproductive tract. This behavior is linked to pregnancy and egg production, challenging previous assumptions about hormonal influences.
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The University of California, Irvine is leading an $8 million National Institutes of Health effort to develop a comprehensive database of human brain cell activity. The project will focus on understanding the underlying mechanisms of motor neuron disorders like ALS and other neurodegenerative diseases.
Cedars-Sinai is part of a 5-center consortium collecting and analyzing thousands of pieces of data to develop molecular signatures for motor neuron disorders. The goal is to create a 'cloud' of information that shows relationships between proteins, genes, and RNA in cells.
Researchers developed two mouse models with mitochondrial distribution defects that mimic neurological problems similar to ALS. The study found that impaired movement and muscle clasp were symptoms of the disease, suggesting a link between mitochondria distribution and motor neuron disease progression.
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A study by Lin Li et al. found that preconditioning crush reduces ventral motor neuron loss after nerve root avulsion. The authors propose a mechanism involving HSP27 induction, which attenuates cytotoxic effects of nitric oxide.
The ALS Association granted Lauren Sciences LLC a grant to develop a V-Smart therapeutic for amyotrophic lateral sclerosis (ALS), also known as Lou Gehrig's disease. The project aims to deliver neurotrophic factors across the blood brain barrier using the company's novel V-Smart nanovesicle platform technology.
Researchers at Harvard University's H SCI have identified a promising new potential treatment for ALS, using compounds already in clinical trials. The study found that inhibiting a specific receptor in glial cells increased survival time in an animal model of the disease.
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Researchers found that non-invasive brain stimulations, including rTMS and tDCS, can regulate the activities of cerebral motor cortex neurons. These findings suggest potential therapeutic benefits for individuals with central nervous system injuries or damage.
Researchers developed a novel theory of how neurons work together during complex movements, revealing a balance between excitatory and inhibitory signals. The new model can accurately reproduce multidimensional movement patterns and may aid in the understanding of brain dynamics.
Researchers found that preconditioning crush can increase the survival rate of motor neurons after spinal root avulsion. The study, published in Neural Regeneration Research, discovered that induction of heat shock protein 27 inhibits neuronal nitric oxide synthase, attenuating cytotoxic effects and preventing ventral motor neuron loss.
Two types of spinal cord neurons were identified as enabling skilled forelimb movement: excitatory interneurons for accuracy and inhibitory interneurons for smooth movement. The discovery may lead to understanding normal human motor function and potentially treating movement disorders.
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Researchers discovered specialized astrocyte functions support specific neurons, contributing to neurodegenerative disorders like ALS. The study also found potential links to developmental disorders such as autism and schizophrenia.
A team of scientists at UCL and King's College London has developed a technique to restore function to paralyzed muscles using light-activated neurons created from stem cells. The method involves transplanting these motor neurons into injured nerve branches, which can be controlled by adjusting blue light pulses.
Researchers at Harvard University have discovered a link between ALS mutations and motor neuron hyperactivity, suggesting a new therapeutic target. The approved medication for epilepsy may be effective in reducing this hyperexcitability, paving the way for clinical trials.
A University of Wisconsin-Madison researcher has pinpointed an error in protein formation that could be the root of amyotrophic lateral sclerosis. Motor neurons that control foot muscles are affected due to a shortage of one of three proteins in neurofilaments, leading to tangles and nerve fiber malfunction.
Researchers aim to deliver GDNF into muscle cells using a viral vector to protect motor neurons and slow disease progression in a rat model of ALS. The study, led by Cedars-Sinai Regenerative Medicine Institute, could pave the way for a clinical trial if successful.
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