What Is Transactive Response Rna Binding Protein 43 )?

Amyotrophic meaning, “no muscle nourishment” in Greek, lateral meaning where the neurons are in the spinal cord, and sclerosis meaning “scarring.” ALS, often known as “Lou Gehrig’s Disease,” named after the New York Yankee who first brought awareness to the disease in the late 1930’s, is a neurodegenerative disease, which affects the neurons in the brain. The nerve cells in the brain and spinal cord that are responsible for sending and receiving motor signals progressively die off, causing the deterioration of simple motor skills in patients with ALS, such as walking, talking, and eventually speaking and breathing, however thinking is not affected by ALS. Early symptoms cause the person to slowly lose mobility of limbs, but in a matter of a few years, the person loses the mobility of most of their body and will eventually lose the ability to eat and breath, which will ultimately cause death. ALS deteriorates the patient's body, however does not affect the patient's state of mind or sanity while the rest of the body shuts down. People usually get ALS between the ages of 40 and 70. However, there is a growing trend where athletes are getting ALS in their thirties. ALS can be contributed to genetic predisposition, which means that the gene that is responsible for ALS is already in the person’s DNA. In recent studies, however, it was observed that individuals who have had suffered multiple concussions or any other head trauma are

Have you ever heard of ALS, better known as Lou Gehrig’s disease? For many people, ALS is a disorder that they may not know much about. I never heard of it either until my father was diagnosed with this disease in 2006. Because there is no known cure, it is important to detect this disease early, so that proper treatments and preparation can be done before it’s too late.

RNA interference, or RNAi, is a biological process in which RNA molecules reduce the gene expression of an organism. This is done typically by causing the destruction of specific mRNA molecules. RNAs are direct products of genes, these small RNAs can bind to other mRNA molecules to either increase or decrease their activity like in the example of preventing an mRNA from producing a protein. There are two types of RNA molecules that are central to RNAi, these molecules are, micro RNA (miRNA) and small interfering RNA (siRNA).

People first need to know what ALS is. ALS is very difficult to diagnose. So far there is no

These include motor neuron disease, corticobasal syndrome, progressive supranuclear palsy and FTDP-17 (FTD with parkinsonism linked to chromosome 17).

Amyotrophic lateral sclerosis also known as ALS or Lou Gehrig’s disease, is a genetic disorder that has proven

While ALS attacks the body except for the brain, CTE is a disease that attacks the brain and can only be diagnosed after death. According to the Concussion Legacy Foundation, the way CTE works is from a protein called tau. This protein clumps, spreads throughout the brain and ends up killing the brain cells. During the disease, the victim's mood and behavior are affected as well as changing their impulse control, causing depression, aggression, and paranoia. The causes of CTE

Tay Sachs Disease, or TSD, is a fatal genetic disorder that result in the degeneration of the nervous system. TSD is presented in three forms, those being classic infantile, juvenile and adult late-onset. The most commonly seen form of TSD is that of classic infantile Tay Sachs Disease. In the classic infantile form, infants generally experience their first symptoms by 6 months of age. Death of infants with TSD is typically by age 5. The symptoms that TSD infants will experience prior to death include: an onset of retardation, paralysis, dementia, blindness and reoccurring seizures. The absence of hexosaminidase-A, or what we will refer to as Hex-A, is the cause

Picture sitting up to reach for an object but are incapable of moving a muscle. Although completely aware of the surroundings, being unable to interact with any of it as if trapped in a shell, or loved ones noticing increasing instability and offbeat behaviour. These are the lives of Amyotrophic Lateral Sclerosis, or ALS but more commonly known as Lou Gehrig’s disease, and Chronic Traumatic Encephalopathy, or CTE. ALS affects those with the disease by causing full body paralysis. However the disease leaves the brain completely intact. Contrary to ALS is a CTE, a disease that attacks the brain. The build of a specific toxin in the brain is the culprit that leads to the disease which causes brain damage. Concussions are another serious factor

Many scientists hypothesize that protein dysfunction plays a role in the progressive damage and death of nerve cells. The nerve cells may be blocked from communication

Gene mutations prevent the breakdown of toxic substances leading to their buildup in nerve cells, which eventually is cell death. (Genetics Home Reference, 2012) Other causes are disorganized immune responses, protein mishandling, and lead exposure. A disorganized immune response may trigger the ALS process because of the proteins in cells, which causes cell death of nerves. In the same fashion, Protein mishandlings can lead to the steady amassing of irregular proteins, causing cell death. Additionally evidence suggests that lead exposure can lead the progression of

Moglia, P. P., & Margolis, D. D. (2017). Amyotrophic lateral sclerosis. Magill’S Medical Guide (Online Edition)

Which functions in transcription of rRNA by interacting with upstream binding factor (UBF), an RNA polymerase I present within the nucleolus (Dauwerse et al., 2010). It was acknowledged that Tcof1 is expressed throughout the neuroepithelium strongly where its role is crucial for cell survival (Dixon et al., 1996), which was supported by an earlier study that demonstrated Tcof1 +/- haploinsufficient mouse models developed Treacher Collin’s syndrome due to widespread apoptosis of neuroepithelial cells, defective synthesis and proliferation of neural crest cells and deficiencies in ribosome biogenesis (Valdez et al.,

The aggregation of misfolded proteins may occur in different cells and regions of the body, originating a variety of disorders. When affecting the central nervous system (CNS) proteinopathies are often neurodegenerative disorders, and can be characterized by one or more proteinaceous aggregates (Bayer, 2015). Neurons are quite sensitive to the effects of misfolded proteins due to their post-mitotic nature and structure (Wolfe, 2012). Indeed, the long and narrow axonal projections of neurons can be easily clogged by accumulating proteins or by inefficient transport of nutrients and organelles (Wolfe, 2012). Additionally, accumulated misfolded proteins cannot be diluted through cell division, thereby turning neuron’s integrity highly dependent

A distinctive characteristic of ALS is that although the motor neurons die, the brain, cognitive functions and sensory neurons stay intact (Porth & Matfin, 2009). This makes the disease especially devastating because patients become trapped inside their dying body, with a fully alert mind, but are unable to move. It is not known what causes the exact death of the motor neurons in the body, but “five percent to 10% of cases are familial; the others are believed to be sporadic” (Porth & Matfin, 2009, p.