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Dyneins are major components of microtubules. Dynein assembly is modulated by a heterogeneous group of dynein axonemal assembly factors (DNAAFs). The present study analyzes dynein axonemal assembly factor 1 (DNAAF1) and leucine-rich repeat-containing protein 50 (LRRC50), the corresponding encoded protein, in lower motor neurons in spinal cord of sALS postmortem samples and hSOD1-G93A transgenic mice compared with controls. DNAAF1 mRNA is significantly reduced in the anterior horn in sALS, and LRRC50 immunoreactivity is significantly reduced in C-boutons of the remaining motor neurons of the anterior horn, dorsal nucleus of the vagus nerve, and hypoglossal nuclei at terminal stages of ALS. LRRC50 immunoreactivity has a perinuclear distribution in motor neurons in sALS thus suggesting a disorder of transport. The number of LRRC50-/S1R-immunoreactive structures is also significantly decreased in hSOD1-G93A transgenic mice at the age of 90 days (preclinical stages), and the number of motor neurons with LRRC50-immunoreactive structures is significantly reduced in animals aged 150 days (clinical stages). These observations suggest cholinergic denervation of motor neurons as a pathogenic factor in motor neuron disease. LRRC50 protein levels were not detected in human CSF.
This article was published in the following journal.
Name: Journal of neuropathology and experimental neurology
The motility of cilia or eukaryotic flagella is powered by the axonemal dyneins, which are preassembled in the cytoplasm by proteins termed dynein arm assembly factors (DNAAFs) before being transporte...
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Universally established comprehensive clinical bulbar scales objectively assessing disease progression in ALS are currently lacking. The goal of this working group project is to design a best practice...
Detailed information about the excitatory and inhibitory synapses on the hypoglossal motoneurons may help understand the neural mechanism for control of the hypoglossal motoneuron excitability and hen...
We investigated the success rate of different surgical techniques for bulbar stricture repair.
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This study evaluates whether whole blood transferred through the new POLFA needle assembly meets supernatant hemoglobin acceptability standards.
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A winged-helix transcription factor that regulates GENE expression in metabolic tissues. It plays a role in HOMEOSTASIS of GLUCOSE and controls expression of GLUT2 PROTEIN.
A regulatory factor X transcription factor that is required for the expression of MHC CLASS II GENES. It also binds to inverted repeats of HEPATITIS B VIRUS DNA and is required for viral gene expression. RFX1 functions as a monomer or heterodimer with other RFX transcription factors.
A basic-leucine zipper transcription factor that was originally described as a transcriptional regulator controlling expression of the BETA-GLOBIN gene. It may regulate the expression of a wide variety of genes that play a role in protecting cells from oxidative damage.
A TALE-type homeodomain protein and transcription factor that functions as a regulator of PAX6 PROTEIN expression and as an activator of PLATELET FACTOR 4 gene expression. It is essential for hematopoiesis, differentiation of MEGAKARYOCYTES, and vascular patterning. It may also have a role in the induction of myeloid leukemias.
A specificity protein transcription factor that regulates expression of a variety of genes including VASCULAR ENDOTHELIAL GROWTH FACTOR and CYCLIN-DEPENDENT KINASE INHIBITOR P27.
Spinal Cord Disorders
The spinal cord is a bundle of nerves that runs down the middle of the back which carry signals back and forth between the body and brain. It is protected by vertebrae, which are the bone disks that make up the spine. An accident that damages the verte...