keyword
MENU ▼
Read by QxMD icon Read
search

MYRF

keyword
https://www.readbyqxmd.com/read/29476661/epigenomic-signature-of-adrenoleukodystrophy-predicts-compromised-oligodendrocyte-differentiation
#1
Agatha Schlüter, Juan Sandoval, Stéphane Fourcade, Angel Díaz-Lagares, Montserrat Ruiz, Patrizia Casaccia, Manel Esteller, Aurora Pujol
Epigenomic changes may either cause disease or modulate its expressivity, adding a layer of complexity to mendelian diseases. X-linked adrenoleukodystrophy (X-ALD) is a rare neurometabolic condition exhibiting discordant phenotypes, ranging from a childhood cerebral inflammatory demyelination (cALD) to an adult-onset mild axonopathy in spinal cords (AMN). The AMN form may occur with superimposed inflammatory brain demyelination (cAMN). All patients harbor loss of function mutations in the ABCD1 peroxisomal transporter of very-long chain fatty acids...
February 24, 2018: Brain Pathology
https://www.readbyqxmd.com/read/29472293/degradation-of-the-endoplasmic-reticulum-anchored-transcription-factor-myrf-by-the-ubiquitin-ligase-scf-fbxw7-in-a-manner-dependent-on-the-kinase-gsk-3
#2
Shogo Nakayama, Kanae Yumimoto, Atsuki Kawamura, Keiichi I Nakayama
The ubiquitin-proteasome system regulates the abundance of many cellular proteins by mediating their targeted degradation. We previously developed a method-differential proteomics-based identification of ubiquitylation substrates (DiPIUS)-for the comprehensive identification of substrates for a given F-box protein subunit of SCF-type ubiquitin ligases. We have now applied DiPIUS to the F-box protein Fbxw7 in three cell lines (mHepa, Neuro2A, and C2C12) and thereby identified myelin regulatory factor (MyRF)-an endoplasmic reticulum-anchored transcription factor that is essential for myelination of nerves in the central nervous system-as a candidate substrate of Fbxw7 specifically in mHepa cells...
February 22, 2018: Journal of Biological Chemistry
https://www.readbyqxmd.com/read/29446546/de-novo-variants-in-myelin-regulatory-factor-myrf-as-candidates-of-a-new-syndrome-of-cardiac-and-urogenital-anomalies
#3
Hailey Pinz, Louise C Pyle, Dong Li, Kosuke Izumi, Cara Skraban, Jennifer Tarpinian, Stephen R Braddock, Aida Telegrafi, Kristin G Monaghan, Elaine Zackai, Elizabeth J Bhoj
Myelin Regulatory Factor (MYRF) is a transcription factor that has previously been associated with the control of the expression of myelin-related genes. However, it is highly expressed in human tissues and mouse embryonic tissues outside the nervous system such as the stomach, lung, and small intestine. It has not previously been reported as a cause of any Mendelian disease. We report here two males with Scimitar syndrome [MIM 106700], and other features including penoscrotal hypospadias, cryptorchidism, pulmonary hypoplasia, tracheal anomalies, congenital diaphragmatic hernia, cleft spleen, thymic involution, and thyroid fibrosis...
February 15, 2018: American Journal of Medical Genetics. Part A
https://www.readbyqxmd.com/read/29343252/insights-into-the-genetics-of-blood-pressure-in-black-south-african-individuals-the-birth-to-twenty-cohort
#4
Liesl M Hendry, Venesa Sahibdeen, Ananyo Choudhury, Shane A Norris, Michèle Ramsay, Zané Lombard
BACKGROUND: Cardiovascular diseases (CVDs) are the leading cause of non-communicable disease deaths globally, with hypertension being a major risk factor contributing to CVDs. Blood pressure is a heritable trait, with relatively few genetic studies having been performed in Africans. This study aimed to identify genetic variants associated with variance in systolic (SBP) and diastolic (DBP) blood pressure in black South Africans. METHODS: Genotyping was performed using the Metabochip in a subset of participants (mixed sex; median age 17...
January 17, 2018: BMC Medical Genomics
https://www.readbyqxmd.com/read/29034508/ulk4-deficiency-leads-to-hypomyelination-in-mice
#5
Min Liu, Ping Xu, Zhenlong Guan, Xiaohong Qian, Peter Dockery, Una Fitzgerald, Timothy O'Brien, Sanbing Shen
Brain nerve fibers are insulated by myelin which is produced by oligodendrocytes. Defects in myelination are increasingly recognized as a common pathology underlying neuropsychiatric and neurodevelopmental disorders, which are associated with deletions of the Unc-51-like kinase 4 (ULK4) gene. Key transcription factors have been identified for oligodendrogenesis, but little is known about their associated regulators. Here we report that Ulk4 acts as a key regulator of myelination. Myelination is reduced by half in the Ulk4(tm1a/tm1a) hypomorph brain, whereas expression of axonal marker genes Tubb3, Nefh, Nefl and Nefm remains unaltered...
January 2018: Glia
https://www.readbyqxmd.com/read/28631093/myelin-regulatory-factor-drives-remyelination-in-multiple-sclerosis
#6
Greg J Duncan, Jason R Plemel, Peggy Assinck, Sohrab B Manesh, Fraser G W Muir, Ryan Hirata, Matan Berson, Jie Liu, Michael Wegner, Ben Emery, G R Wayne Moore, Wolfram Tetzlaff
Remyelination is limited in the majority of multiple sclerosis (MS) lesions despite the presence of oligodendrocyte precursor cells (OPCs) in most lesions. This observation has led to the view that a failure of OPCs to fully differentiate underlies remyelination failure. OPC differentiation requires intricate transcriptional regulation, which may be disrupted in chronic MS lesions. The expression of few transcription factors has been differentially compared between remyelinating lesions and lesions refractory to remyelination...
September 2017: Acta Neuropathologica
https://www.readbyqxmd.com/read/28623291/crystal-structure-of-the-dna-binding-domain-of-myelin-gene-regulatory-factor
#7
Xiangkai Zhen, Bowen Li, Fen Hu, Shufeng Yan, Gabriele Meloni, Huiliang Li, Ning Shi
Myelin-gene Regulatory Factor (MyRF) is one of the master transcription factors controlling myelin formation and development in oligodendrocytes which is crucial for the powerful brain functions. The N-terminal of MyRF, which contains a proline-rich region and a DNA binding domain (DBD), is auto-cleaved from the ER membrane, and then enters the nucleus to participate in transcription regulation of the myelin genes. Here we report the crystal structure of MyRF DBD. It shows an Ig-fold like architecture which consists of two antiparallel β-sheets with 7 main strands, packing against each other, forming a β-sandwich...
June 16, 2017: Scientific Reports
https://www.readbyqxmd.com/read/28441531/myrf-er-bound-transcription-factors-drive-c-elegans-synaptic-plasticity-via-cleavage-dependent-nuclear-translocation
#8
Jun Meng, Xiaoxia Ma, Huaping Tao, Xia Jin, Daniel Witvliet, James Mitchell, Ming Zhu, Meng-Qiu Dong, Mei Zhen, Yishi Jin, Yingchuan B Qi
Synaptic refinement is a critical step in nervous system maturation, requiring a carefully timed reorganization and refinement of neuronal connections. We have identified myrf-1 and myrf-2, two C. elegans homologs of Myrf family transcription factors, as key regulators of synaptic rewiring. MYRF-1 and its paralog MYRF-2 are functionally redundant specifically in synaptic rewiring. They co-exist in the same protein complex and act cooperatively to regulate synaptic rewiring. We find that the MYRF proteins localize to the ER membrane and that they are cleaved into active N-terminal fragments, which then translocate into the nucleus to drive synaptic rewiring...
April 24, 2017: Developmental Cell
https://www.readbyqxmd.com/read/28441525/myrfs-on-the-move-to-rewire-circuits
#9
REVIEW
Colleen N McLaughlin, Heather T Broihier
Synaptic plasticity occurs in response to intrinsic and extrinsic cues and is a key step in the formation of mature neuronal circuits. In this issue of Developmental Cell, Meng et al. (2017) find that two conserved Myrf transcription factors coexist in the same complex to promote developmental circuit remodeling.
April 24, 2017: Developmental Cell
https://www.readbyqxmd.com/read/28334935/genome-wide-association-study-of-iron-traits-and-relation-to-diabetes-in-the-hispanic-community-health-study-study-of-latinos-hchs-sol-potential-genomic-intersection-of-iron-and-glucose-regulation
#10
Laura M Raffield, Tin Louie, Tamar Sofer, Deepti Jain, Eli Ipp, Kent D Taylor, George J Papanicolaou, Larissa Avilés-Santa, Leslie A Lange, Cathy C Laurie, Matthew P Conomos, Timothy A Thornton, Yii-Der Ida Chen, Qibin Qi, Scott Cotler, Bharat Thyagarajan, Neil Schneiderman, Jerome I Rotter, Alex P Reiner, Henry J Lin
Genetic variants contribute to normal variation of iron-related traits and may also cause clinical syndromes of iron deficiency or excess. Iron overload and deficiency can adversely affect human health. For example, elevated iron storage is associated with increased diabetes risk, although mechanisms are still being investigated. We conducted the first genome-wide association study of serum iron, total iron binding capacity (TIBC), transferrin saturation, and ferritin in a Hispanic/Latino cohort, the Hispanic Community Health Study/Study of Latinos (>12 000 participants) and also assessed the generalization of previously known loci to this population...
May 15, 2017: Human Molecular Genetics
https://www.readbyqxmd.com/read/28193689/signaling-by-fgf-receptor-2-not-fgf-receptor-1-regulates-myelin-thickness-through-activation-of-erk1-2-mapk-which-promotes-mtorc1-activity-in-an-akt-independent-manner
#11
Miki Furusho, Akihiro Ishii, Rashmi Bansal
FGF signaling has emerged as a significant "late-stage" regulator of myelin thickness in the CNS, independent of oligodendrocyte differentiation. Therefore, it is critically important to identify the specific FGF receptor type and its downstream signaling molecules in oligodendrocytes to obtain better insights into the regulatory mechanisms of myelin growth. Here, we show that FGF receptor type 2 (FGFR2) is highly enriched at the paranodal loops of myelin. Conditional ablation of this receptor-type, but not FGF receptor type 1 (FGFR1), resulted in attenuation of myelin growth, expression of major myelin genes, key transcription factor Myrf and extracellular signal-regulated protein kinase 1 and 2 (ERK1/2) activity...
March 15, 2017: Journal of Neuroscience: the Official Journal of the Society for Neuroscience
https://www.readbyqxmd.com/read/28160598/homo-trimerization-is-essential-for-the-transcription-factor-function-of-myrf-for-oligodendrocyte-differentiation
#12
Dongkyeong Kim, Jin-Ok Choi, Chuandong Fan, Randall S Shearer, Mohamed Sharif, Patrick Busch, Yungki Park
Myrf is a key transcription factor for oligodendrocyte differentiation and central nervous system myelination. We and others have previously shown that Myrf is generated as a membrane protein in the endoplasmic reticulum (ER), and that it undergoes auto-processing to release its N-terminal fragment from the ER, which enters the nucleus to work as a transcription factor. These previous studies allow a glimpse into the unusual complexity behind the biogenesis and function of the transcription factor domain of Myrf...
May 19, 2017: Nucleic Acids Research
https://www.readbyqxmd.com/read/27891578/dual-specificity-phosphatase-15-regulates-erk-activation-in-schwann-cells
#13
José F Rodríguez-Molina, Camila Lopez-Anido, Ki H Ma, Chongyu Zhang, Tyler Olson, Katharina N Muth, Matthias Weider, John Svaren
Schwann cells and oligodendrocytes are the myelinating cells of the peripheral and central nervous system, respectively. Despite having different myelin components and different transcription factors driving their terminal differentiation there are shared molecular mechanisms between the two. Sox10 is one common transcription factor required for several steps in development of myelinating glia. However, other factors are divergent as Schwann cells need the transcription factor early growth response 2/Krox20 and oligodendrocytes require Myrf...
February 2017: Journal of Neurochemistry
https://www.readbyqxmd.com/read/27532821/the-dual-specificity-phosphatase-dusp15-is-regulated-by-sox10-and-myrf-in-myelinating-oligodendrocytes
#14
Katharina N Muth, Sandra Piefke, Matthias Weider, Elisabeth Sock, Irm Hermans-Borgmeyer, Michael Wegner, Melanie Küspert
Differentiation of oligodendrocytes and myelin production in the vertebrate central nervous system require highly concerted changes in gene expression. The transcription factors Sox10 and Myrf are both central to this process and jointly regulate expression of myelin genes. Here we show that Sox10 and Myrf also cooperate in the activation of the gene coding for the dual specificity protein phosphatase Dusp15 (also known as VHY) during this process. Activation is mediated by the Dusp15 promoter, which is also sufficient to drive oligodendroglial gene expression in vivo...
December 2016: Glia
https://www.readbyqxmd.com/read/27455109/rapid-production-of-new-oligodendrocytes-is-required-in-the-earliest-stages-of-motor-skill-learning
#15
Lin Xiao, David Ohayon, Ian A McKenzie, Alexander Sinclair-Wilson, Jordan L Wright, Alexander D Fudge, Ben Emery, Huiliang Li, William D Richardson
We identified mRNA encoding the ecto-enzyme Enpp6 as a marker of newly forming oligodendrocytes, and used Enpp6 in situ hybridization to track oligodendrocyte differentiation in adult mice as they learned a motor skill (running on a wheel with unevenly spaced rungs). Within just 2.5 h of exposure to the complex wheel, production of Enpp6-expressing immature oligodendrocytes was accelerated in subcortical white matter; within 4 h, it was accelerated in motor cortex. Conditional deletion of myelin regulatory factor (Myrf) in oligodendrocyte precursors blocked formation of new Enpp6(+) oligodendrocytes and impaired learning within the same ∼2-3 h time frame...
September 2016: Nature Neuroscience
https://www.readbyqxmd.com/read/26980732/genomic-markers-of-panitumumab-resistance-including-erbb2-her2-in-a-phase-ii-study-of-kras-wild-type-wt-metastatic-colorectal-cancer-mcrc
#16
Garrett S Barry, Maggie C Cheang, Hector Li Chang, Hagen F Kennecke
A prospective study was conducted to identify biomarkers associated with resistance to panitumumab monotherapy in patients with metastatic colorectal cancer (mCRC). Patients with previously treated, codon 12/13 KRAS wt, mCRC were prospectively administered panitumumab 6 mg/kg IV q2weeks. Of 34 panitumumab-treated patients, 11 (32%) had progressive disease at 8 weeks and were classified as non-responders. A Nanostring nCounter-based assay identified a 5-gene expression signature (ERBB2, MLPH, IRX3, MYRF, and KLK6) associated with panitumumab resistance (P = 0...
April 5, 2016: Oncotarget
https://www.readbyqxmd.com/read/26474911/evolution-of-the-cns-myelin-gene-regulatory-program
#17
REVIEW
Huiliang Li, William D Richardson
Myelin is a specialized subcellular structure that evolved uniquely in vertebrates. A myelinated axon conducts action potentials many times faster than an unmyelinated axon of the same diameter; for the same conduction speed, the unmyelinated axon would need a much larger diameter and volume than its myelinated counterpart. Hence myelin speeds information transfer and saves space, allowing the evolution of a powerful yet portable brain. Myelination in the central nervous system (CNS) is controlled by a gene regulatory program that features a number of master transcriptional regulators including Olig1, Olig2 and Myrf...
June 15, 2016: Brain Research
https://www.readbyqxmd.com/read/25974668/differential-sox10-genomic-occupancy-in-myelinating-glia
#18
Camila Lopez-Anido, Guannan Sun, Matthias Koenning, Rajini Srinivasan, Holly A Hung, Ben Emery, Sunduz Keles, John Svaren
Myelin is formed by specialized myelinating glia: oligodendrocytes and Schwann cells in the central and peripheral nervous systems, respectively. While there are distinct developmental aspects and regulatory pathways in these two cell types, myelination in both systems requires the transcriptional activator Sox10. Sox10 interacts with cell type-specific transcription factors at some loci to induce myelin gene expression, but it is largely unknown how Sox10 transcriptional networks globally compare between oligodendrocytes and Schwann cells...
May 14, 2015: Glia
https://www.readbyqxmd.com/read/25789755/mutant-huntingtin-downregulates-myelin-regulatory-factor-mediated-myelin-gene-expression-and-affects-mature-oligodendrocytes
#19
Brenda Huang, WenJie Wei, Guohao Wang, Marta A Gaertig, Yue Feng, Wei Wang, Xiao-Jiang Li, Shihua Li
Growing evidence indicates that non-neuronal mutant huntingtin toxicity plays an important role in Huntington's disease (HD); however, whether and how mutant huntingtin affects oligodendrocytes, which are vitally important for neural function and axonal integrity, remains unclear. We first verified the presence of mutant huntingtin in oligodendrocytes in HD140Q knockin mice. We then established transgenic mice (PLP-150Q) that selectively express mutant huntingtin in oligodendrocytes. PLP-150Q mice show progressive neurological symptoms and early death, as well as age-dependent demyelination and reduced expression of myelin genes that are downstream of myelin regulatory factor (MYRF or MRF), a transcriptional regulator that specifically activates and maintains the expression of myelin genes in mature oligodendrocytes...
March 18, 2015: Neuron
https://www.readbyqxmd.com/read/25680202/elevated-in-vivo-levels-of-a-single-transcription-factor-directly-convert-satellite-glia-into-oligodendrocyte-like-cells
#20
Matthias Weider, Amélie Wegener, Christian Schmitt, Melanie Küspert, Simone Hillgärtner, Michael R Bösl, Irm Hermans-Borgmeyer, Brahim Nait-Oumesmar, Michael Wegner
Oligodendrocytes are the myelinating glia of the central nervous system and ensure rapid saltatory conduction. Shortage or loss of these cells leads to severe malfunctions as observed in human leukodystrophies and multiple sclerosis, and their replenishment by reprogramming or cell conversion strategies is an important research aim. Using a transgenic approach we increased levels of the transcription factor Sox10 throughout the mouse embryo and thereby prompted Fabp7-positive glial cells in dorsal root ganglia of the peripheral nervous system to convert into cells with oligodendrocyte characteristics including myelin gene expression...
February 2015: PLoS Genetics
keyword
keyword
92663
1
2
Fetch more papers »
Fetching more papers... Fetching...
Read by QxMD. Sign in or create an account to discover new knowledge that matter to you.
Remove bar
Read by QxMD icon Read
×

Search Tips

Use Boolean operators: AND/OR

diabetic AND foot
diabetes OR diabetic

Exclude a word using the 'minus' sign

Virchow -triad

Use Parentheses

water AND (cup OR glass)

Add an asterisk (*) at end of a word to include word stems

Neuro* will search for Neurology, Neuroscientist, Neurological, and so on

Use quotes to search for an exact phrase

"primary prevention of cancer"
(heart or cardiac or cardio*) AND arrest -"American Heart Association"