Myelin Proteolipid Protein
Myelin Proteins
Pelizaeus-Merzbacher Disease
Myelin Sheath
Diffuse Cerebral Sclerosis of Schilder
Proteolipids
Oligodendroglia
Myelin Basic Protein
Encephalomyelitis, Autoimmune, Experimental
Palmitoyl Coenzyme A
Multiple Sclerosis
Myelin and Lymphocyte-Associated Proteolipid Proteins
Peptide Fragments
Brain
Molecular Sequence Data
Amino Acid Sequence
Apoproteins
Myelin-Associated Glycoprotein
Myelin P0 Protein
Demyelinating Diseases
Central Nervous System
Proteolipid protein gene product can be secreted and exhibit biological activity during early development. (1/415)
A gene encoding myelin proteolipid protein (PLP) and its smaller isoform DM20 is expressed at least 1 week before myelination. Mutations within the gene cause abnormalities in the development of premyelinating oligodendrocytes, resulting in hypomyelinating disorders. These findings suggest a premyelinating function of the PLP gene products. We previously demonstrated that PLP gene expression is directly associated with secretion of a factor that increases the number of oligodendrocytes. Here we show that this activity is mediated by a secreted fragment containing the C-terminal portion of PLP. This factor increased the bromodeoxyuridine incorporation rate in both oligodendrocyte and astrocyte lineage cells; a synthetic peptide (PLP 215-232) exhibited a similar activity. Dose-response curves of PLP and PLP peptide showed maximum activities at a concentration in the picomolar range, which decreased at higher concentrations. These observations demonstrate that a secreted PLP gene product exerts biological activity at a premyelinating stage before the major induction of the gene. (+info)Suppressive immunization with DNA encoding a self-peptide prevents autoimmune disease: modulation of T cell costimulation. (2/415)
Usually we rely on vaccination to promote an immune response to a pathogenic microbe. In this study, we demonstrate a suppressive from of vaccination, with DNA encoding a minigene for residues 139-151 of myelin proteolipid protein (PLP139-151), a pathogenic self-Ag. This suppressive vaccination attenuates a prototypic autoimmune disease, experimental autoimmune encephalomyelitis, which presents clinically with paralysis. Proliferative responses and production of the Th1 cytokines, IL-2 and IFN-gamma, were reduced in T cells responsive to PLP139-151. In the brains of mice that were successfully vaccinated, mRNA for IL-2, IL-15, and IFN-gamma were reduced. A mechanism underlying the reduction in severity and incidence of paralytic autoimmune disease and the reduction in Th1 cytokines involves altered costimulation of T cells; loading of APCs with DNA encoding PLP139-151 reduced the capacity of a T cell line reactive to PLP139-151 to proliferate even in the presence of exogenous CD28 costimulation. DNA immunization with the myelin minigene for PLP-altered expression of B7.1 (CD80), and B7.2 (CD86) on APCs in the spleen. Suppressive immunization against self-Ags encoded by DNA may be exploited to treat autoimmune diseases. (+info)Spontaneous regression of primary autoreactivity during chronic progression of experimental autoimmune encephalomyelitis and multiple sclerosis. (3/415)
Experimental autoimmune encephalomyelitis (EAE) is a widely used animal model for multiple sclerosis (MS). EAE is typically initiated by CD4(+) T helper cell type 1 (Th1) autoreactivity directed against a single priming immunodominant myelin peptide determinant. Recent studies have shown that clinical progression of EAE involves the accumulation of neo-autoreactivity, commonly referred to as epitope spreading, directed against peptide determinants not involved in the priming process. This study directly addresses the relative roles of primary autoreactivity and secondary epitope spreading in the progression of both EAE and MS. To this end we serially evaluated the development of several epitope-spreading cascades in SWXJ mice primed with distinctly different encephalitogenic determinants of myelin proteolipid protein. In a series of analogous experiments, we examined the development of epitope spreading in patients with isolated monosymptomatic demyelinating syndrome as their disease progressed to clinically definite MS. Our results indicate that in both EAE and MS, primary proliferative autoreactivity associated with onset of clinical disease invariably regresses with time and is often undetectable during periods of disease progression. In contrast, the emergence of sustained secondary autoreactivity to spreading determinants is consistently associated with disease progression in both EAE and MS. Our results indicate that chronic progression of EAE and MS involves a shifting of autoreactivity from primary initiating self-determinants to defined cascades of secondary determinants that sustain the self-recognition process during disease progression. (+info)Programmed cell death without DNA fragmentation in the jimpy mouse: secreted factors can enhance survival. (4/415)
Jimpy is one of many related mutations affecting the myelin proteolipid protein gene that causes severe hypomyelination in the central nervous system (CNS). Underlying the hypomyelination is a failure of oligodendrocytes (OLs) to differentiate, and the premature death of large numbers of OLs during the developmental period. Previous light and electron microscopic evidence suggested that jimpy OLs die in a manner consistent with programmed cell death. We have used TUNEL staining as a biochemical marker for apoptosis in conjunction with immunostaining for OL and myelin markers. At 13 - 14 days postnatal, a time when the number of dying OLs in jimpy CNS is increased more than five times normal, there are only modest increases (70% in spinal cord; 20% in cerebral cortex) in TUNEL labeled cells in mutant CNS tissues. The results in vitro are similar, and only a small per cent of TUNEL labeled cells have the antigenic phenotype of OLs. The discrepancy between numbers of dying and TUNEL labeled cells suggests either that most jimpy OLs do not undergo programmed cell death or that the biochemical pathways leading to their death do not involve DNA fragmentation which is detected by the TUNEL method. We also present evidence that jimpy OLs show increased survival and enhanced differentiation when they are grown in vitro in medium conditioned by cells lines which express products of the proteolipid protein gene. Cell lines expressing proteolipid protein and the alternatively spliced DM20 protein have differential effects on cell numbers and production of myelin-like membranes. (+info)Changes in the strength of co-stimulation through the B7/CD28 pathway alter functional T cell responses to altered peptide ligands. (5/415)
T cells require a TCR and a co-stimulatory signal for activation. We have examined the effect of the strength of TCR and co-stimulatory signals on proliferation and production of cytokines by differentiated T cell clones. The TCR signal was varied using antigen dose and altered peptide ligands. The co-stimulatory signal was varied by using as antigen-presenting cells, Chinese hamster ovary cell transfectants that express different levels of the B7-1 molecule with similar levels of MHC class II. Our results show that the level of co-stimulation has a profound effect on the response to an antigen, and that a strong co-stimulatory signal can convert a weak agonist into a full agonist and an agonist into a superagonist. Antigenicity is not absolute but a function of the strengths of the TCR and co-stimulatory signals. Increasing the strength of co-stimulation can lower antigen concentration required for maximal proliferative responses by T cell clones by 5 log. These results show that the level of expression of co-stimulatory molecules will profoundly regulate T cell clonal expansion and effector functions. (+info)Embryonic stem cell-derived glial precursors: a source of myelinating transplants. (6/415)
Self-renewing, totipotent embryonic stem (ES) cells may provide a virtually unlimited donor source for transplantation. A protocol that permits the in vitro generation of precursors for oligodendrocytes and astrocytes from ES cells was devised. Transplantation in a rat model of a human myelin disease shows that these ES cell-derived precursors interact with host neurons and efficiently myelinate axons in brain and spinal cord. Thus, ES cells can serve as a valuable source of cell type-specific somatic precursors for neural transplantation. (+info)Serial MR imaging of experimental autoimmune encephalomyelitis induced by human white matter or by chimeric myelin-basic and proteolipid protein in the common marmoset. (7/415)
BACKGROUND AND PURPOSE: Experimental autoimmune encephalomyelitis (EAE) in the marmoset was monitored by serial MR imaging to determine correlates to the natural-history MR studies in multiple sclerosis (MS). The relationships of MR-revealed lesions to clinical status and histopathologic findings were also explored. METHODS: We induced EAE by subcutaneous inoculation in two marmosets by human white matter (HWM) and in seven marmosets by MP4 (a chimeric recombinant fusion protein of myelin-basic and proteolipid protein) in adjuvant along with intravenous inactivated pertussis vaccine to facilitate the disease process. The HWM-inoculated animals were induced with Freund's adjuvant as the established model of marmoset EAE. The MP4-inoculated animals were induced with either Freund's incomplete adjuvant or TiterMax as part of a preclinical treatment trial. MR imaging was performed at 1.5 T at baseline, and repeated at 1- to 2-week intervals for a period of up to 16 weeks in six EAE-induced marmosets, and intermittently for up to 70 weeks in three EAE-induced and two control marmosets. Proton density- (PD-) and T2-weighted, pre- and postgadopentetate dimeglumine enhancement, T1-weighted, and magnetization transfer (MT) images were obtained. The brains were prepared for histologic evaluation of lesion distribution and counts, characterization of lesions as demyelinating or inflammatory, and histopathologic scoring. The clinical, MR, and pathologic scoring were done on grading systems, and correlated for evaluation. RESULTS: White matter (WM) changes after EAE induction were observed first at 9 days in the HWM-induced animals and at 2.5 weeks in the MP4-induced animals, with subsequent week-to-week fluctuations on PD- and T2-weighted images. Contrast-enhancing lesions were not observed in all animals. MR-revealed WM lesions correlated to histopathologic analysis of EAE lesions, measuring from 0.5 mm to 1.5 mm. The lesion count and extent of demyelination was greater in the HWM-induced animals than in the MP4-induced animals. Some MR-revealed lesions correlated directly to clinical symptoms, but the majority of lesions were clinically silent. CONCLUSION: On MR images, lesions in the EAE marmoset model were confined to the WM, and their development, resolution, distribution, and enhancing characteristics fluctuated over the duration of the study. The dynamic presentation of MR-revealed lesions confirms the parallels between EAE in the marmoset and relapsing-remitting MS. Clinical symptoms alone were not representative of ongoing pathologic brain lesions. Therefore, serial MR imaging serves as a very important adjunct to clinical and histologic surveillance of the development of new and the persistence of existing brain lesions in this animal model of MS. (+info)Endogenous presentation of self myelin epitopes by CNS-resident APCs in Theiler's virus-infected mice. (8/415)
The mechanisms underlying the initiation of virus-induced autoimmune disease are not well understood. Theiler's murine encephalomyelitis virus-induced demyelinating disease (TMEV-IDD), a mouse model of multiple sclerosis, is initiated by TMEV-specific CD4(+) T cells targeting virally infected central nervous system-resident (CNS-resident) antigen-presenting cells (APCs), leading to chronic activation of myelin epitope-specific CD4(+) T cells via epitope spreading. Here we show that F4/80(+), I-A(s+), CD45(+) macrophages/microglia isolated from the CNS of TMEV-infected SJL mice have the ability to endogenously process and present virus epitopes at both acute and chronic stages of the disease. Relevant to the initiation of virus-induced autoimmune disease, only CNS APCs isolated from TMEV-infected mice with preexisting myelin damage, not those isolated from naive mice or mice with acute disease, were able to endogenously present a variety of proteolipid protein epitopes to specific Th1 lines. These results offer a mechanism by which localized virus-induced, T cell-mediated inflammatory myelin destruction leads to the recruitment/activation of CNS-resident APCs that can process and present endogenous self epitopes to autoantigen-specific T cells, and thus provide a mechanistic basis by which epitope spreading occurs. (+info)Myelin Proteolipid Protein (PLP) is a major component of the myelin sheath, which is a fatty insulating substance that covers and protects nerve fibers in the central nervous system (CNS). PLP makes up about 50% of the proteins found in the myelin sheath. It plays a crucial role in the structure and function of the myelin sheath, including maintaining its compactness and stability. Defects or mutations in the gene that encodes for PLP can lead to various demyelinating diseases, such as X-linked adrenoleukodystrophy (X-ALD) and Pelizaeus-Merzbacher disease (PMD), which are characterized by the degeneration of the myelin sheath and subsequent neurological impairments.
I'm sorry for any confusion, but "Jimpy" is not a recognized medical term or condition associated with mice in the field of veterinary medicine. It may be a colloquial or informal term used to describe a particular characteristic or behavior in mice, but without further context, it's challenging to provide an accurate definition. If you could provide more information about where you encountered this term or its intended meaning, I would be happy to help you further.
Myelin proteins are proteins that are found in the myelin sheath, which is a fatty (lipid-rich) substance that surrounds and insulates nerve fibers (axons) in the nervous system. The myelin sheath enables the rapid transmission of electrical signals (nerve impulses) along the axons, allowing for efficient communication between different parts of the nervous system.
There are several types of myelin proteins, including:
1. Proteolipid protein (PLP): This is the most abundant protein in the myelin sheath and plays a crucial role in maintaining the structure and function of the myelin sheath.
2. Myelin basic protein (MBP): This protein is also found in the myelin sheath and helps to stabilize the compact structure of the myelin sheath.
3. Myelin-associated glycoprotein (MAG): This protein is involved in the adhesion of the myelin sheath to the axon and helps to maintain the integrity of the myelin sheath.
4. 2'3'-cyclic nucleotide 3' phosphodiesterase (CNP): This protein is found in oligodendrocytes, which are the cells that produce the myelin sheath in the central nervous system. CNP plays a role in maintaining the structure and function of the oligodendrocytes.
Damage to myelin proteins can lead to demyelination, which is a characteristic feature of several neurological disorders, including multiple sclerosis (MS), Guillain-Barré syndrome, and Charcot-Marie-Tooth disease.
Pelizaeus-Merzbacher disease (PMD) is a rare X-linked recessive genetic disorder affecting the nervous system. It is caused by mutations in the PLP1 gene, which provides instructions for making proteins that are important for the formation and maintenance of the myelin sheath, the protective covering that wraps around nerve cell fibers (axons) in the brain and spinal cord to ensure efficient transmission of electrical signals.
In individuals with PMD, the myelin sheath is either partially or completely absent, leading to progressive neurological symptoms. The classic form of PMD is characterized by early onset of nystagmus (involuntary eye movements), ataxia (loss of muscle coordination and balance), and intellectual disability. Other features may include hypotonia (low muscle tone), spasticity (stiff or rigid muscles), and seizures. The severity and progression of the disease can vary widely among affected individuals, ranging from a severe, lethal form to a milder form with a slower disease course.
Currently, there is no cure for PMD, and treatment is focused on managing symptoms and improving quality of life.
The myelin sheath is a multilayered, fatty substance that surrounds and insulates many nerve fibers in the nervous system. It is essential for the rapid transmission of electrical signals, or nerve impulses, along these nerve fibers, allowing for efficient communication between different parts of the body. The myelin sheath is produced by specialized cells called oligodendrocytes in the central nervous system (CNS) and Schwann cells in the peripheral nervous system (PNS). Damage to the myelin sheath, as seen in conditions like multiple sclerosis, can significantly impair nerve function and result in various neurological symptoms.
Diffuse cerebral sclerosis of Schilder, also known as Schilder's disease, is a rare inflammatory demyelinating disorder of the central nervous system. It primarily affects children and young adults, but can occur at any age. The condition is characterized by widespread destruction of the myelin sheath, which surrounds and protects nerve fibers in the brain.
The hallmark feature of Schilder's disease is the presence of multiple, large, symmetrical lesions in the white matter of both cerebral hemispheres. These lesions are typically located in the parieto-occipital regions of the brain and can extend to involve other areas as well.
The symptoms of Schilder's disease vary depending on the location and extent of the lesions, but may include:
* Progressive intellectual decline
* Seizures
* Visual disturbances
* Weakness or paralysis on one side of the body (hemiparesis)
* Loss of sensation in various parts of the body
* Speech difficulties
* Behavioral changes, such as irritability, mood swings, and depression
The exact cause of Schilder's disease is not known, but it is believed to be an autoimmune disorder, in which the body's own immune system mistakenly attacks the myelin sheath. There is no cure for Schilder's disease, and treatment typically involves corticosteroids or other immunosuppressive therapies to reduce inflammation and slow the progression of the disease. Despite treatment, many patients with Schilder's disease experience significant disability and may require long-term care.
Proteolipids are a type of complex lipid-containing proteins that are insoluble in water and have a high content of hydrophobic amino acids. They are primarily found in the plasma membrane of cells, where they play important roles in maintaining the structural integrity and function of the membrane. Proteolipids are also found in various organelles, including mitochondria, lysosomes, and peroxisomes.
Proteolipids are composed of a hydrophobic protein core that is tightly associated with a lipid bilayer through non-covalent interactions. The protein component of proteolipids typically contains several transmembrane domains that span the lipid bilayer, as well as hydrophilic regions that face the cytoplasm or the lumen of organelles.
Proteolipids have been implicated in various cellular processes, including signal transduction, membrane trafficking, and ion transport. They are also associated with several neurological disorders, such as Alzheimer's disease, Parkinson's disease, and multiple sclerosis. The study of proteolipids is an active area of research in biochemistry and cell biology, with potential implications for the development of new therapies for neurological disorders.
Oligodendroglia are a type of neuroglial cell found in the central nervous system (CNS) of vertebrates, including humans. These cells play a crucial role in providing support and insulation to nerve fibers (axons) in the CNS, which includes the brain and spinal cord.
More specifically, oligodendroglia produce a fatty substance called myelin that wraps around axons, forming myelin sheaths. This myelination process helps to increase the speed of electrical impulse transmission (nerve impulses) along the axons, allowing for efficient communication between different neurons.
In addition to their role in myelination, oligodendroglia also contribute to the overall health and maintenance of the CNS by providing essential nutrients and supporting factors to neurons. Dysfunction or damage to oligodendroglia has been implicated in various neurological disorders, such as multiple sclerosis (MS), where demyelination of axons leads to impaired nerve function and neurodegeneration.
Myelin Basic Protein (MBP) is a key structural protein found in the myelin sheath, which is a multilayered membrane that surrounds and insulates nerve fibers (axons) in the nervous system. The myelin sheath enables efficient and rapid transmission of electrical signals (nerve impulses) along the axons, allowing for proper communication between different neurons.
MBP is one of several proteins responsible for maintaining the structural integrity and organization of the myelin sheath. It is a basic protein, meaning it has a high isoelectric point due to its abundance of positively charged amino acids. MBP is primarily located in the intraperiod line of the compact myelin, which is a region where the extracellular leaflets of the apposing membranes come into close contact without fusing.
MBP plays crucial roles in the formation, maintenance, and repair of the myelin sheath:
1. During development, MBP helps mediate the compaction of the myelin sheath by interacting with other proteins and lipids in the membrane.
2. MBP contributes to the stability and resilience of the myelin sheath by forming strong ionic bonds with negatively charged phospholipids in the membrane.
3. In response to injury or disease, MBP can be cleaved into smaller peptides that act as chemoattractants for immune cells, initiating the process of remyelination and repair.
Dysregulation or damage to MBP has been implicated in several demyelinating diseases, such as multiple sclerosis (MS), where the immune system mistakenly attacks the myelin sheath, leading to its degradation and loss. The presence of autoantibodies against MBP is a common feature in MS patients, suggesting that an abnormal immune response to this protein may contribute to the pathogenesis of the disease.
Autoimmune encephalomyelitis (EAE) is a model of inflammatory demyelinating disease used in medical research to study the mechanisms of multiple sclerosis (MS) and develop new therapies. It is experimentally induced in laboratory animals, typically mice or rats, through immunization with myelin antigens or T-cell transfer. The resulting immune response leads to inflammation, demyelination, and neurological dysfunction in the central nervous system (CNS), mimicking certain aspects of MS.
EAE is a valuable tool for understanding the pathogenesis of MS and testing potential treatments. However, it is essential to recognize that EAE is an experimental model and may not fully recapitulate all features of human autoimmune encephalomyelitis.
Neurologic mutant mice are genetically engineered or spontaneously mutated rodents that are used as models to study various neurological disorders and conditions. These mice have specific genetic modifications or mutations that affect their nervous system, leading to phenotypes that resemble human neurological diseases.
Some examples of neurologic mutant mice include:
1. Alzheimer's disease models: Mice that overexpress genes associated with Alzheimer's disease, such as the amyloid precursor protein (APP) or presenilin 1 (PS1), to study the pathogenesis and potential treatments of this disorder.
2. Parkinson's disease models: Mice that have genetic mutations in genes associated with Parkinson's disease, such as alpha-synuclein or parkin, to investigate the mechanisms underlying this condition and develop new therapies.
3. Huntington's disease models: Mice that carry an expanded CAG repeat in the huntingtin gene to replicate the genetic defect seen in humans with Huntington's disease and study disease progression and treatment strategies.
4. Epilepsy models: Mice with genetic mutations that cause spontaneous seizures or increased susceptibility to seizures, used to investigate the underlying mechanisms of epilepsy and develop new treatments.
5. Stroke models: Mice that have surgical induction of stroke or genetic modifications that increase the risk of stroke, used to study the pathophysiology of stroke and identify potential therapeutic targets.
Neurologic mutant mice are essential tools in biomedical research, allowing scientists to investigate the complex interactions between genes and the environment that contribute to neurological disorders. These models help researchers better understand disease mechanisms, develop new therapies, and test their safety and efficacy before moving on to clinical trials in humans.
Palmitoyl Coenzyme A, often abbreviated as Palmitoyl-CoA, is a type of fatty acyl coenzyme A that plays a crucial role in the body's metabolism. It is formed from the esterification of palmitic acid (a saturated fatty acid) with coenzyme A.
Medical Definition: Palmitoyl Coenzyme A is a fatty acyl coenzyme A ester, where palmitic acid is linked to coenzyme A via an ester bond. It serves as an important intermediate in lipid metabolism and energy production, particularly through the process of beta-oxidation in the mitochondria. Palmitoyl CoA also plays a role in protein modification, known as S-palmitoylation, which can affect protein localization, stability, and function.
Acylation is a medical and biological term that refers to the process of introducing an acyl group (-CO-) into a molecule. This process can occur naturally or it can be induced through chemical reactions. In the context of medicine and biology, acylation often occurs during post-translational modifications of proteins, where an acyl group is added to specific amino acid residues, altering the protein's function, stability, or localization.
An example of acylation in medicine is the administration of neuraminidase inhibitors, such as oseltamivir (Tamiflu), for the treatment and prevention of influenza. These drugs work by inhibiting the activity of the viral neuraminidase enzyme, which is essential for the release of newly formed virus particles from infected cells. Oseltamivir is administered orally as an ethyl ester prodrug, which is then hydrolyzed in the body to form the active acylated metabolite that inhibits the viral neuraminidase.
In summary, acylation is a vital process in medicine and biology, with implications for drug design, protein function, and post-translational modifications.
Multiple Sclerosis (MS) is a chronic autoimmune disease that affects the central nervous system (CNS), which includes the brain, spinal cord, and optic nerves. In MS, the immune system mistakenly attacks the protective covering of nerve fibers, called myelin, leading to damage and scarring (sclerosis). This results in disrupted communication between the brain and the rest of the body, causing a variety of neurological symptoms that can vary widely from person to person.
The term "multiple" refers to the numerous areas of scarring that occur throughout the CNS in this condition. The progression, severity, and specific symptoms of MS are unpredictable and may include vision problems, muscle weakness, numbness or tingling, difficulty with balance and coordination, cognitive impairment, and mood changes. There is currently no cure for MS, but various treatments can help manage symptoms, modify the course of the disease, and improve quality of life for those affected.
Myelin and lymphocyte-associated proteolipid proteins (MAL/PLP) are a family of proteolipid proteins that play crucial roles in the formation and maintenance of the myelin sheath in the central nervous system (CNS). The myelin sheath is a multilayered membrane that surrounds nerve cell axons, allowing for efficient and rapid electrical impulse transmission.
The MAL/PLP family includes two major proteins:
1. Myelin and lymphocyte protein (MAL): This protein is primarily expressed in the plasma membrane of oligodendrocytes, the CNS glial cells responsible for myelination. MAL is involved in the organization and maintenance of the lipid rafts, which are specialized microdomains within the plasma membrane that facilitate signal transduction and membrane trafficking.
2. Proteolipid protein (PLP) or proteolipid protein 1 (PLP1): This is the most abundant protein in the CNS myelin sheath, constituting approximately 50% of its total protein content. PLP is primarily located within the intracellular leaflets of the multilayered myelin membrane and plays a critical role in maintaining the integrity and compaction of the myelin sheath.
Mutations in the genes encoding these proteins can lead to various demyelinating disorders, such as Pelizaeus-Merzbacher disease (PMD) and spastic paraplegia type 2 (SPG2), which are characterized by abnormalities in the myelin sheath and neurological dysfunction.
A peptide fragment is a short chain of amino acids that is derived from a larger peptide or protein through various biological or chemical processes. These fragments can result from the natural breakdown of proteins in the body during regular physiological processes, such as digestion, or they can be produced experimentally in a laboratory setting for research or therapeutic purposes.
Peptide fragments are often used in research to map the structure and function of larger peptides and proteins, as well as to study their interactions with other molecules. In some cases, peptide fragments may also have biological activity of their own and can be developed into drugs or diagnostic tools. For example, certain peptide fragments derived from hormones or neurotransmitters may bind to receptors in the body and mimic or block the effects of the full-length molecule.
The brain is the central organ of the nervous system, responsible for receiving and processing sensory information, regulating vital functions, and controlling behavior, movement, and cognition. It is divided into several distinct regions, each with specific functions:
1. Cerebrum: The largest part of the brain, responsible for higher cognitive functions such as thinking, learning, memory, language, and perception. It is divided into two hemispheres, each controlling the opposite side of the body.
2. Cerebellum: Located at the back of the brain, it is responsible for coordinating muscle movements, maintaining balance, and fine-tuning motor skills.
3. Brainstem: Connects the cerebrum and cerebellum to the spinal cord, controlling vital functions such as breathing, heart rate, and blood pressure. It also serves as a relay center for sensory information and motor commands between the brain and the rest of the body.
4. Diencephalon: A region that includes the thalamus (a major sensory relay station) and hypothalamus (regulates hormones, temperature, hunger, thirst, and sleep).
5. Limbic system: A group of structures involved in emotional processing, memory formation, and motivation, including the hippocampus, amygdala, and cingulate gyrus.
The brain is composed of billions of interconnected neurons that communicate through electrical and chemical signals. It is protected by the skull and surrounded by three layers of membranes called meninges, as well as cerebrospinal fluid that provides cushioning and nutrients.
Molecular sequence data refers to the specific arrangement of molecules, most commonly nucleotides in DNA or RNA, or amino acids in proteins, that make up a biological macromolecule. This data is generated through laboratory techniques such as sequencing, and provides information about the exact order of the constituent molecules. This data is crucial in various fields of biology, including genetics, evolution, and molecular biology, allowing for comparisons between different organisms, identification of genetic variations, and studies of gene function and regulation.
An amino acid sequence is the specific order of amino acids in a protein or peptide molecule, formed by the linking of the amino group (-NH2) of one amino acid to the carboxyl group (-COOH) of another amino acid through a peptide bond. The sequence is determined by the genetic code and is unique to each type of protein or peptide. It plays a crucial role in determining the three-dimensional structure and function of proteins.
Apoproteins are the protein components of lipoprotein complexes, which are responsible for transporting fat molecules, such as cholesterol and triglycerides, throughout the body. Apoproteins play a crucial role in the metabolism of lipids by acting as recognition signals that allow lipoproteins to interact with specific receptors on cell surfaces.
There are several different types of apoproteins, each with distinct functions. For example, apolipoprotein A-1 (apoA-1) is the major protein component of high-density lipoproteins (HDL), which are responsible for transporting excess cholesterol from tissues to the liver for excretion. Apolipoprotein B (apoB) is a large apoprotein found in low-density lipoproteins (LDL), very low-density lipoproteins (VLDL), and lipoprotein(a). ApoB plays a critical role in the assembly and secretion of VLDL from the liver, and it also mediates the uptake of LDL by cells.
Abnormalities in apoprotein levels or function can contribute to the development of various diseases, including cardiovascular disease, diabetes, and Alzheimer's disease. Therefore, measuring apoprotein levels in the blood can provide valuable information for diagnosing and monitoring these conditions.
Myelin-Associated Glycoprotein (MAG) is a glycoprotein found on the surface of myelin sheaths, which are the protective insulating layers around nerve fibers in the nervous system. MAG plays a role in the adhesion and interaction between the myelin sheath and the axon it surrounds. It's particularly important during the development and maintenance of the nervous system. Additionally, MAG has been implicated in the regulation of neuronal growth and signal transmission. In certain autoimmune diseases like Guillain-Barré syndrome, the immune system may mistakenly attack MAG, leading to damage of the myelin sheath and associated neurological symptoms.
Myelin P0 protein, also known as P0 or MPZ (myelin protein zero), is a major structural component of the myelin sheath in the peripheral nervous system. The myelin sheath is a multilayered membrane that surrounds and insulates nerve fibers to increase the speed of electrical impulse transmission.
P0 protein is a transmembrane glycoprotein, which means it spans the lipid bilayer of the myelin membrane and has sugar molecules (glycans) attached to it. It plays a crucial role in maintaining the compact structure of the myelin sheath by forming homodimers that interact with each other through their extracellular domains, creating tight junctions between the apposing layers of the myelin membrane.
P0 protein also contributes to the stability and integrity of the myelin sheath by interacting with other myelin proteins, such as connexin 32 and peripheral myelin protein 22 (PMP22). Mutations in the MPZ gene can lead to various peripheral neuropathies, including Charcot-Marie-Tooth disease type 1B and Dejerine-Sottas syndrome.
Demyelinating diseases are a group of disorders that are characterized by damage to the myelin sheath, which is the protective covering surrounding nerve fibers in the brain, optic nerves, and spinal cord. Myelin is essential for the rapid transmission of nerve impulses, and its damage results in disrupted communication between the brain and other parts of the body.
The most common demyelinating disease is multiple sclerosis (MS), where the immune system mistakenly attacks the myelin sheath. Other demyelinating diseases include:
1. Acute Disseminated Encephalomyelitis (ADEM): An autoimmune disorder that typically follows a viral infection or vaccination, causing widespread inflammation and demyelination in the brain and spinal cord.
2. Neuromyelitis Optica (NMO) or Devic's Disease: A rare autoimmune disorder that primarily affects the optic nerves and spinal cord, leading to severe vision loss and motor disability.
3. Transverse Myelitis: Inflammation of the spinal cord causing damage to both sides of one level (segment) of the spinal cord, resulting in various neurological symptoms such as muscle weakness, numbness, or pain, depending on which part of the spinal cord is affected.
4. Guillain-Barré Syndrome: An autoimmune disorder that causes rapid-onset muscle weakness, often beginning in the legs and spreading to the upper body, including the face and breathing muscles. It occurs when the immune system attacks the peripheral nerves' myelin sheath.
5. Central Pontine Myelinolysis (CPM): A rare neurological disorder caused by rapid shifts in sodium levels in the blood, leading to damage to the myelin sheath in a specific area of the brainstem called the pons.
These diseases can result in various symptoms, such as muscle weakness, numbness, vision loss, difficulty with balance and coordination, and cognitive impairment, depending on the location and extent of the demyelination. Treatment typically focuses on managing symptoms, modifying the immune system's response, and promoting nerve regeneration and remyelination when possible.
The Central Nervous System (CNS) is the part of the nervous system that consists of the brain and spinal cord. It is called the "central" system because it receives information from, and sends information to, the rest of the body through peripheral nerves, which make up the Peripheral Nervous System (PNS).
The CNS is responsible for processing sensory information, controlling motor functions, and regulating various autonomic processes like heart rate, respiration, and digestion. The brain, as the command center of the CNS, interprets sensory stimuli, formulates thoughts, and initiates actions. The spinal cord serves as a conduit for nerve impulses traveling to and from the brain and the rest of the body.
The CNS is protected by several structures, including the skull (which houses the brain) and the vertebral column (which surrounds and protects the spinal cord). Despite these protective measures, the CNS remains vulnerable to injury and disease, which can have severe consequences due to its crucial role in controlling essential bodily functions.
Autoimmunity is a medical condition in which the body's immune system mistakenly attacks and destroys healthy tissues within the body. In normal function, the immune system recognizes and fights off foreign substances such as bacteria, viruses, and toxins. However, when autoimmunity occurs, the immune system identifies self-molecules or tissues as foreign and produces an immune response against them.
This misguided response can lead to chronic inflammation, tissue damage, and impaired organ function. Autoimmune diseases can affect various parts of the body, including the joints, skin, glands, muscles, and blood vessels. Some common examples of autoimmune diseases are rheumatoid arthritis, lupus, multiple sclerosis, type 1 diabetes, Hashimoto's thyroiditis, and Graves' disease.
The exact cause of autoimmunity is not fully understood, but it is believed to involve a combination of genetic, environmental, and lifestyle factors that trigger an abnormal immune response in susceptible individuals. Treatment for autoimmune diseases typically involves managing symptoms, reducing inflammation, and suppressing the immune system's overactive response using medications such as corticosteroids, immunosuppressants, and biologics.
Myelin proteolipid protein
myelin-proteolipid) O-palmitoyltransferase
Myelin
Proteolipid protein 1
Myelin basic protein
Pelizaeus-Merzbacher disease
Proteolipid
Cyclin-dependent kinase 5
Axon
List of MeSH codes (D10)
Site-specific recombinase technology
Oligodendrocyte progenitor cell
Myelinoid
Palmitoylation
Molecular mimicry
Myelinogenesis
MAL (gene)
Marjorie Lees
Paul J. Tesar
Neurodegenerative disease
Jordi Folch Pi
MYT1
Brian Andrew Hills
MALL
List of EC numbers (EC 2)
X chromosome
Myelin proteolipid protein - Wikipedia
JCI - Induction of circulating myelin basic protein and proteolipid protein-specific transforming growth factor-beta1-secreting...
Myelin Proteolipid Protein (PLP 139-151), 15 mg - MD Bioproducts
Encephalitogenic Myelin Proteolipid Fragment
Myelin Proteolipid Protein (PLP 139-151), 15 mg - MD Bioproducts
Human PLPL(Myelin Proteolipid Protein Like Protein) ELISA Kit - Operatie Biotech Research Purchasing (BRP)
Jimpy mutant mouse: a 74-base deletion in the mRNA for myelin proteolipid protein and evidence for a primary defect in RNA...
![CCDC177 coiled-coil domain containing 177 [Homo sapiens (human)] - Gene - NCBI](data:image/png;base64,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)
CCDC177 coiled-coil domain containing 177 [Homo sapiens (human)] - Gene - NCBI
Atypical retinopathy in patients with nephronophthisis type 1: an uncommon ophthalmological finding
Index by author - October 01, 1991, 11 (10) | Journal of Neuroscience
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PLP1
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Year 1 - CIRM
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SMART: Pfam domain zf-C2HC
SMART: Pfam domain zf-C2HC
Whole exome sequencing in family trios reveals de novo mutations in PURA as a cause of severe neurodevelopmental delay and...
Glycoprotein5
- Purified myelin oligodendrocyte glycoprotein (MOG) for MOG-induced EAE models in mouse and rat. (mdbioproducts.com)
- We have completed the optimization of two models for induction of EAE MS using Proteolipid Protein and Myelin-oligodendrocyte glycoprotein. (ca.gov)
- The former is defined by the presence in macrophages of major and small molecular weight myelin proteins, such as cyclic nucleotide diphosphoesterase (CNP), myelin oligodendrocyte glycoprotein (MOG), or myelin-associated protein (MAG), whereas macrophages in the latter demonstrate merely the presence of the major myelin proteins MBP or PLP. (nih.gov)
- Whole mounts of optic nerve, chiasm, and optic tract were sectioned horizontally and incubated with antibodies to myelin basic protein (MBP), proteolipid protein (PLP), myelin-associated glycoprotein (MAG), "Rip," and the neurite inhibitory protein (IN-1), followed by visualization with diaminobenzidine and a peroxidase-conjugated secondary antibody. (ox.ac.uk)
- We used a novel mouse model (oLucR) expressing luciferase in myelin oligodendrocyte glycoprotein-positive oligodendrocytes under the control of a β-actin promoter. (biomedcentral.com)
PLP16
- Description: A competitive ELISA for quantitative measurement of Human Myelin proteolipid protein(PLP1) in samples from blood, plasma, serum, cell culture supernatant and other biological fluids. (operatiebrp.nl)
- Description: A sandwich quantitative ELISA assay kit for detection of Human Proteolipid Protein 1, Myelin (PLP1) in samples from tissue homogenates, cell lysates or other biological fluids. (operatiebrp.nl)
- [ 1 ] is a congenital hypomyelination disorder caused by changes affecting the proteolipid protein 1 gene (PLP1) located on Xq22.2. (medscape.com)
- Although Pelizaeus-Merzbacher disease and X-linked spastic paraplegia type 2 are nosologically distinguished, they are at opposite ends of a clinical spectrum of X-linked diseases caused by mutations of the same gene, the proteolipid protein 1 ( PLP1 ) gene, and result in defective central nervous system (CNS) myelination (see the image below). (medscape.com)
- Severe clinical syndromes (sometimes referred to as the connatal forms of Pelizaeus-Merzbacher disease) are typically caused by missense and other small mutations that affect critical positions in PLP1 , whereas the milder spastic paraplegia syndrome is caused by mutations that presumably affect less critical regions of the protein. (medscape.com)
- PLP1 encodes 2 major products, PLP1 and a smaller protein, DM20, that results from alternative splicing. (medscape.com)
Antibodies1
- For this classification of MS lesions, identification of myelin with histological stains [such as luxol fast blue-PAS] or by immunohistochemistry using antibodies against myelin basic-protein (MBP) or proteolipid-protein (PLP), as well as, detection of macrophages/microglia by, e.g., anti-CD68 is sufficient. (nih.gov)
Gene7
- The mouse mutant jimpy carries an X chromosome-linked recessive gene defect that affects the formation of myelin in the central nervous system. (mpg.de)
- Developing myelin specific promoters for schwannoma gene therapy. (harvard.edu)
- From NCBI Gene: This gene encodes a transmembrane proteolipid protein that is the predominant component of myelin. (nih.gov)
- The evidence is suggestive that the SPG4 gene regulates production of a protein referred to as, 'spastin. (disabled-world.com)
- The other x-linked form of HSP that has been identified is believed to be caused by mutations in a gene that regulates production of a myelin protein referred to as, 'proteolipid protein (PLP). (disabled-world.com)
- SPG7 has been associated with mutations of a gene which regulates the production of a protein referred to as, 'paraplegin. (disabled-world.com)
- The classic form is X-chromosome linked, has its onset in infancy and is associated with a mutation of the proteolipid protein gene. (uams.edu)
Peripheral2
Compact myelin1
- These proteins constitute about 50% of the mass of CNS white matter and are believed to serve an important structural function in compact myelin. (medscape.com)
Sheath3
- The myelin sheath is a multi-layered membrane, unique to the nervous system, that functions as an insulator to greatly increase the efficiency of axonal impulse conduction. (wikipedia.org)
- Proteins found in the myelin sheath. (harvard.edu)
- Proteolipid protein (PLP) is the most abundant protein in the central nervous system (CNS) myelin sheath and is highly conserved among species. (mobitec.com)
Antibody1
- Description: Enzyme-linked immunosorbent assay based on the Double-antibody Sandwich method for detection of Human Myelin Proteolipid Protein Like Protein (PLPL) in samples from Tissue homogenates and other biological fluids. (operatiebrp.nl)
Transmembrane1
- PLP is a highly conserved hydrophobic protein of 276 to 280 amino acids which seems to contain four transmembrane segments, two disulfide bonds and which covalently binds lipids (at least six palmitate groups in mammals). (wikipedia.org)
Oligodendrocytes4
- During vertebrate brain development, axons are enwrapped by myelin, an insulating membrane produced by oligodendrocytes. (rupress.org)
- In this study, we show that neurons regulate myelin membrane trafficking in oligodendrocytes. (rupress.org)
- ε-Toxin sensitivity is also dependent on oligodendrocyte expression of the proteolipid myelin and lymphocyte protein (MAL), as MAL-deficient oligodendrocytes are insensitive to ε-toxin. (cornell.edu)
- We find that ε-toxin specifically targets the myelin-forming cells of the central nervous system (CNS), oligodendrocytes, leading to cell death. (cornell.edu)
Peptides1
- Today, myelin related proteins or peptides are used in the disease. (mdbioproducts.com)
Highly conserved1
- PURA encodes Pur-α, a highly conserved multifunctional protein that has an important role in normal postnatal brain development in animal models. (bmj.com)
Bovine3
- Specifically, 4,860 short-term T cell lines were generated to either MBP, PLP, or tetanus toxoid (TT) from 34 relapsing-remitting MS patients: 17 orally treated with bovine myelin daily for a minimum of 2 yr as compared to 17 nontreated patients. (jci.org)
- Bovine Collagen Type VI protein purified from human placenta. (mdbioproducts.com)
- Immunization Grade Collagen type II (CII) protein, purified from fetal bovine articular cartilage, for the induction of arthritis in the Collagen-I. (mdbioproducts.com)
Lipid2
- Our study demonstrates that TMEM106B protein abundance is increased with brain ageing in humans, establishes that dementia risk allele rs1990622-A predisposes to TMEM106B fibril formation in the hippocampus, and provides the first evidence that rs1990622-A affects brain lipid homeostasis, particularly myelin lipids. (biomedcentral.com)
- Targeting myelin lipid metabolism as a potential therapeutic strategy in a model of CMT1A neuropathy. (mpg.de)
Immunization1
- In C57BL/6 (B6) mice, EAE can be induced by immunization with antigenic myelin components, or by the adoptive transfer of myelin-specific CD4 + T cells that have been reactivated in vitro ( 1 , 2 , 3 ). (aai.org)
MRNA1
- Jimpy mutant mouse: a 74-base deletion in the mRNA for myelin proteolipid protein and evidence for a primary defect in RNA splicing. (mpg.de)
Demyelinating diseases1
- Microfibrillar-associated protein 4 as a potential marker of acute relapse in inflammatory demyelinating diseases of the central nervous system: Pathological and clinical aspects. (cuanschutz.edu)
MeSH1
- Myelin Proteins" is a descriptor in the National Library of Medicine's controlled vocabulary thesaurus, MeSH (Medical Subject Headings) . (harvard.edu)
Novo2
- Results Protein-altering de novo mutations in PURA were identified in four subjects. (bmj.com)
- Together, these data demonstrate that the inability to process antigenic peptide from intact myelin protein results in resistance to EAE and that de novo processing and presentation of myelin Ags in the CNS is absolutely required for the initiation of autoimmune demyelinating disease. (aai.org)
Lipids1
- Hippocampal lipids were not significantly affected by APOE genotype, however levels of myelin-enriched sulfatides and hexosylceramides were significantly lower, and polyunsaturated phospholipids were higher, in rs1990622-A carriers after controlling for APOE genotype. (biomedcentral.com)
Induction2
- Induction of circulating myelin basic protein and proteolipid protein-specific transforming growth factor-beta1-secreting Th3 T cells by oral administration of myelin in multiple sclerosis patients. (jci.org)
- 99%) Collagen type II (CII) protein, purified from chicken sternum, for the induction of arthritis in the Collagen-Induced Art. (mdbioproducts.com)
Actin2
- In the absence of neurons, the major myelin membrane protein, the proteolipid protein (PLP), is internalized and stored in late endosomes/lysosomes (LEs/Ls) by a cholesterol-dependent and clathrin-independent endocytosis pathway that requires actin and the RhoA guanosine triphosphatase. (rupress.org)
- These membrane domains remain directly connected to the cell body with a complex underlying cytoarchitecture comprising microtubules distributed in larger processes and actin filaments enriched in thinner myelin domains and in paranodes [ 6 , 7 ]. (biomedcentral.com)
Cytoplasm1
- This distinction is based on the presence or absence of myelin degradation products within the cytoplasm of macrophages/microglia. (nih.gov)
Genes1
- We use basic and clinical research to understand the cells, proteins, and genes that lead to successful recovery of function, and also how complications develop that impact quality of life after stroke. (stanford.edu)
Mice3
- C57BL/6 (B6) mice deficient for the class II transactivator, which have defects in MHC class II, invariant chain (Ii), and H-2M (DM) expression, are resistant to initiation of myelin oligodendrocyte protein (MOG) peptide, MOG 35-55 -specific EAE by both priming and adoptive transfer of encephalitogenic T cells. (aai.org)
- However, class II transactivator-deficient mice can prime a suboptimal myelin-specific CD4 + Th1 response. (aai.org)
- This phenotype is not Ag-specific, as DM- and Ii-deficient mice are also resistant to initiation of EAE by proteolipid protein peptide PLP 178-191 . (aai.org)
Peptide1
- Although both Ii-deficient and DM-deficient APCs can present MOG peptide to CD4 + T cells, neither is capable of processing and presenting the encephalitogenic peptide of intact MOG protein. (aai.org)
Macrophages2
- Active and mixed active/inactive lesions can be further subdivided into lesions with ongoing myelin destruction (demyelinating lesions) and lesions in which the destruction of myelin has ceased, but macrophages are still present (post-demyelinating lesions). (nih.gov)
- In both cases, T cells specific for CNS myelin infiltrate the CNS, causing the influx of macrophages and activation of CNS resident cells ( 4 ). (aai.org)
Mutation1
- To understand the molecular basis of the jimpy mutation, we have examined the expression of mRNAs encoding myelin proteolipid protein (PLP). (mpg.de)
Central nervou1
- Myelin proteolipid protein (PLP or lipophilin) is the major myelin protein from the central nervous system (CNS). (wikipedia.org)
Specificity1
- Epitope spreading is defined as the diversification of epitope specificity from the initial focused, dominant epitope-specific immune response, directed against a self or foreign protein, to subdominant and/or cryptic epitopes on that protein (intramolecular spreading) or other proteins (intermolecular spreading). (nature.com)
Lesions1
- Also, the fact that ODCs close to or within neuroinflammatory lesions that have been deprived of their myelin processes can survive this insult [ 25 , 26 ] suggests the existence of active mechanisms of cellular plasticity. (biomedcentral.com)
Kinase1
- In addition, although protective functions are well documented for AMP-activated protein kinase (AMPK), paradoxically, disease-promoting effects have also been demonstrated for this enzyme. (mdpi.com)
Major1
- This graph shows the total number of publications written about "Myelin Proteins" by people in Harvard Catalyst Profiles by year, and whether "Myelin Proteins" was a major or minor topic of these publication. (harvard.edu)
Axonal2
Molecular1
- Moreover, at molecular level, each heparan sulfate proteoglycan (HSPG) consists of a core protein linked to one or more linear heparan sulfate (HS) chains composed of alternating D-glucosamine and uronic acids, leading to N - or O -sulfated glycan [ 5 ]. (biomedcentral.com)
Clinical1
- Studies in two models of multiple sclerosis, experimental autoimmune encephalomyelitis (EAE) and Theiler's murine encephalitogenic virus-induced demyelinating disease (TMEV-IDD) have shown conclusively that epitope spreading plays a pathological role in ongoing disease and that blocking this process by inducing tolerance to spread myelin epitopes or blocking costimulation of T cells (necessary for epitope spreading) blocks (EAE) or inhibits (TMEV-IDD) ongoing clinical disease. (nature.com)
Disorder2
- In humans, point mutations in PLP are the cause of Pelizaeus-Merzbacher disease (PMD), a neurologic disorder of myelin metabolism. (wikipedia.org)
- A rare, slowly progressive disorder of myelin formation. (uams.edu)
Cells4
- Here, we investigated whether in MS patients oral myelin treatment, containing both myelin basic protein (MBP) and proteolipid protein (PLP), induced antigen specific MBP or PLP reactive T cells that either secreted IL4, TGF-beta1, or alternatively did Th1 type sensitization occur as measured by IFN-gamma secretion. (jci.org)
- Tr1 cell-mediated protection against autoimmune disease by intranasal administration of a fusion protein targeting cDC1 cells. (mdbioproducts.com)
- Expression of circular RNA CDR1-AS in colon cancer cells increases cell surface PD-L1 protein levels. (harvard.edu)
- Examples include finding proteins indicating cardiac problems or looking for circulating tumour cells that can indicate the spread of cancer. (50webs.com)
Expression4
- Expression of myelin proteins in the opossum optic nerve: late appearance of inhibitors implicates an earlier non-myelin factor in preventing ganglion cell regeneration. (ox.ac.uk)
- Protein expression progresses along the optic nerve to reach the lamina cribrosa by P34, coincident with the time of eye opening. (ox.ac.uk)
- iv) Expression of the developmentally regulated proteolipid MAL is required for the cytotoxic effects. (cornell.edu)
- Importantly, ε-toxin-induced oligodendrocyte death results in demyelination and is dependent on expression of myelin and lymphocyte protein (MAL). (cornell.edu)
Profiles1
- Below are the most recent publications written about "Myelin Proteins" by people in Profiles. (harvard.edu)
Results1
- These results therefore suggest that regeneration in the developing retinofugal projection of the opossum is restricted by an earlier non-myelin factor, which is in contrast to current literature on the spinal cord. (ox.ac.uk)
Formation2
- It plays an important role in the formation or maintenance of the multilamellar structure of myelin. (wikipedia.org)
- 2-Fluoropalmitic acid is a synthetic inhibitor of palmitoyl-CoA formation by long chain acyl-CoA synthetase thereby inhibiting sphingosine biosynthesis and protein palmitoylation. (matreya.com)
Cell1
- These severe mutations are believed to result in misfolding of the newly synthesized protein, which then accumulates in the endoplasmic reticulum and triggers apoptosis, or programmed cell death. (medscape.com)
Mouse2
- Purified myelin proteolipid protein (PLP) for PLP-induced EAE models in mouse and rat. (mdbioproducts.com)
- Protein citrullination marks myelin protein aggregation and disease progression in mouse ALS models. (harvard.edu)
Domains1
- Zinc finger (Znf) domains are relatively small protein motifs which contain multiple finger-like protrusions that make tandem contacts with their target molecule. (embl.de)
Function1
- Vertebrate L3MBTL, a member of the Polycomb group of proteins, which function as transcriptional repressors in large protein complexes. (embl.de)
