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NPC1
Niemann-Pick disease, type C1 (NPC1) is a disease of a membrane protein that mediates intracellular cholesterol trafficking in mammals. In humans the protein is encoded by the ''NPC1'' gene (chromosome location 18q11). Function NPC1 was identified as the gene that when mutated, results in Niemann-Pick disease, type C. Niemann-Pick disease, type C is a rare neurovisceral lipid storage disorder resulting from autosomal recessively inherited loss-of-function mutations in either NPC1 or NPC2. This disrupts intracellular lipid transport, leading to the accumulation of lipid products in the late endosomes and lysosomes. Approximately 95% of NPC patients are found to have mutations in the NPC1 gene. NPC1 encodes a putative integral membrane protein containing sequence motifs consistent with a role in intracellular transport of cholesterol and sphingosine to post-lysosomal destinations. Clinical significance Obesity Mutations in the NPC1 gene have been strongly linked with ob ... [...More Info...] [...Related Items...] OR: [Wikipedia] [Google] [Baidu] |
NPC1 Location
Niemann-Pick disease, type C1 (NPC1) is a disease of a membrane protein that mediates intracellular cholesterol trafficking in mammals. In humans the protein is encoded by the ''NPC1'' gene (chromosome location 18q11). Function NPC1 was identified as the gene that when mutated, results in Niemann-Pick disease, type C. Niemann-Pick disease, type C is a rare neurovisceral lipid storage disorder resulting from autosomal recessively inherited loss-of-function mutations in either NPC1 or NPC2. This disrupts intracellular lipid transport, leading to the accumulation of lipid products in the late endosomes and lysosomes. Approximately 95% of NPC patients are found to have mutations in the NPC1 gene. NPC1 encodes a putative integral membrane protein containing sequence motifs consistent with a role in intracellular transport of cholesterol and sphingosine to post-lysosomal destinations. Clinical significance Obesity Mutations in the NPC1 gene have been strongly linked with ob ... [...More Info...] [...Related Items...] OR: [Wikipedia] [Google] [Baidu] |
Ebola Virus
''Zaire ebolavirus'', more commonly known as Ebola virus (; EBOV), is one of six known species within the genus ''Ebolavirus''. Four of the six known ebolaviruses, including EBOV, cause a severe and often fatal hemorrhagic fever in humans and other mammals, known as Ebola virus disease (EVD). Ebola virus has caused the majority of human deaths from EVD, and was the cause of the 2013–2016 epidemic in western Africa, which resulted in at least suspected cases and confirmed deaths. Ebola virus and its genus were both originally named for Zaire (now the Democratic Republic of the Congo), the country where it was first described, and was at first suspected to be a new "strain" of the closely related Marburg virus. The virus was renamed "Ebola virus" in 2010 to avoid confusion. Ebola virus is the single member of the species ''Zaire ebolavirus'', which is assigned to the genus ''Ebolavirus'', family ''Filoviridae'', order ''Mononegavirales''. The members of the species are cal ... [...More Info...] [...Related Items...] OR: [Wikipedia] [Google] [Baidu] |
Marburg Virus
Marburg virus (MARV) is a hemorrhagic fever virus of the ''Filoviridae'' family of viruses and a member of the species '' Marburg marburgvirus'', genus ''Marburgvirus''. It causes Marburg virus disease in primates, a form of viral hemorrhagic fever. The virus is considered to be extremely dangerous. The World Health Organization (WHO) rates it as a Risk Group 4 Pathogen (requiring biosafety level 4-equivalent containment). In the United States, the National Institute of Allergy and Infectious Diseases ranks it as a Category A Priority Pathogen and the Centers for Disease Control and Prevention lists it as a Category A Bioterrorism Agent. It is also listed as a biological agent for export control by the Australia Group. The virus can be transmitted by exposure to one species of fruit bats or it can be transmitted between people via body fluids through unprotected sex and broken skin. The disease can cause haemorrhage, fever, and other symptoms similar to Ebola, which belongs t ... [...More Info...] [...Related Items...] OR: [Wikipedia] [Google] [Baidu] |
Myelin Gene Regulatory Factor
Myelin regulatory factorMyRF, also known as myelin gene regulatory factor (MRF), is a protein that in humans is encoded by the ''MYRF'' gene. Orthologs Myelin regulatory factor is encoded by the ''Myrf/GM98'' gene in mice and by the ''MYRF'' gene in humans. The family of MyRF-like-proteins also contains the orthologues pqn-47 from ''C. elegans'' and MYRFA from ''Dictyostelium''. All orthologs have a DNA-binding domain of high homology to the ''Saccharomyces cerevisiae'' protein Ndt80 (a p53-like transcription factor) and therefore likely act as a transcription factor. Function MyRF is a transcription factor that promotes the expression of many genes important in the production of myelin. It is therefore of critical importance in the development and maintenance of myelin sheaths. The expression of MYRF is specific to mature, myelinating oligodendrocytes in the CNS. It has been shown to be critical for the maintenance of myelin by these cells. Following ablation of MYRF ... [...More Info...] [...Related Items...] OR: [Wikipedia] [Google] [Baidu] |
Nef (protein)
Nef (Negative Regulatory Factor) is a small 27-35 kDa myristoylated protein encoded by primate lentiviruses. These include Human Immunodeficiency Viruses (HIV-1 and HIV-2) and Simian Immunodeficiency Virus (SIV). Nef localizes primarily to the cytoplasm but also partially to the Plasma membrane (PM) and is one of many pathogen-expressed proteins, known as virulence factors, which function to manipulate the host's cellular machinery and thus allow infection, survival or replication of the pathogen. Nef stands for "Negative Factor" and although it is often considered indispensable for HIV-1 replication, in infected hosts the viral protein markedly elevates viral titers. Function The expression of Nef early in the viral life cycle ensures T-cell activation and the establishment of a persistent state of infection, two basic attributes of HIV infection. Viral expression of Nef induces numerous changes within the infected cell including the modulation of protein cell surface express ... [...More Info...] [...Related Items...] OR: [Wikipedia] [Google] [Baidu] |
Transcription Factor
In molecular biology, a transcription factor (TF) (or sequence-specific DNA-binding factor) is a protein that controls the rate of transcription of genetic information from DNA to messenger RNA, by binding to a specific DNA sequence. The function of TFs is to regulate—turn on and off—genes in order to make sure that they are expressed in the desired cells at the right time and in the right amount throughout the life of the cell and the organism. Groups of TFs function in a coordinated fashion to direct cell division, cell growth, and cell death throughout life; cell migration and organization (body plan) during embryonic development; and intermittently in response to signals from outside the cell, such as a hormone. There are up to 1600 TFs in the human genome. Transcription factors are members of the proteome as well as regulome. TFs work alone or with other proteins in a complex, by promoting (as an activator), or blocking (as a repressor) the recruitment of RNA ... [...More Info...] [...Related Items...] OR: [Wikipedia] [Google] [Baidu] |
Small Molecule
Within the fields of molecular biology and pharmacology, a small molecule or micromolecule is a low molecular weight (≤ 1000 daltons) organic compound that may regulate a biological process, with a size on the order of 1 nm. Many drugs are small molecules; the terms are equivalent in the literature. Larger structures such as nucleic acids and proteins, and many polysaccharides are not small molecules, although their constituent monomers (ribo- or deoxyribonucleotides, amino acids, and monosaccharides, respectively) are often considered small molecules. Small molecules may be used as research tools to probe biological function as well as leads in the development of new therapeutic agents. Some can inhibit a specific function of a protein or disrupt protein–protein interactions. Pharmacology usually restricts the term "small molecule" to molecules that bind specific biological macromolecules and act as an effector, altering the activity or function of the target. Small ... [...More Info...] [...Related Items...] OR: [Wikipedia] [Google] [Baidu] |
Viral Envelope
A viral envelope is the outermost layer of many types of viruses. It protects the genetic material in their life cycle when traveling between host cells. Not all viruses have envelopes. Numerous human pathogenic viruses in circulation are encased in lipid bilayers, and they infect their target cells by causing the viral envelope and cell membrane to fuse. Although there are effective vaccines against some of these viruses, there is no preventative or curative medicine for the majority of them. In most cases, the known vaccines operate by inducing antibodies that prevent the pathogen from entering cells. This happens in the case of enveloped viruses when the antibodies bind to the viral envelope proteins. The membrane fusion event that triggers viral entrance is caused by the viral fusion protein. Many enveloped viruses only have one protein visible on the surface of the particle, which is required for both mediating adhesion to the cell surface and for the subsequent membrane fusi ... [...More Info...] [...Related Items...] OR: [Wikipedia] [Google] [Baidu] |
Filovirus
''Filoviridae'' () is a family of single-stranded negative-sense RNA viruses in the order ''Mononegavirales''. Two members of the family that are commonly known are Ebola virus and Marburg virus. Both viruses, and some of their lesser known relatives, cause severe disease in humans and nonhuman primates in the form of viral hemorrhagic fevers. All filoviruses are classified by the US as select agents, by the World Health Organization as Risk Group 4 Pathogens (requiring Biosafety Level 4-equivalent containment), by the National Institutes of Health/National Institute of Allergy and Infectious Diseases as Category A Priority Pathogens, and by the Centers for Disease Control and Prevention as Category A Bioterrorism Agents, and are listed as Biological Agents for Export Control by the Australia Group. Use of term The family ''Filoviridae'' is a virological taxon that was defined in 1982 and emended in 1991, 1998, 2000, 2005, 2010 and 2011. The family currently includes the six ... [...More Info...] [...Related Items...] OR: [Wikipedia] [Google] [Baidu] |
Lipid Raft
The plasma membranes of cells contain combinations of glycosphingolipids, cholesterol and protein receptors organised in glycolipoprotein lipid microdomains termed lipid rafts. Their existence in cellular membranes remains somewhat controversial. It has been proposed that they are specialized membrane microdomains which compartmentalize cellular processes by serving as organising centers for the assembly of signaling molecules, allowing a closer interaction of protein receptors and their effectors to promote kinetically favorable interactions necessary for the signal transduction. Lipid rafts influence membrane fluidity and membrane protein trafficking, thereby regulating neurotransmission and receptor trafficking. Lipid rafts are more ordered and tightly packed than the surrounding bilayer, but float freely within the membrane bilayer. Although more common in the cell membrane, lipid rafts have also been reported in other parts of the cell, such as the Golgi apparatus and lysosome ... [...More Info...] [...Related Items...] OR: [Wikipedia] [Google] [Baidu] |
Protein
Proteins are large biomolecules and macromolecules that comprise one or more long chains of amino acid residues. Proteins perform a vast array of functions within organisms, including catalysing metabolic reactions, DNA replication, responding to stimuli, providing structure to cells and organisms, and transporting molecules from one location to another. Proteins differ from one another primarily in their sequence of amino acids, which is dictated by the nucleotide sequence of their genes, and which usually results in protein folding into a specific 3D structure that determines its activity. A linear chain of amino acid residues is called a polypeptide. A protein contains at least one long polypeptide. Short polypeptides, containing less than 20–30 residues, are rarely considered to be proteins and are commonly called peptides. The individual amino acid residues are bonded together by peptide bonds and adjacent amino acid residues. The sequence of amino acid residue ... [...More Info...] [...Related Items...] OR: [Wikipedia] [Google] [Baidu] |
