• [ 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)
  • 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)
  • The crystal structure of the S. cerevisiae c-subunit ring with bound oligomycin revealed the inhibitor docked on the outer face of the proton-binding sites, deep in the transmembrane region ( Zhou and Faraldo-Gómez 2018 ). (tcdb.org)
  • This probably results from duplication (intragenic and/or intergenic) of the proteolipid (c) subunit. (tcdb.org)
  • This gene encodes a multispan transmembrane protein belonging to the MAL proteolipid family. (wikipedia.org)
  • This gene encodes a transmembrane proteolipid protein that is the predominant component of myelin. (nih.gov)
  • FHM type 4 is attributed to mutations in the PRRT2 gene, which encodes a proline-rich transmembrane protein of as yet unknown function. (frontiersin.org)
  • which encodes a proline-rich transmembrane protein of still unknown function. (frontiersin.org)
  • Correlation Between Anti-Myelin Proteolipid Protein (PLP) Antibodies and Disease Severity in Multiple Sclerosis Patients With PLP Response-Permissive HLA Types. (nih.gov)
  • Spin-label ESR studies on avidin-biotinyl phosphatidylethanolamine in membrane recombinants with myelin proteolipid proteins. (mpg.de)
  • Microwave frequency dependence of ESR-spectra from spin labels undergoing 2-site exchange in myelin proteolipid membranes. (mpg.de)
  • Exchange rates at the lipid-protein interface of the myelin proteolipid protein determined by saturation transfer electron spin resonance and continuous wave saturation studies. (mpg.de)
  • Influence of polar residue deletions on lipid-protein interactions with the myelin proteolipid protein. (mpg.de)
  • 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)
  • AtpR genes are #=GF CC found in the N-ATPase (archaeal-type F1-Fo-ATPase) operons and #=GF CC are predicted to interact with the conserved Glu/Asp residues in #=GF CC the c subunits, regulating the assembly and/or function of the #=GF CC membrane-embedded ring of 'c' (proteolipid) subunits #=GF CC (PFAM:PF00137). (xfam.org)
  • Protein kinase A anchor protein, nuclear localisation signal domain [Interproscan]. (ntu.edu.sg)
  • A-kinase anchoring protein 11 [Sourc. (gsea-msigdb.org)
  • Perilipin 5 (PLIN5) is a lipid-droplet-associated protein that coordinates intracellular lipolysis in highly oxidative tissues and is thought to regulate lipid metabolism in response to phosphorylation by protein kinase A (PKA). (uci.edu)
  • 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)
  • Micrograph of a section of the cerebral cortex from a person with Alzheimer's disease , immunostained with an antibody to amyloid beta (brown), a protein fragment that accumulates in amyloid plaques and cerebral amyloid angiopathy . (wikipedia.org)
  • Description: This is Double-antibody Sandwich Enzyme-linked immunosorbent assay for detection of Mouse EGF Like Domain Protein, Multiple 7 (EGFL7) in serum, plasma, tissue homogenates and other biological fluids. (gkts.net)
  • Description: Enzyme-linked immunosorbent assay based on the Double-antibody Sandwich method for detection of Mouse EGF Like Domain Protein, Multiple 7 (EGFL7) in samples from Serum, plasma, tissue homogenates and other biological fluids with no significant corss-reactivity with analogues from other species. (gkts.net)
  • That is to say that all 3 phases of DNA were being used to encode different proteins. (lindau-nobel.org)
  • So the mitochondria contain about 2,000 different proteins in human cells and only 13 of them are encoded in the mitochondrial genome. (lindau-nobel.org)
  • [9] Subsequent research has shown that many different proteins can form amyloid, and that all amyloids show birefringence in cross- polarized light after staining with the dye Congo red , as well as a fibrillar ultrastructure when viewed with an electron microscope . (wikipedia.org)
  • The sponsor cell plasma membrane can be a complex framework cis-Urocanic acid formed with a liquid and powerful lipid bilayer to which different proteins and ligands with different natural functions are connected [12]. (healthanddietblog.info)
  • Protein MAL2 is a protein that in humans is encoded by the MAL2 gene. (wikipedia.org)
  • When an E5 mutant (M1) in which the first putative transmembrane helix had been disrupted was used for the transfections calnexin-E5 co-precipitation was strongly impaired. (biomedcentral.com)
  • To invade and establish the PV sequentially discharges proteins from three secretory organelles - the micronemes rhoptries (ROP) and dense granules (1 2 The micronemes contain several adhesion proteins that allow the attachment of Ricasetron parasites to host cells (3). (colinsbraincancer.com)
  • GRA15 is sufficient for host nuclear factor kappa B activation (10) and GRA1 GRA4 and GRA7 are effective antigens used in DNA vaccines (11 12 These findings demonstrate that secretory proteins play a significant role in host cell modulation and immune responses. (colinsbraincancer.com)
  • It is required for transcytosis, an intracellular transport pathway used to deliver membrane-bound proteins and exogenous cargos from the basolateral to the apical surface. (wikipedia.org)
  • Semaphorins are a family of secreted and membrane-bound proteins, known to regulate axonal pathfinding. (ox.ac.uk)
  • LRRFIP1 can be a leucine wealthy repeat site containing proteins like the TLRs but cytoplasmic in localization3. (researchdataservice.com)
  • Anillin (ANLN) is an actin-binding protein that is essential for cell division and contributes to cell growth and migration. (biomedcentral.com)
  • The final results show that homeopathy can easily alleviate the particular myocardial ischemia involving cardiac tissues, lessen substantially the activities of serum Grass along with MDA, as well as thus affect the necessary protein expressions this website associated with CFTR and also CLC-2 throughout CL- programs. (interleukinreceptor.com)
  • The outcome from the examine means that chinese medicine suppresses the particular pathological adjustments of heart failure tissue of rodents together with myocardial ischemia as well as regulates your health proteins expression of CFTR and CLC-2 CL- channels, which may serve as 1 achievable procedure to reduce myocardial ischemia.Subject. (interleukinreceptor.com)
  • And what Fred asked me to do was to sequence the proteins that were encoded in the genes that he was sequencing because he wanted to be sure about the accuracy of his DNA sequencing methods. (lindau-nobel.org)
  • And again my task was to try to identify the protein genes that were. (lindau-nobel.org)
  • the genes encoding proteins in that genome. (lindau-nobel.org)
  • 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)
  • Lipid-protein interactions and effect of local anesthetics in acetylcholine receptor-rich membranes from Torpedo marmorata electric organ. (mpg.de)
  • Double-labeling experiments with different markers of oligodendrocyte lineage such as olig1, olig2, platelet-derived growth factor receptor alpha, and proteolipid protein showed that Sema4D was expressed selectively by oligodendrocytes and myelin. (ox.ac.uk)
  • DEC-205 (CD205), a member of the macrophage mannose receptor protein family, is the prototypic endocytic receptor of dendritic cells, whose ligands include phosphorothioated cytosine-guanosine (CpG) oligonucleotides, a motif often seen in bacterial or viral DNA. (uci.edu)
  • Here we describe the 3.2 Å cryo-EM structure of human DEC-205, thereby illuminating the structure of the mannose receptor protein family. (uci.edu)
  • The evolutionarily ancient rhomboid protein family represents a unique class of membrane-bound proteases (1 2 The proteolytic function of rhomboids was initially recognized and extensively studied in Drosophila where in fact the proteases play a crucial role in epidermal growth factor receptor signaling by controlling the release of membrane-bound growth factors (3). (gasyblog.com)
  • Though this protein is not a DNA receptor STING deficient mice represents. (researchdataservice.com)
  • SLAM is usually indicated in values as compared with Ed-H. Although some alterations were observed none of the mutations introduced at the dimer-dimer interfaces of the head domain greatly affected the cell surface expression receptor binding and conversation with the F protein of MV-H. FIGURE 2. (conferencedequebec.org)
  • HEK293T cells were transfected with an empty vector ((31) exhibited using transcomplementation experiments that receptor binding to only one dimer of the MV-H head domain name dimer of dimers can induce F protein triggering mediated by the stalks of the other dimer. (conferencedequebec.org)
  • The results suggest that receptor binding and F protein triggering could be communicated across two MV-H dimers either at the head domain or at the stalk region. (conferencedequebec.org)
  • Description: A sandwich ELISA kit for detection of EGF Like Domain Protein, Multiple 7 from Mouse in samples from blood, serum, plasma, cell culture fluid and other biological fluids. (gkts.net)
  • [13] [14] Because proteins share a common structural feature known as the polypeptide backbone, all proteins have the potential to misfold under some circumstances. (wikipedia.org)
  • Protein Purification The core catalytic domain 473727-83-2 supplier name of Escherichia coli GlpG and the fusion substrate maltose-binding protein-Gurken-thioredoxin were prepared as described previously (23). (gasyblog.com)
  • The likely solution is that the fusion pore must be of a hybrid composition incorporating protein as well as lipids and that both SNARE TM domains Rutaecarpine (Rutecarpine) and lipids collection the pore. (biotech2012.org)
  • 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)
  • MARV assembles and buds from the host cell plasma where MARV matrix protein (mVP40) dimers associate with anionic lipids at the plasma membrane inner leaflet and undergo a dynamic and extensive self-oligomerization into the structural matrix layer. (uci.edu)
  • EXPERIMENTAL PROCEDURES Reagents The detergents used in membrane protein purification and crystallization were purchased from Affymetrix Inc. (Santa Clara CA). Diisopropyl fluorophosphate (DFP) was purchased from EMD Chemicals Inc. 1 2 (DMPC) was purchased from Avanti Polar Lipids Inc. (Alabaster AL). (gasyblog.com)
  • Integration of a K+ channel-associated peptide in a lipid bilayer: Conformation, lipid-protein interactions, and rotational diffusion. (mpg.de)
  • The transmembrane semaphorin Sema4D/CD100, an inhibitor of axonal growth, is expressed on oligodendrocytes and upregulated after CNS lesion. (ox.ac.uk)
  • After dialysis freshly prepared DFP (50 mm in Me2SO) was added to the protein solution (5:1 inhibitor/protease molar ratio) to completely inactivate the protease (24). (gasyblog.com)
  • Nanodiscs are self-assembled contaminants which contain an individual phospholipid bilayer with nanometer measurements stabilized by an encircling membrane scaffold proteins (MSP) 10. (biotech2012.org)
  • The other two families contain uncharacterized proteins annotated as hypothetical unknown. (colinsbraincancer.com)
  • Our studies show that these hypothetical unknowns are new GRA proteins that localize to the PVM and PV. (colinsbraincancer.com)
  • In addition, we show that the M1 mutant is only able to marginally down-regulate HLA-I surface expression compared to the wild-type protein. (biomedcentral.com)
  • Interaction of membrane-spanning proteins with peripheral and lipid-anchored membrane proteins: perspectives from protein- lipid interactions (Review). (mpg.de)
  • Multiple expansion microscopy approaches have been successfully used in the analysis of centrioles, centrosomes, and cilia, helping to reveal the localization of numerous centrosomal and ciliary proteins at nanoscale resolution. (bvsalud.org)
  • FAM161 centrosomal protein A [Source. (gsea-msigdb.org)
  • protein_coding" "Cz01g39090.t1","No alias","Chromochloris zofingiensis","Protein N-terminal asparagine amidohydrolase [Interproscan]. (ntu.edu.sg)
  • Protein N-terminal asparagine amidohydrolase [Interproscan]. (ntu.edu.sg)
  • Measles virus (MV) an enveloped RNA pathogen owned by the family members enters the cell through membrane fusion mediated by two viral envelope protein an attachment proteins hemagglutinin (H) and a fusion (F) proteins. (conferencedequebec.org)
  • Interactions between lipid-anchored and transmembrane proteins. (mpg.de)
  • Lipid-protein interactions in ADP-ATP carrier/egg phosphatidylcholine recombinants studied by spin-label ESR spectroscopy. (mpg.de)
  • E5 impairs ligand-mediated apoptosis by reducing the amount of surface CD95 proteins or inhibiting the formation of the DISC complex [ 12 ], and affects the normal functioning of a number of membrane associated proteins, probably by modifying the composition and the interactions in the cell membranes [ 13 ]. (biomedcentral.com)
  • FLIM-FRET analysis of protein-protein interactions showed that PLIN5 S155 phosphorylation regulates PLIN5 interaction with adipose triglyceride lipase at the lipid droplet, but not with α-β hydrolase domain-containing 5. (uci.edu)
  • GRA5 is usually secreted as a soluble protein into the PV and becomes stably associated with the PVM (19). (colinsbraincancer.com)
  • Reverse transcription quantitative polymerase chain reaction and immunohistochemistry were used to determine ANLN mRNA and protein expression in colorectal cancer (CRC), gastric cancer (GC), and hepatocellular carcinoma (HCC) cell lines. (biomedcentral.com)
  • proteinopathic adj ), or proteopathy , protein conformational disorder , or protein misfolding disease , is a class of diseases in which certain proteins become structurally abnormal, and thereby disrupt the function of cells , tissues and organs of the body. (wikipedia.org)
  • rearrangements from the stalk area which cause conformational adjustments from the F proteins. (conferencedequebec.org)
  • protein_coding" "Cz02g09110.t1","No alias","Chromochloris zofingiensis","2-hydroxy-palmitic acid dioxygenase Mpo1-like [Interproscan]. (ntu.edu.sg)
  • And the importance of this was not just that it was the first protein, but it demonstrated that proteins had a defined sequence which some people doubted. (lindau-nobel.org)
  • In this chapter, we describe the use of two stable STED dyes in combination with expansion microscopy, which allows the robust detection by conventional and STED microscopy of proteins immunolabeled prior to sample expansion. (bvsalud.org)
  • Description: A sandwich quantitative ELISA assay kit for detection of Human EGF Like Domain Protein, Multiple 7 (EGFL7) in samples from serum, plasma, tissue homogenates or other biological fluids. (gkts.net)
  • When reconstituted into liposomes these protein represent a minor equipment that promotes fusion 2-4 which includes resulted in the hypothesis how the SNARE proteins open up the fusion pore which allows vesicular material to become released into extracellular space. (biotech2012.org)
  • protein_coding" "Cz03g09200.t1","No alias","Chromochloris zofingiensis","UV radiation resistance protein/autophagy-related protein 14 [Interproscan]. (ntu.edu.sg)
  • protein_coding" "Cz03g33270.t1","No alias","Chromochloris zofingiensis","Domain of unknown function DUF155 [Interproscan]. (ntu.edu.sg)
  • protein_coding" "Cz10g28290.t1","No alias","Chromochloris zofingiensis","Chorismate pyruvate-lyase Rv2949c-like [Interproscan]. (ntu.edu.sg)
  • protein_coding" "Cz01g27080.t1","No alias","Chromochloris zofingiensis","Solute-binding protein family 5 domain [Interproscan]. (ntu.edu.sg)
  • protein_coding" "Cz03g01030.t1","No alias","Chromochloris zofingiensis","Rieske [2Fe-2S] iron-sulphur domain [Interproscan]. (ntu.edu.sg)
  • The human papillomavirus type 16 E5 protein (HPV16 E5) down-regulates surface expression of HLA-I molecules. (biomedcentral.com)
  • Besides, we demonstrate that E5 forms a ternary complex with calnexin and the heavy chain of HLA-I, which is mediated by the first hydrophobic region of the E5 protein. (biomedcentral.com)
  • We demonstrate the applicability of this approach by analyzing both a centriole appendage protein Cep164 and a ciliary protein ARL13B. (bvsalud.org)
  • The protein is a component of lipid rafts and, in polarized cells, it primarily localizes to endosomal structures beneath the apical membrane. (wikipedia.org)
  • Type We Interferons are cytokines from the innate disease fighting capability that creates antiviral proteins manifestation in response to AT7867 viral disease. (researchdataservice.com)
  • Recent studies possess determined IFITM proteins as the 1st ISG to inhibit viral admittance processes and exposed mechanistic knowledge of known anti-viral ISGs such as ISG15 and Viperin. (researchdataservice.com)
  • The HT/PEXEL motif serves as a cleavage signal and may contributes to protein-protein associations. (colinsbraincancer.com)
  • The framework of ~12nm MSP1E2 was modeled predicated on Rutaecarpine (Rutecarpine) crystal framework … If fusion skin pores can't be lipidic as concluded from the tiny nanodisc size they might be formed by proteins transmembrane domains as an ion route or space junction pore. (biotech2012.org)
  • The eukaryotic proteins are more complicated than the bacterial enzyme complexes. (tcdb.org)
  • Then, a survey is given about ATP1A2 mutations implicated in migraine cases as documented in the literature with focus on mutations that were described to completely destroy enzyme function, or lead to misfolded or mistargeted protein in particular model cell lines. (frontiersin.org)
  • Furthermore our outcomes indicate that even though the stalk and transmembrane regions may be mainly responsible for the tetramer formation of MV-H the head domain alone can form tetramers albeit at a low efficiency. (conferencedequebec.org)
  • 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)