• Voltage-gated potassium channel (VGKC) antibody-associated encephalopathy has been identified as a potentially immunotherapy-responsive form of limbic encephalitis. (psychiatrist.com)
  • Detect antibodies to voltage-gated potassium channel (VGKC) antibody receptor complex-associated, leucine-rich glioma inactivated 1 protein (LGI1) and contactin associated protein 2 (CASPR2). (aruplab.com)
  • see Voltage-Gated Potassium Channel (VGKC) Antibody with Reflex to LGI1 and CASPR2 Screen and Titer, Serum ( 2009463 ). (aruplab.com)
  • Voltage-gated potassium channel antibodies (VGKC-Ab) have recently been reported in three cases of reversible limbic encephalitis. (ox.ac.uk)
  • OBJECTIVES: Autoantibodies against the extracellular domains of the voltage-gated potassium channel (VGKC) complex proteins, leucine-rich glioma-inactivated 1 (LGI1) and contactin-associated protein-2 (CASPR2), are found in patients with limbic encephalitis, faciobrachial dystonic seizures, Morvan's syndrome and neuromyotonia. (ox.ac.uk)
  • However, in routine testing, VGKC complex antibodies without LGI1 or CASPR2 reactivities (double-negative) are more common than LGI1 or CASPR2 specificities. (ox.ac.uk)
  • RESULTS: VGKC complex antibodies were found in 162 of 1131 (14%) sera. (ox.ac.uk)
  • Of the remaining 72 double-negative sera, 10 (14%) immunoprecipitated 125I-αDTX itself, and 27 (38%) bound to solubilised co-expressed Kv1.1/1.2/1.6 subunits and/or Kv1.2 subunits alone, at levels proportionate to VGKC complex antibody levels (r=0.57, p=0.0017). (ox.ac.uk)
  • CONCLUSIONS: Double-negative VGKC complex antibodies are often directed against cytosolic epitopes of Kv1 subunits and occasionally against non-mammalian αDTX. (ox.ac.uk)
  • N-Methyl-D-Aspartate(NMDA) Receptor and Voltage-Gated Potassium Channel (VGKC) Antibody-Associated Encephalitides Presenting as First Episode Acute Psychosis. (bvsalud.org)
  • Our study aimed to describe the frequency, clinical spectrum, and long-term outcomes in patients presenting with acute psychosis who have anti- N-methyl-D-aspartate ( NMDA ) receptor or anti- voltage-gated potassium channel (VGKC) encephalitis . (bvsalud.org)
  • Eighty-two available samples were retested for antibodies to N -methyl- d -aspartate receptor (NMDAR), the glycine receptor (GlyR), voltage-gated potassium channel (VGKC)-complex and the associated proteins, leucine-rich glioma inactivated 1 (LGI1) and contactin-associated protein 2 (CASPR2). (bmj.com)
  • two had antibodies to NMDAR, and two to the VGKC-complex, one of which was also positive for GlyR antibodies. (bmj.com)
  • Over the same period three patients with autoimmune encephalitis and high VGKC-complex antibodies were initially referred as sCJD. (bmj.com)
  • By contrast, three patients referred with possible prion disease had a clinical picture in keeping with autoimmune encephalitis and very high VGKC-complex/LGI1 antibodies. (bmj.com)
  • Conversely, there have been occasional reports of patients presenting with encephalopathy and low levels of serum antibodies to neuronal protein such as the N -methyl- d -aspartate receptor (NMDAR), voltage-gated potassium channel complex (VGKC-complex) or glycine receptor (GlyR), who were later confirmed to have sporadic CJD (sCJD). (bmj.com)
  • Recent studies reported that some patients confirmed to have sCJD had low levels of serum antibodies to NMDAR, glycine receptor, and voltage-gated potassium channel (VGKC) complex ( 2 - 4 ). (frontiersin.org)
  • Conversely, some researchers have found that some forms of autoimmune encephalitis could be misdiagnosed as CJD, including Hashimoto's and VGKC complex antibody-associated encephalopathies ( 5 , 6 ). (frontiersin.org)
  • Conversely, low levels of antibodies to neuronal proteins have been reported in patients with sCJD. (bmj.com)
  • Abcam: antibodies, proteins, kits. (abcam.com)
  • Found associated with Kv4 channels are a group of calcium-binding proteins termed KChIPs (Kv channel interacting proteins). (fishersci.com)
  • The therapeutic and biochemical properties of proteins including antibodies can be enhanced by custom chemical functionalization that enables modifications, such as small molecule drug conjugation, PE. (warf.org)
  • In addition to these autoantibodies, patients with thymoma-associated MG produce autoantibodies to various neuromuscular antigens, including antibodies to the skeletal muscle calcium release channel (ryanodine receptor of sarcoplasmic reticulum) and antibodies to cytoplasmic filamentous proteins (particularly titin) or neurofilaments. (medscape.com)
  • A UW-Madison researcher has developed a rabbit polyclonal antibody for methylated PKM2. (warf.org)
  • 90 of these (56%) had antibodies targeting the extracellular domains of LGI1 or CASPR2. (ox.ac.uk)
  • UW-Madison researchers have developed and characterized nine murine monoclonal antibody (mAb) reagents that react uniquely with the EC1 (2x), EC2 (2x), EC5 (3x), and EC6 (2x) extracellular cadherin re. (warf.org)
  • Conclusions: IMBX can block TREK-1 channels though the PKA pathway, it also can bind to the extracellular side of THIK-1 or THIK-2, leading to a direct block of the channels. (uni-marburg.de)
  • These intracellular Kv1 antibodies mainly associated with non-immune disease aetiologies, poor longitudinal clinical-serological correlations and a limited immunotherapy response. (ox.ac.uk)
  • Sequential antibodies to potassium channels and glutamic acid decarboxylase in neuromyotonia. (ox.ac.uk)
  • A patient with thymoma-associated neuromyotonia and voltage-gated potassium channel (Kv1.2 and Kv1.6) antibodies by immunoprecipitation and rat brain immunolabeling was treated successfully with immunoadsorption and cyclophosphamide. (ox.ac.uk)
  • The range of antibodies produced in thymoma-associated neuromyotonia is richer, and the timing of antibody appearance more complex, than previously suspected. (ox.ac.uk)
  • Antibodies against a presynaptic structure, the voltage-gated potassium channels of peripheral nerves, have been detected in patients with neuromyotonia with or without thymoma. (medscape.com)
  • Neuromyotonia and antibodies to the voltage-gated potassium channels have also been found in patients with MG. Twenty percent of patients with MG and neuromyotonia have been demonstrated to have thymoma. (medscape.com)
  • The sera with LGI1 and CASPR2 antibodies immunoprecipitated neither preparation. (ox.ac.uk)
  • A nonclassical neurological syndrome, no paraneoplastic antibodies, and cancer that presents within 2 years of the neurological syndrome. (medscape.com)
  • Lumbar puncture is essential for determination of the CSF profile and detection of intrathecal paraneoplastic antibodies. (medscape.com)
  • 5% patients with sCJD develop serum antibodies to these neuronal antigens and, when positive, only at low titres. (bmj.com)
  • Although the absence of NMDAR antibodies in 346 referred cerebrospinal fluid (CSF) samples, including samples from 49 confirmed sCJD cases 4 was reported recently, the frequency of disease-relevant serum antibodies in patients with sCJD prior to diagnosis, and how frequently an incorrect CJD diagnosis could have been averted, have not been studied systematically. (bmj.com)
  • Antibodies associated with these conditions may be present in the serum or cerebrospinal fluid (CSF) and can serve as useful markers of disease. (arupconsult.com)
  • Testing for the presence of antineural antibodies in both serum and CSF is recommended in most situations. (arupconsult.com)
  • These specialized antibodies are tailored for various scientific applications like WB, ELISA, FACS, IHC, IF (cc), providing you with options like polyclonal, recombinant, and monoclonal antibodies, sourced from different host species such as Rabbit. (antibodies-online.com)
  • Find available monoclonal or polyclonal KCNH2 Antibodies. (antibodies-online.com)
  • UW-Madison researchers have developed three novel monoclonal antibodies that all have exceptional activity against human and mouse GATA2. (warf.org)
  • The present studies identify distinct functional domains that confer differential sensitivities of Kv7.4 and Kv7.5 to stimulatory and inhibitory signaling and reveal structural features of the channel subunits that determine their biophysical properties. (aspetjournals.org)
  • These antibodies are to be used as research laboratory reagents and are not for use as diagnostic or therapeutic reagents in humans. (antibodiesinc.com)
  • human brain tissue were subjected to SDS PAGE followed by western blot with 12696-1-AP (Kir5.1 antibody) at dilution of 1:400 incubated at room temperature for 1.5 hours. (ptglab.com)
  • antibody detection limit dilution 1:64000. (everestbiotech.com)
  • Kvβ1.1 is a cytoplasmic subunit that modulates the characteristics of the membrane spanning, channel-forming alpha-subunits. (antibodiesinc.com)
  • this sequence is reminiscent of the ER retention/retrieval motif RKR identified in subunits of the ATP-sensitive K+ channel. (nih.gov)
  • Beta subunits can promote the closure of channels or enhance channel activity depending on the channel members. (antibodiesinc.com)
  • We expressed chimeric channels composed of different components of the Kv7.4 and Kv7.5 α -subunits in vascular smooth muscle cells to determine which components are essential for enhancement or inhibition of channel activity. (aspetjournals.org)
  • functional channels form as homo- or hetero-tetramers of α -subunits ( Delmas and Brown, 2005 ). (aspetjournals.org)
  • Our patients presented with rapidly progressive dementia, memory problems, psychiatric symptoms, and movement disorders, and we considered all these symptoms as a presenting feature of sCJD at first, but the cerebrospinal fluid examination showed positive results for both the 14-3-3 protein and antibodies against NMDAR. (frontiersin.org)
  • The examination for the presence of NMDAR antibodies is necessary, even with the presence of 14-3-3 protein. (frontiersin.org)
  • Here, we described two patients first diagnosed with suspected sCJD presenting with rapidly progressive dementia, but the cerebrospinal fluid (CSF) examination showed positive results for both the 14-3-3 protein and antibodies against NMDAR. (frontiersin.org)
  • Smooth muscle cells express Kv7.4 and Kv7.5 voltage-dependent potassium channels, which have each been implicated as regulators of smooth muscle contractility, though they display different sensitivities to signaling via cAMP/protein kinase A (PKA) and protein kinase C (PKC). (aspetjournals.org)
  • Potassium two pore domain channel subfamily K member 16 is encoded by the gene KCNK16. (antibodiesinc.com)
  • This gene encodes a member of the potassium channel, voltage-gated, shab-related subfamily. (nih.gov)
  • Kvß1.1 is a member of the potassium channel, voltage-gated, shaker-related subfamily. (antibodiesinc.com)
  • Early immunomodulatory therapy should be considered, especially for patients with high titers of NMDAR antibodies. (frontiersin.org)
  • His antibody titers decreased to 152 pM 5 months after treatment and to 135 pM 6 months after treatment (reference range, 0-100 pM). (psychiatrist.com)
  • To analyze the surface expression of the channel we used HA-tagged THIK-2 and thus quantified the copy number of the channels at the cell membrane using an antibody-based assay. (uni-marburg.de)
  • The Proteintech guarantee covers Proteintech antibodies in any species and any application, including those not listed on the datasheet. (ptglab.com)
  • At Proteintech, we pride ourselves on our antibody quality, customer service and transparency. (ptglab.com)
  • Find KCNH2 Antibodies with a specific conjugate such as FITC, Biotin, HRP. (antibodies-online.com)
  • Bulk Order Inquiry for Anti-Kvbeta1.1 K+ Channel Antibody FL650 Conjugate ------- (please add any order requirements, including desired quantity, timing, etc. (antibodiesinc.com)
  • Voltage-gated potassium channel subunit beta-1 or Kvβ1.1 is encoded by the gene KCNAB1. (antibodiesinc.com)
  • Voltage-gated potassium channel subunit beta-1 (EC 1.1.1. (antibodiesinc.com)
  • This gene encodes a sodium-activated potassium channel subunit which is thought to function in ion conductance and developmental signaling pathways. (antikoerper-online.de)
  • Finally, it seems appropriate to consider the "sodium channel syndrome" (mutations in the gene of the α subunit of the sodium channel, SCN5A gene) as a single clinical entity that may manifest in a wide range of phenotypes, to thus have a better insight on these cardiac syndromes and potential outcomes for their clinical treatment. (bvsalud.org)
  • Objectives There have been reports of patients with antibodies to neuronal antigens misdiagnosed as sporadic Creutzfeldt-Jakob disease (sCJD). (bmj.com)
  • The antibody was generated using peptides enco. (warf.org)
  • Antineural antibodies serve as useful markers of these diseases, and their detection may help establish a diagnosis, support treatment decisions, aid prognostication, serve as a prerequisite for enrollment in clinical trials, and guide the search for an associated malignancy. (arupconsult.com)
  • Utilize our KCNH2 antibodies in your research endeavors for dependable KCNH2 detection. (antibodies-online.com)
  • Channelopathies are a group of genetic, autoimmune, or inflammatory conditions that alter cardiomyocyte ion channel function in a manner that predisposes to bradyarrhythmias or tachyarrhythmias in the absence of a structural heart disorder. (merckmanuals.com)
  • and inflammatory disorders producing cytokinins that affect cardiomyocyte ion channel function are increasingly being recognized. (merckmanuals.com)
  • Autoimmune antibody production and inflammatory cytokinins may also be responsible for temporal variability in arrhythmia propensities, such as the deleterious effects of fever in patients with Brugada syndrome. (merckmanuals.com)
  • Methods We reviewed 256 consecutive cases of sCJD seen in the National Prion Clinic, of whom 150 had sera previously referred for selected antibody tests. (bmj.com)
  • The efficacy of our antibodies is well-established, demonstrated through multiple methods. (antibodies-online.com)
  • The present invention provides antibodies that target glioblastoma stem-like cells and methods and kits for their use. (warf.org)
  • Methods: Whole cell recording patch clamp measurements in mammalian cells was used to analyze K2P channels mentioned above. (uni-marburg.de)
  • However, the frequency of misdiagnoses, or of antibodies in patients with subsequently confirmed sCJD, is not clear. (bmj.com)
  • Low titres of neuronal antibodies occur only rarely in suspected patients with sCJD and when present should be interpreted with caution. (bmj.com)
  • Voltage-gated potassium (Kv) channels represent the most complex class of voltage-gated ion channels from both functional and structural standpoints. (nih.gov)
  • Potassium channels represent the most complex class of voltage-gated ion channels from both functional and structural standpoints. (antikoerper-online.de)
  • Functional states of the sodium channel (closed, open, and inactivated) and their structure help to understand the cardiac regulation processes. (bvsalud.org)
  • Heart relaxation also stands out as an active process, dependent on the energetic output and on specific ion and enzymatic actions, with the role of sodium channel being outstanding in the functional process. (bvsalud.org)
  • These channels regulate nerve excitability. (medscape.com)
  • The prominence of Kv7 channels as regulators of electrical excitability may be ascribed to their unique properties, such as a very negative threshold of activation (negative to −50 mV), absence of time-dependent inactivation, and high sensitivity to signal transduction pathways that influence their probability of opening, either positively or negatively ( Delmas and Brown, 2005 ). (aspetjournals.org)
  • SIGNIFICANCE STATEMENT Kv7.4 and Kv7.5 channels are known signal transduction intermediates and drug targets for regulation of smooth muscle tone. (aspetjournals.org)
  • Testing for these antineural antibodies should be considered when patients experience subacute onset of new, unexplained neurologic symptoms. (arupconsult.com)
  • Four sequence-related potassium channel genes - shaker, shaw, shab, and shal - have been identified in Drosophila, and each has been shown to have human homolog(s). (nih.gov)
  • Short QT Interval Syndromes The short QT interval syndromes (SQTS) are extremely rare congenital or very rarely acquired disorders of cardiac ion channel function or regulation that shorten ventricular myocyte action potential. (merckmanuals.com)
  • These findings may improve our understanding of the roles of these channels in smooth muscle physiology and disease, particularly in conditions where Kv7.4 and Kv7.5 are differentially expressed. (aspetjournals.org)