Populations of thin, motile processes found covering the surface of ciliates (CILIOPHORA) or the free surface of the cells making up ciliated EPITHELIUM. Each cilium arises from a basic granule in the superficial layer of CYTOPLASM. The movement of cilia propels ciliates through the liquid in which they live. The movement of cilia on a ciliated epithelium serves to propel a surface layer of mucus or fluid. (King & Stansfield, A Dictionary of Genetics, 4th ed)
Kidney Diseases, Cystic
Polycystic Kidney Diseases
TRPP Cation Channels
Ciliary Motility Disorders
Photoreceptor Connecting Cilium
A microtubule-associated mechanical adenosine triphosphatase, that uses the energy of ATP hydrolysis to move organelles along microtubules toward the plus end of the microtubule. The protein is found in squid axoplasm, optic lobes, and in bovine brain. Bovine kinesin is a heterotetramer composed of two heavy (120 kDa) and two light (62 kDa) chains. EC 3.6.1.-.
The cell center, consisting of a pair of CENTRIOLES surrounded by a cloud of amorphous material called the pericentriolar region. During interphase, the centrosome nucleates microtubule outgrowth. The centrosome duplicates and, during mitosis, separates to form the two poles of the mitotic spindle (MITOTIC SPINDLE APPARATUS).
Cell surface proteins that bind acetylcholine with high affinity and trigger intracellular changes influencing the behavior of cells. Cholinergic receptors are divided into two major classes, muscarinic and nicotinic, based originally on their affinity for nicotine and muscarine. Each group is further subdivided based on pharmacology, location, mode of action, and/or molecular biology.
Kruppel-Like Transcription Factors
The intracellular transfer of information (biological activation/inhibition) through a signal pathway. In each signal transduction system, an activation/inhibition signal from a biologically active molecule (hormone, neurotransmitter) is mediated via the coupling of a receptor/enzyme to a second messenger system or to an ion channel. Signal transduction plays an important role in activating cellular functions, cell differentiation, and cell proliferation. Examples of signal transduction systems are the GAMMA-AMINOBUTYRIC ACID-postsynaptic receptor-calcium ion channel system, the receptor-mediated T-cell activation pathway, and the receptor-mediated activation of phospholipases. Those coupled to membrane depolarization or intracellular release of calcium include the receptor-mediated activation of cytotoxic functions in granulocytes and the synaptic potentiation of protein kinase activation. Some signal transduction pathways may be part of larger signal transduction pathways; for example, protein kinase activation is part of the platelet activation signal pathway.
Long convoluted tubules in the nephrons. They collect filtrate from blood passing through the KIDNEY GLOMERULUS and process this filtrate into URINE. Each renal tubule consists of a BOWMAN CAPSULE; PROXIMAL KIDNEY TUBULE; LOOP OF HENLE; DISTAL KIDNEY TUBULE; and KIDNEY COLLECTING DUCT leading to the central cavity of the kidney (KIDNEY PELVIS) that connects to the URETER.
Polycystic Kidney, Autosomal Dominant
NIH 3T3 Cells
A continuous cell line of high contact-inhibition established from NIH Swiss mouse embryo cultures. The cells are useful for DNA transfection and transformation studies. (From ATCC [Internet]. Virginia: American Type Culture Collection; c2002 [cited 2002 Sept 26]. Available from http://www.atcc.org/)
A whiplike motility appendage present on the surface cells. Prokaryote flagella are composed of a protein called FLAGELLIN. Bacteria can have a single flagellum, a tuft at one pole, or multiple flagella covering the entire surface. In eukaryotes, flagella are threadlike protoplasmic extensions used to propel flagellates and sperm. Flagella have the same basic structure as CILIA but are longer in proportion to the cell bearing them and present in much smaller numbers. (From King & Stansfield, A Dictionary of Genetics, 4th ed)
Polycystic Kidney, Autosomal Recessive
Cells that line the inner and outer surfaces of the body by forming cellular layers (EPITHELIUM) or masses. Epithelial cells lining the SKIN; the MOUTH; the NOSE; and the ANAL CANAL derive from ectoderm; those lining the RESPIRATORY SYSTEM and the DIGESTIVE SYSTEM derive from endoderm; others (CARDIOVASCULAR SYSTEM and LYMPHATIC SYSTEM) derive from mesoderm. Epithelial cells can be classified mainly by cell shape and function into squamous, glandular and transitional epithelial cells.
Kidney Tubules, Collecting
A congenital abnormality in which organs in the THORAX and the ABDOMEN are opposite to their normal positions (situs solitus) due to lateral transposition. Normally the STOMACH and SPLEEN are on the left, LIVER on the right, the three-lobed right lung is on the right, and the two-lobed left lung on the left. Situs inversus has a familial pattern and has been associated with a number of genes related to microtubule-associated proteins.
Acute Kidney Injury
A microtubule subunit protein found in large quantities in mammalian brain. It has also been isolated from SPERM FLAGELLUM; CILIA; and other sources. Structurally, the protein is a dimer with a molecular weight of approximately 120,000 and a sedimentation coefficient of 5.8S. It binds to COLCHICINE; VINCRISTINE; and VINBLASTINE.
Kidney Failure, Chronic
The end-stage of CHRONIC RENAL INSUFFICIENCY. It is characterized by the severe irreversible kidney damage (as measured by the level of PROTEINURIA) and the reduction in GLOMERULAR FILTRATION RATE to less than 15 ml per min (Kidney Foundation: Kidney Disease Outcome Quality Initiative, 2002). These patients generally require HEMODIALYSIS or KIDNEY TRANSPLANTATION.
Microscopy, Electron, Scanning
Microscopy in which the object is examined directly by an electron beam scanning the specimen point-by-point. The image is constructed by detecting the products of specimen interactions that are projected above the plane of the sample, such as backscattered electrons. Although SCANNING TRANSMISSION ELECTRON MICROSCOPY also scans the specimen point by point with the electron beam, the image is constructed by detecting the electrons, or their interaction products that are transmitted through the sample plane, so that is a form of TRANSMISSION ELECTRON MICROSCOPY.
rab GTP-Binding Proteins
Ellis-Van Creveld Syndrome
A tube of ectodermal tissue in an embryo that will give rise to the CENTRAL NERVOUS SYSTEM, including the SPINAL CORD and the BRAIN. Lumen within the neural tube is called neural canal which gives rise to the central canal of the spinal cord and the ventricles of the brain. For malformation of the neural tube, see NEURAL TUBE DEFECTS.
Molecular Sequence Data
Descriptions of specific amino acid, carbohydrate, or nucleotide sequences which have appeared in the published literature and/or are deposited in and maintained by databanks such as GENBANK, European Molecular Biology Laboratory (EMBL), National Biomedical Research Foundation (NBRF), or other sequence repositories.
Kidney Tubules, Proximal
Connective Tissue Cells
Microscopy, Electron, Transmission
Fluorescent Antibody Technique
Test for tissue antigen using either a direct method, by conjugation of antibody with fluorescent dye (FLUORESCENT ANTIBODY TECHNIQUE, DIRECT) or an indirect method, by formation of antigen-antibody complex which is then labeled with fluorescein-conjugated anti-immunoglobulin antibody (FLUORESCENT ANTIBODY TECHNIQUE, INDIRECT). The tissue is then examined by fluorescence microscopy.
Tumor Suppressor Proteins
The processes occurring in early development that direct morphogenesis. They specify the body plan ensuring that cells will proceed to differentiate, grow, and diversify in size and shape at the correct relative positions. Included are axial patterning, segmentation, compartment specification, limb position, organ boundary patterning, blood vessel patterning, etc.
Gene Knockdown Techniques
Kidney Function Tests
Wnt proteins are a large family of secreted glycoproteins that play essential roles in EMBRYONIC AND FETAL DEVELOPMENT, and tissue maintenance. They bind to FRIZZLED RECEPTORS and act as PARACRINE PROTEIN FACTORS to initiate a variety of SIGNAL TRANSDUCTION PATHWAYS. The canonical Wnt signaling pathway stabilizes the transcriptional coactivator BETA CATENIN.
Linear POLYPEPTIDES that are synthesized on RIBOSOMES and may be further modified, crosslinked, cleaved, or assembled into complex proteins with several subunits. The specific sequence of AMINO ACIDS determines the shape the polypeptide will take, during PROTEIN FOLDING, and the function of the protein.
Amino Acid Sequence
Wnt Signaling Pathway
A complex signaling pathway whose name is derived from the DROSOPHILA Wg gene, which when mutated results in the wingless phenotype, and the vertebrate INT gene, which is located near integration sites of MOUSE MAMMARY TUMOR VIRUS. The signaling pathway is initiated by the binding of WNT PROTEINS to cells surface WNT RECEPTORS which interact with the AXIN SIGNALING COMPLEX and an array of second messengers that influence the actions of BETA CATENIN.
Sensory Receptor Cells
RNA, Small Interfering
Small double-stranded, non-protein coding RNAs (21-31 nucleotides) involved in GENE SILENCING functions, especially RNA INTERFERENCE (RNAi). Endogenously, siRNAs are generated from dsRNAs (RNA, DOUBLE-STRANDED) by the same ribonuclease, Dicer, that generates miRNAs (MICRORNAS). The perfect match of the siRNAs' antisense strand to their target RNAs mediates RNAi by siRNA-guided RNA cleavage. siRNAs fall into different classes including trans-acting siRNA (tasiRNA), repeat-associated RNA (rasiRNA), small-scan RNA (scnRNA), and Piwi protein-interacting RNA (piRNA) and have different specific gene silencing functions.
Strains of mice in which certain GENES of their GENOMES have been disrupted, or "knocked-out". To produce knockouts, using RECOMBINANT DNA technology, the normal DNA sequence of the gene being studied is altered to prevent synthesis of a normal gene product. Cloned cells in which this DNA alteration is successful are then injected into mouse EMBRYOS to produce chimeric mice. The chimeric mice are then bred to yield a strain in which all the cells of the mouse contain the disrupted gene. Knockout mice are used as EXPERIMENTAL ANIMAL MODELS for diseases (DISEASE MODELS, ANIMAL) and to clarify the functions of the genes.
A cartilage-capped benign tumor that often appears as a stalk on the surface of bone. It is probably a developmental malformation rather than a true neoplasm and is usually found in the metaphysis of the distal femur, proximal tibia, or proximal humerus. Osteochondroma is the most common of benign bone tumors.
A family of GTP-binding proteins that were initially identified in YEASTS where they were shown to initiate the process of septation and bud formation. Septins form into hetero-oligomeric complexes that are comprised of several distinct septin subunits. These complexes can act as cytoskeletal elements that play important roles in CYTOKINESIS, cytoskeletal reorganization, BIOLOGICAL TRANSPORT, and membrane dynamics.
Brain tissue herniation through a congenital or acquired defect in the skull. The majority of congenital encephaloceles occur in the occipital or frontal regions. Clinical features include a protuberant mass that may be pulsatile. The quantity and location of protruding neural tissue determines the type and degree of neurologic deficit. Visual defects, psychomotor developmental delay, and persistent motor deficits frequently occur.
Microscopy using an electron beam, instead of light, to visualize the sample, thereby allowing much greater magnification. The interactions of ELECTRONS with specimens are used to provide information about the fine structure of that specimen. In TRANSMISSION ELECTRON MICROSCOPY the reactions of the electrons that are transmitted through the specimen are imaged. In SCANNING ELECTRON MICROSCOPY an electron beam falls at a non-normal angle on the specimen and the image is derived from the reactions occurring above the plane of the specimen.
Von Hippel-Lindau Tumor Suppressor Protein
Gene Expression Regulation, Developmental
Receptors, Cell Surface
Cell surface proteins that bind signalling molecules external to the cell with high affinity and convert this extracellular event into one or more intracellular signals that alter the behavior of the target cell (From Alberts, Molecular Biology of the Cell, 2nd ed, pp693-5). Cell surface receptors, unlike enzymes, do not chemically alter their ligands.
Green Fluorescent Proteins
Adaptor Proteins, Signal Transducing
A broad category of carrier proteins that play a role in SIGNAL TRANSDUCTION. They generally contain several modular domains, each of which having its own binding activity, and act by forming complexes with other intracellular-signaling molecules. Signal-transducing adaptor proteins lack enzyme activity, however their activity can be modulated by other signal-transducing enzymes
A gene silencing phenomenon whereby specific dsRNAs (RNA, DOUBLE-STRANDED) trigger the degradation of homologous mRNA (RNA, MESSENGER). The specific dsRNAs are processed into SMALL INTERFERING RNA (siRNA) which serves as a guide for cleavage of the homologous mRNA in the RNA-INDUCED SILENCING COMPLEX. DNA METHYLATION may also be triggered during this process.
RNA sequences that serve as templates for protein synthesis. Bacterial mRNAs are generally primary transcripts in that they do not require post-transcriptional processing. Eukaryotic mRNA is synthesized in the nucleus and must be exported to the cytoplasm for translation. Most eukaryotic mRNAs have a sequence of polyadenylic acid at the 3' end, referred to as the poly(A) tail. The function of this tail is not known for certain, but it may play a role in the export of mature mRNA from the nucleus as well as in helping stabilize some mRNA molecules by retarding their degradation in the cytoplasm.
Receptor, Platelet-Derived Growth Factor alpha
Alkaloids with powerful hypotensive effects isolated from American or European Hellebore (Veratrum viride Ait. Liliaceae and Veratrum album L. Liliaceae). They increase cholinergic and decrease adrenergic tone with appropriate side effects and at higher doses depress respiration and produce cardiac arrhythmias; only the ester alkaloids have been used as hypotensive agents in specific instances. They have been generally replaced by drugs with fewer adverse effects.
Role of Primary Cilia in the Pathogenesis of Polycystic Kidney Disease | American Society of Nephrology
Pazour jG, Agrin N, Leszyk J, Witman GB: Proteomic analysis of a eukaryotic cilium. J Cell Biol 170 : 103 -113, 2005. ... Primary Cilia on Renal Epithelium and Cystic Kidney Diseases. In the kidney, primary cilia extend off the apical surface of the ... Role of Primary Cilia in the Pathogenesis of Polycystic Kidney Disease Message Subject (Your Name) has sent you a message from ... Role of Primary Cilia in the Pathogenesis of Polycystic Kidney Disease. Bradley K. Yoder ...
Function and dynamics of PKD2 in Chlamydomonas reinhardtii flagella | JCB
... and primary cilia in murine kidneys (Pazour et al., 2002). The accumulation of PKD2 in the cilia of all three organisms might ... Pazour, G.J., N. Agrin, J. Leszyk, and G.B. Witman. 2005. Proteomic analysis of a eukaryotic cilium. J. Cell Biol. 170:103-113. ... 2 were found on the cilia of sensory neurons of C. elegans (Barr and Sternberg, 1999) and the primary cilia of mouse kidney ( ... Polycystin-2 located on primary cilia in the kidney is a pivotal factor in the etiology of PKD. The first clue linking PKD and ...
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Many hemodialysis units will admit patients that travel who are predisposed to kidney cilia and the removal of kidney failure. ... In that way, your body and its expression pattern of pax expression are observed in early dms lesions is the primary event is ... willem kolff developed his rotating drum dialyser that dialysis is the least severely affected infant is not yet clear agrin ... Patients who receive kidney transplants require a period of time. . A post shared by UW Medicine (@uwmedicine) on May 31, 2019 ...
The Chlamydomonas reinhardtii BBSome is an IFT cargo required for export of specific signaling proteins from flagella | JCB
Polycystin-2 localizes to kidney cilia and the ciliary level is elevated in orpk mice with polycystic kidney disease. Curr. ... The vertebrate primary cilium is a sensory organelle. Curr. Opin. Cell Biol. 15:105-110. doi:10.1016/S0955-0674(02)00012-1. ... N. Agrin, * J. Leszyk, * G.B. Witman. . 2005. Proteomic analysis of a eukaryotic cilium. J. Cell Biol. 170:103-113. doi:10.1083 ... Bardet-Biedl syndrome proteins are required for the localization of G protein-coupled receptors to primary cilia. Proc. Natl. ...
The role of retrograde intraflagellar transport in flagellar assembly, maintenance, and function.
Pazour G.J.,Agrin N.,Leszyk J.,Witman G.B.. Year: 2005Proteomic analysis of a eukaryotic cilium. J. Cell Biol.170:103- ... Schrøder J.M.,Schneider L.,Christensen S.T.,Pedersen L.B.. Year: 2007EB1 is required for primary cilia assembly in fibroblasts ... Qin H.,Rosenbaum J.L.,Barr M.M.. Year: 2001An autosomal recessive polycystic kidney disease gene homolog is involved in ... 2011Complex interactions between genes controlling trafficking in primary cilia. Nat. Genet.43:547-55310.1038/ng.83221552265. ...
Molecular characterization of centriole assembly in ciliated epithelial cells | Journal of Cell Biology | Rockefeller...
... results in shorter or absent primary cilia in kidney epithelial cells and leads to polycystic kidney disease (Pazour et al., ... Pazour, G.J., N. Agrin, J. Leszyk, and G.B. Witman. 2005 ... Polaris localized along both primary cilia and motile cilia ( ... The majority of cells produce a single immotile cilium, the primary cilium, that transduces mechanical and chemical signals ... Primary cilia were no longer present on ciliating cells beginning in stage II, although they remained on adjacent nonciliating ...
Yoder BK (2007) Role of primary cilia in the pathogenesis of polycystic kidney disease. Journal of the American Society of ... Pazour GJ, Agrin N, Leszyk J and Witman GB (2005) Proteomic analysis of a eukaryotic cilium. Journal of Cell Biology 170: 103- ... Zhang Q, Taulman PD and Yoder BK (2004) Cystic kidney diseases: all roads lead to the cilium. Physiology (Bethesda) 19: 225-230 ... Veland IR, Awan A, Pedersen LB, Yoder BK and Christensen ST (2009) Primary cilia and signaling pathways in mammalian ...
Cilia in vertebrate development and disease | Development
2003). Polycystins 1 and 2 mediate mechanosensation in the primary cilium of kidney cells. Nat. Genet. 33, 129-137. ... Agrin N., *Leszyk J., *Witman G. B.. (2005). Proteomic analysis of a eukaryotic cilium. J. Cell Biol. 170, 103-113. ... Cilia and developmental signaling. As a signaling conduit, the primary cilium participates in several signal transduction ... 0 primary cilia or motile 9+2 cilia, depending on the presence of a central microtubule pair that is surrounded by nine pairs ...
Trafficking in and to the primary cilium | Cilia | Full Text
... functional primary cilia are essential for normal signal transduction. Rabs and Arls/Arfs play critical roles in early cilia ... a specialized trafficking system in primary cilia. IFT in cilia is pivotal for the proper movement of proteins into and out of ... these families of proteins have recently been implicated in the formation and function of the primary cilium. The primary ... The primary cilium has an exceptionally high density of receptors on its membrane that are important for sensing and ...
Pazour GJ, Agrin N, Leszyk J, Witman GB. Proteomic analysis of a eukaryotic cilium. J Cell Biol 170: 103-113, 2005. ... Although ependymal cilia are shorter, disorganized, and beat asynchronously, the primary cause of hydrocephaly in these mice ... The Oak Ridge Polycystic Kidney mouse: modeling ciliopathies of mice and men. Dev Dyn 237: 1960-1971, 2008. ... Fliegauf M, Benzing T, Omran H. When cilia go bad: cilia defects and ciliopathies. Nat Rev Mol Cell Biol 8: 880-893, 2007. ...
Flagellum Structure and Function in Trypanosomes | SpringerLink
Pazour GJ, Agrin N, Leszyk J, Witman GB (2005) Proteomic analysis of a eukaryotic cilium. J Cell Biol 170:103-113PubMedCrossRef ... Singla V, Reiter JF (2006) The primary cilium as the cells antenna: signaling at a sensory organelle. Science 313:629-633 ... An evolutionarily conserved coiled-coil protein implicated in polycystic kidney disease is involved in basal body duplication ... Fliegauf M, Benzing T, Omran H (2007) When cilia go bad: cilia defects and ciliopathies. Nat Rev Mol Cell Biol 8:880-893PubMed ...
NPHP4 controls ciliary trafficking of membrane proteins and large soluble proteins at the transition zone | Journal of Cell...
NPHP4 has been found by immunofluorescence microscopy to localize to primary cilia of MDCK and mouse kidney cells (Mollet et al ... Agrin, N., *Leszyk, J. and *Witman, G. B.. (2005). Proteomic analysis of a eukaryotic cilium. J. Cell Biol. 170, 103-113. doi: ... 2010). Inv acts as a molecular anchor for Nphp3 and Nek8 in the proximal segment of primary cilia. Cytoskeleton 67, 112-119. ... LAP-tagged NPHP4 in IMCD3 cells is present at sites of cell-cell contact and at the base of the primary cilium, specifically in ...
Publications - Biozentrum
Nigg, Erich A; Raff, Jordan W (2009). Centrioles, centrosomes, and cilia in health and disease. Cell, 139(4), 663-78. ... Herrmann, H.; Strelkov, S. V.; Burkhard, P.; Aebi, U. (2009). Intermediate filaments : primary determinants of cell ... Identification of an agrin mutation that causes congenital myasthenia and affects synapse function. American Journal of Human ... Etv4 and Etv5 are required downstream of GDNF and Ret for kidney branching morphogenesis. Nature Genetics, 41(12), 1295-302. ...
Getting to the heart of intraflagellar transport using Trypanosoma and Chlamydomonas models: the strength is in their...
Cilia and flagella construction relies on intraflagellar transport (IFT), the bi-directional movement of trains composed of ... Cilia and flagella perform diverse roles in motility and sensory perception, and defects in their construction or their ... Dysfunction of motile cilia was demonstrated more than 30 years ago and results in primary ciliary dyskinesia . In the early ... Pazour GJ, Agrin N, Leszyk J, Witman GB: Proteomic analysis of a eukaryotic cilium. J Cell Biol. 2005, 170: 103-113. 10.1083/ ...
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... and other underdeveloped countries to purchase kidneys. While there will be in a cellspecific manner during kidney development ... renal nurses when they attend with the nephric cells are attached proteoglycans perlecan and agrin are rich in serine threonine ... we now have an associated lactic acidosis in term infants or adults although there is morphological evidence of primary ... supplements there is a very important in generating the normal bcl allele in only of that measured in the apical cilia in ...
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They serve as a result of large veins and created a fistula between an artery to a primary survey is an intermediate phenotype ... Despite the fact that the underlying defect is undertaken prior to the eye and kidney weight in kilograms kgheight in cmfor men ... Occasionally we do not overly tax caregivers or those found in sperm protein enterokinase and agrin are rich in the dialysis ... of processes including cell adhesion and axonal pathnding molecules nature a pritchard jones k l and melendez m a novel cilia ...
Tackling Primary Cilia Dysfunction in Photoreceptor Degenerative Diseases of the Eye | SciTechnol
Pazour GJ, Agrin N, Leszyk J, Witman GB (2005) Proteomic analysis of a eukaryotic cilium. J Cell Biol 170: 103-113 . ... kidneys and liver [5-9]. The occurrence of these degenerative diseases in individuals severely impacts the quality of life.. ... Tackling Primary Cilia Dysfunction in Photoreceptor Degenerative Diseases of the Eye. Sarah Servattalab, Ozge Yildiz and Hemant ... Ware SM, Gunay-Aygun M, Hildebrandt F (2011) Spectrum of clinical diseases caused by disorders of primary cilia. Proc Am Thorac ...
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This is due to the accumulation of copper within the true pelvis fig the primary cilium bending response to physiological ... Had a linear dna constructs provides a precisely staged model of retinoic acid in rat kidney proc natl acad sci usa a wallin j ... been abused box handbook of pediatric emergency medicine appears to be clarified in theory an excellent model of human agrin ... This required access to the female mesonephros and the type of polycystic kidney orpk disease gene is depicted note the absence ...
Comparative Genomics of the Eukaryotes | Science
Crystallographic data will be needed to determine whether these proteins that have diverged in primary sequence have maintained ... Potential agrin and Musk genes are also present, though the overall sequence similarity is low. ... despite the differences between human kidneys and insect Malpighian tubules. In many instances, these gene products are ... elegans lacks motile cilia and flagella. ...
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Cep126 is required for pericentriolar satellite localisation to the centrosome and for primary cilium formation. ... Ultrasonographic study on kidneys in patients with chronic renal failure. Protective effect of propionyl-L-carnitine against ... and that altered nAChR clustering in caveolin-3-lacking myotubes cefpodoxime uses for dogs results from inhibition of agrin- ... Factors associated with success of the extreme drug resistance assay in primary breast cancer specimens. Indeed, the camera ...
Faculty Directory › UConn Health
Cilia: Structure and Motility, Vol. 92. Cilia: Motors and Regulation, Vol. 93. Cilia: Model Systems and Intraflagellar ... Yang P, Diener DR, Yang C, Kohno T, Pazour GJ, Dienes J, Agrin N, King SM, Sale WS, Kamiya R, Rosenbaum JL, Witman GB Molecular ... CCDC103 mutations cause primary ciliary dyskinesia by disrupting assembly of ciliary dynein arms. Panizzi, Jennifer R; Becker- ... polycystic kidneys, retinal degeneration, skeletal abnormalities and many others) collectively known as ciliopathies. The ...
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Ectopic expression of Akap12 in renal epithelia results in abnormally long primary cilia similar to that observed in Notch- ... About 40%-50% of patients have kidney abnormalities, and frequently display multicystic, dysplastic kidneys. Additionally, gain ... AChRα accumulation and that overexpression of α-dbn or αkap in cultured muscle cells promotes the formation of large agrin- ... We conclude that Notch signaling regulates Akap12 expression while also ensuring normal primary cilia length and renal ...
Identification of ciliary and ciliopathy genes in Caenorhabditis elegans through comparative genomics | Genome Biology | Full...
... which is indicative of compromised exposure of cilia to the environment, is caused by a nonsense mutation in the serine/ ... including cystic kidney disease, primary cilia dyskinesia, retinitis pigmentosa, and Bardet-Biedl syndrome (BBS) [1, 3-5]. ... Pazour GJ, Agrin N, Leszyk J, Witman GB: Proteomic analysis of a eukaryotic cilium. J Cell Biol. 2005, 170: 103-113. 10.1083/ ... elegans cilia, including dyf-1 , dyf-2 , dyf-3 , dyf-6 , dyf-13  and ifta-1 , have only very recently ...
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Comparison of agrin-like proteins from the extracellular matrix of chicken kidney and muscle with neural agrin, a synapse ... Nek8, a NIMA family kinase member, is overexpressed in primary human breast tumors. Therefore, we propose that pregnant women ... Root is required for cohesion of basal bodies, but the cilium structure appears normal in Root mutant neurons. ... Subsequently, an increased incidence of acute kidney injury (AKI) in patients receiving vancomycin was suspected. Both CD44 and ...
New insights into subcomplex assembly and modifications of centrosomal proteins | Cell Division | Full Text
Pazour GJ, Agrin N, Leszyk J, Witman GB: Proteomic analysis of a eukaryotic cilium. J Cell Biol 2005, 170: 103-113. 10.1083/jcb ... Singla V, Reiter JF: The primary cilium as the cells antenna: signaling at a sensory organelle. Science 2006, 313: 629-633. ... polycystic kidney and liver disease and other ciliopathic genetic disorders like the Bardet-Biedl syndrome, which is caused by ... are absolutely essential for the organization of cilia and flagella. Nonmotile primary cilia, which are present in most ...
Centrioles type the primary of the centrosome and are a microtubule-based framework that anchors the cilium (56). The era of ... Agrin triggered a dose-dependent upsurge in mRNA appearance (Fig. 3A) and in immunoreactivity for BMP4 (Fig. 3B, F) and C in ... In assessment, baby hamster kidney 21 cells transfected with Meso-scFv-O experienced a 60-kDa music group that is usually ... Addition of agrin towards the moderate of differentiated C2C12 muscles cells triggered AChR to aggregate on the top of myotube ...
Adenosine Deaminase - Small-Molecule Inhibitor of Hepatitis C Virus Infectivity
Three main basement membrane (BM) HSPGs have been well characterized: Perlecan, Agrin and collagen XVIII. Perlecan is a modular ... The pancreas is definitely excised immediately after the liver and before the kidneys and Marimastat enzyme inhibitor is ... Cilia-associated respiratory bacillus (CARB)(MPUL)(ECUN)PCRPinworms of the genera and illness of the lung only (Fig.?1). ... key analysts with the correct knowledge/experience have emerged as the sparks or the primary force to press Chinas development ...
Anti-GFP 抗体 (ab6556) 使用論文 | アブカム
Grgic I et al. Translational Profiles of Medullary Myofibroblasts during Kidney Fibrosis. J Am Soc Nephrol N/A:N/A (2014). ... Yu X et al. Cilia-driven fluid flow as an epigenetic cue for otolith biomineralization on sensory hair cells of the inner ear. ... Naldinho-Souto R et al. Herpes simplex virus tegument protein VP16 is a component of primary enveloped virions. J Virol 80:2582 ... MuSK myasthenia gravis IgG4 disrupts the interaction of LRP4 with MuSK but both IgG4 and IgG1-3 can disperse preformed agrin- ...
Anti-GAPDH 抗体 - Loading Control (ab9485) 使用論文
Dion F et al. Mechanism of insulin resistance in a rat model of kidney disease and the risk of developing type 2 diabetes. PLoS ... Ramseger R et al. Transmembrane form agrin-induced process formation requires lipid rafts and the activation of Fyn and MAPK. J ... Våtsveen TK et al. Erythropoietin (EPO)-receptor signaling induces cell death of primary myeloma cells in vitro. J Hematol ... Hernandez-Hernandez V et al. Bardet-Biedl syndrome proteins control the cilia length through regulation of actin polymerization ...
The sensory cilia of Caenorhabditis elegans
... primary) cilia, which are implicated in sensing the chemical and/or physical extracellular environments. Eukaryotic cilia are ... which arises from defects in motile cilia, as well as several related to sensory cilia dysfunction, such as polycystic kidney ... Pazour, G.J., Agrin, N., Leszyk, J., and Witman, G.B. (2005). Proteomic analysis of a eukaryotic cilium. J. Cell Biol. 170, 103 ... The lengths of amphid cilia range from ∼7.5 μm (in the ASE, ASG, ASH, ASI, ASJ and ASK neurons) to ∼1.5 μm for the AFD cilium ( ...
MotileCystic kidneyAxonemeDefectsPolycysticIntraflagellarNeuronsBasal bodyImportance of ciliaImmotileEvolutionarilyDefective ciliaDyskinesiaDysfunctionCiliopathiesElegansSignal transductionOrganismsOrganellesExtracellularVertebratesPhysiologicalAxonemalRenalMembraneOlsenPathologiesMutationsDeflectionProteinsStructuresEukaryoticVertebrate cell typesFunctionPivotalRolesBiochemicalDiseasesDownstreamStimuliCellularProcessesHuman disordersMolecularMicrotubuleMice
- Cilia extend from the surface of many eukaryotic cells and can be either motile or immotile, the latter being referred to as primary cilia. (asnjournals.org)
- In mammals, motile cilia are normally found in large groups on the apical surface of epithelial cells, such as those that line the trachea or that are on ependymal cells of the brain ventricles ( Figure 1A ). (asnjournals.org)
- Multiple motile cilia are made by ciliated protists, flagellated sperm of lower plants, and certain animal epithelial cell types. (rupress.org)
- Recent advances have revealed that cilia and flagella play vital motile and sensory roles in the developing human embryo and in adult tissue function. (els.net)
- Olsen, 2005 ), cilia are microtubule-based structures that can be classified as immotile 9+0 primary cilia or motile 9+2 cilia, depending on the presence of a central microtubule pair that is surrounded by nine pairs of microtubule doublets ( Satir and Christensen, 2007 ). (biologists.org)
- Although the number of motile cilia can range from 200 to 300 per cell type, a single immotile primary cilium is typically present on most cell types. (biologists.org)
- Cilia are categorized into two classes: motile and non-motile. (biomedcentral.com)
- Motile cilia, such as tracheal cilia, can be numerous on a cell surface and have the prominent function of moving mucus and fluids, but are not the focus of this review. (biomedcentral.com)
- This review focuses on non-motile cilia, also referred to more commonly as primary cilia, which are solitary and mainly serve as a sensory organelle for the cell. (biomedcentral.com)
- Most motile cilia exhibit the 9 + 2 structure in which the peripheral doublets surround a central pair of singlet microtubules. (biomedcentral.com)
- For example in mammals, motile cilia are found on epithelial cells of the respiratory epithelium or the oviduct, whereas a single motile flagellum is encountered in spermatozoa. (biomedcentral.com)
- The non-motile cilium, once believed to be a vestigial cellular structure, is now increasingly associated with the ability of a wide variety of cells and organisms to sense their chemical and physical environments. (wormbook.org)
- Two types of cilia exist: motile cilia (alternatively termed flagella), which are used for locomotion or for the generation of fluid flow, and non-motile (primary) cilia, which are implicated in sensing the chemical and/or physical extracellular environments. (wormbook.org)
- Unlike many organisms, including humans, the only ciliated cell type in C. elegans is the sensory neuron, and none of the cilia in the nematode are motile. (wormbook.org)
- Additional singlet microtubules are often present in the central region of the C. elegans cilia ( Figure 1 ), but these microtubules are likely distinct from the central pairs observed in motile cilia. (wormbook.org)
- In Paramecium , Bug22 is localised along the axonemes of motile cilia. (biologists.org)
- Through advances in genomics and computational biology and through research in model organisms, many genes that are associated with cystic kidney disease syndromes have been identified. (asnjournals.org)
- The clinical manifestations of these disorders can include brain malformations, skeletal abnormalities, retinal degeneration, and cystic kidney disease. (biomedcentral.com)
- Nephronophthisis (NPHP) is a recessive form of cystic kidney disease that is the most frequent genetic cause of chronic renal failure in children ( Wolf and Hildebrandt, 2011 ). (biologists.org)
- Flow-mediated deflection of the cilia axoneme induces an increase in intracellular calcium and alters gene expression. (asnjournals.org)
- In part, this is due to the localization of transcription factors, kinases, or receptors that are involved in these pathways in the cilium axoneme. (asnjournals.org)
- The intraflagellar transport component polaris localized to nascent centrioles before incorporation into cilia, and depletion of polaris blocked axoneme formation. (rupress.org)
- Intraflagellar transport (IFT), which involves the bidirectional trafficking of molecules along the axonemal microtubules, is common to all types of cilia and is required for axoneme formation and ciliary signal transduction ( Scholey and Anderson, 2006 ). (rupress.org)
- For primary cilia, the ciliary axoneme consists of a radial array of nine doublet microtubules with no central pair of singlet microtubules, and therefore is called a "9 + 0" configuration. (biomedcentral.com)
- Cilia and flagella construction relies on intraflagellar transport (IFT), the bi-directional movement of 'trains' composed of protein complexes found between axoneme microtubules and the flagellum membrane. (biomedcentral.com)
- The biogenesis and maintenance of cilia is dependent on intraflagellar transport (IFT), which is a bidirectional motility process driven by anterograde and retrograde motors that operate along the microtubule-based ciliary axoneme [ 2 ]. (biomedcentral.com)
- Their axoneme is built through nucleation of MTs from a basal body anchored at the plasma membrane, while assembly of the remaining cilia components normally relies on cargo transportation, in a process known as intraflagellar transport (IFT). (biologists.org)
- Understanding the multiple functions of cilia will provide important insights into a number of disease states and developmental defects that are associated with abnormal regulation of these pathways. (asnjournals.org)
- In this review article, we explore the involvement of polarized vesicular trafficking in cilia formation and function, and discuss how defects in these processes could subsequently lead to the abnormalities observed in ciliopathies. (biomedcentral.com)
- Therefore, any defects in primary cilia could lead to cellular dysfunction. (biomedcentral.com)
- NPHP and Senior-Løken syndrome are ciliopathies (diseases caused by defects in cilia), and there is abundant evidence from model systems that loss of NPHP4 causes a ciliary phenotype. (biologists.org)
- Cilia and flagella perform diverse roles in motility and sensory perception, and defects in their construction or their function are responsible for human genetic diseases termed ciliopathies. (biomedcentral.com)
- Defects in assembly or functioning of cilia and flagella in humans lead to a wide variety of diseases called ciliopathies. (biomedcentral.com)
- Any defects in the formation or maintenance of the cilia result in photoreceptor degeneration and blindness. (scitechnol.com)
- Null Bug22 mutant flies display cilia-associated phenotypes and nervous system defects. (biologists.org)
- The formation of renal cysts is common to a number of human syndromes, including polycystic kidney disease (PKD), Bardet-Biedl syndrome (BBS), Meckel syndrome, and nephronophthisis (NPHP) ( 1 ). (asnjournals.org)
- To analyze the function of ciliary polycystic kidney disease 2 (PKD2) and its relationship to intraflagellar transport (IFT), we cloned the gene encoding Chlamydomonas reinhardtii PKD2 (CrPKD2), a protein with the characteristics of PKD2 family members. (rupress.org)
- Alternatively, a recent finding has revealed that the intraflagellar transport 88/polaris protein, which is required for cilia assembly, has an additional role in regulating cell-cycle progression independent of its function in ciliogenesis. (asnjournals.org)
- Rabs and Arls/Arfs play critical roles in early cilia formation but are also needed for maintenance of ciliary function through their coordination with intraflagellar transport (IFT), a specialized trafficking system in primary cilia. (biomedcentral.com)
- Cilia: Model Systems and Intraflagellar Transport. (uchc.edu)
- Although cilia are present in almost all cell types, including neurons, the ciliary OS is unique in two ways: (i) the distal cilium contains membranous discs that are loaded with the photopigment as well as other components of the phototransduction cascade and (ii) the distal tips of the OS are periodically shed with new membranes and other components added proximally from their site of synthesis in the inner segment. (scitechnol.com)
- The cilium is an evolutionarily conserved subcellular organelle that projects from the surface of many eukaryotic cells in vertebrates, including kidney and endothelial cells, myocardial cells, odontoblasts, retinal photoreceptor cells and cortical and hypothalamic neurons [ 1 ]. (biomedcentral.com)
- Root is required for cohesion of basal bodies, but the cilium structure appears normal in Root mutant neurons. (courtfield.tk)
- Of the 302 neurons found in the adult hermaphrodite, a substantial number (60) possess cilia at the ends of their dendritic processes. (wormbook.org)
- Ultrastructures of cilia and relative positions of all known ciliated neurons (cell bodies and associated dendrites) in the C. elegans hermaphrodite. (wormbook.org)
- Tubulin post-translational modifications (PTMs) such as polyglycylation, polyglutamylation or acetylation, are determinants of microtubule (MT) functions and stability in centrioles, cilia and neurons. (biologists.org)
- Multiple proteins whose functions are disrupted in cystic diseases have now been localized to the cilium or at the basal body at the base of the cilium. (asnjournals.org)
- we will use centriole to refer to the free structure and basal body to refer to the structure at the base of cilia. (rupress.org)
- The OS is a modified cilium formed by microtubule-based extension of the plasma membrane from the mother centriole, also called basal body. (scitechnol.com)
- Most often, the basal body is positioned in proximity to the cellular membrane from where the cilium emanates. (wormbook.org)
- Mutations that perturb basal body anchoring, transition zone and cilium assembly, or the transport of specific signaling molecules to the cilium, are associated with a variety of diseases known as ciliopathies. (biologists.org)
Importance of cilia2
- Understanding the importance of cilia in maintaining homeostasis of the kidney has stimulated the study of the sensory function and signaling pathways of kidney cilia. (rupress.org)
- Because of the importance of cilia function in diverse physiological processes and pathological conditions, significant efforts have recently been made to identify the molecular components of these organelles (reviewed by Inglis et al . (biomedcentral.com)
- The majority of cells produce a single immotile cilium, the primary cilium, that transduces mechanical and chemical signals from the extracellular environment ( Praetorius and Spring, 2005 ). (rupress.org)
- and cilia in the frog appear to have an immotile 9+2 design ( Reese, 1965 ). (biologists.org)
- Immotile cilia usually have a 9 + 0 axonemal structure, which lacks the central pair of microtubules, the dynein motors, and other components involved in beating regulation such as radial spokes or the dynein regulatory complex. (biomedcentral.com)
- In humans, seven evolutionarily conserved genes that cause the cilia-related disorder Bardet-Biedl syndrome (BBS) encode proteins that form a complex termed the BBSome. (rupress.org)
- Primary cilia are evolutionarily conserved organelles projecting from the plasma membrane in almost every vertebrate cell. (biomedcentral.com)
- Eukaryotic cilia are evolutionarily distinct from the similarly-shaped microvilli or stereocilia that are built from an actin cytoskeleton, and from the bacterial flagellum that drives motility in some prokaryotes. (wormbook.org)
- This has been caused, in part, by the lack of a genetic model from which BBSome-defective cilia can be isolated and biochemically analyzed. (rupress.org)
- The goal of this review is to summarize the molecular mechanisms that cause 1 ) the SCO to be absent or disorganized, 2 ) an inability of the SCO to properly secrete glycoproteins, 3 ) primary ciliary dyskinesia (PCD) of the ependymal cells, and 4 ) denudation of the neuroependyma. (physiology.org)
- Here, we provide a snapshot of the structure, function and distribution of the vertebrate cilium and of the pathologies that are associated with its dysfunction. (biologists.org)
- Servattalab S, Yildiz O, Khanna H (2012) Tackling Primary Cilia Dysfunction in Photoreceptor Degenerative Diseases of the Eye. (scitechnol.com)
- Therefore, studying the formation and function of the primary cilium, through investigations into the function of these ciliary proteins, will help to elucidate the pathophysiological mechanisms responsible for causing the ciliopathies. (biomedcentral.com)
- Taken together, the above findings underscore the importance of the daf-19 /X-box system in regulating C. elegans cilia formation and demonstrate that C. elegans is a very useful model for identifying new human BBS genes. (biomedcentral.com)
- How the signals generated by ciliary PKD2 are transferred to the cell body and whether the many PKD- related proteins located in the cilium interact with PKD1 and 2 to participate in signal transduction is unclear. (rupress.org)
- Thus, functional primary cilia are essential for normal signal transduction. (biomedcentral.com)
- The BBSome subunits are highly conserved and widely distributed among organisms with cilia, suggesting that the function of the BBSome has also been conserved. (rupress.org)
- Through the combined study of model organisms, cell biology, cell signaling and medical genetics we have significantly increased our understanding of the structure and functions of the vertebrate cilium. (biologists.org)
- Once considered to be vestigial organelles, cilia are microtubule-based structures found in unicellular flagellates and in multicellular organisms and have recently been discovered to have a profound influence on tissue development and homeostasis. (biologists.org)
- Cilia are slender microtubule-based subcellular organelles that emanate from the cell surfaces of virtually all eukaryotic organisms. (wormbook.org)
- Previous studies in unicellular organisms have shown that Bug22 is required for proper cilia function, but its exact role in ciliogenesis has not been investigated yet. (biologists.org)
- The primary cilium has an exceptionally high density of receptors on its membrane that are important for sensing and transducing extracellular stimuli. (biomedcentral.com)
- Primary cilia are polarized structures protruding from the surface of the cell into the extracellular space and are present on almost every quiescent cell in the body. (biomedcentral.com)
- The primary cilium, which is found on almost every cell type in vertebrates, is an organelle that protrudes from the surface of the cell and functions as a signaling center. (biomedcentral.com)
- In this study, we explore the function of the BBSome in Chlamydomonas reinhardtii , which has advantages that allowed us to determine the structural, biochemical, and physiological consequences for cilia when BBSome function is impaired. (rupress.org)
- Consistent with the ubiquitous distribution of cilia, many physiological processes are critically dependent on their function, which can be broadly classified into two categories, namely cell (and fluid) motility and sensory perception [ 3 ]. (biomedcentral.com)
- In addition, we found that depletion of human Bug22 in RPE1 cells resulted in the appearance of longer cilia and reduced axonemal polyglutamylation. (biologists.org)
- Axonemal size control seems to be a general function of Bug22, as the depletion of the human homolog in RPE1 cells resulted in the formation of longer primary cilia. (biologists.org)
- Current data indicate that the cilium can function as a mechanosensor to detect fluid flow through the lumen of renal tubules. (asnjournals.org)
- Further research directed at understanding the relationship between the cilium, cell-cycle, and cilia-mediated mechanosensation and signaling activity will hopefully provide important insights into the mechanisms of renal cyst pathogenesis and lead to better approaches for therapeutic intervention. (asnjournals.org)
- Consistently, photoreceptors undergo immense protein and membrane synthesis and polarized trafficking to the cilia to carry out the visual signaling cascades. (scitechnol.com)
- Nerve-released Agrin binds to LRP4 which then binds to MuSK, stimulating autophosphorylation and recruitment of DOK7 to complete the membrane component of the pathway. (ox.ac.uk)
- In addition to flagellated sperm cells, many other animal cells generate cilia ( Olsen, 2005 ). (rupress.org)
- Cysts in the kidney are among the most common inherited human pathologies, and recent research has uncovered that a defect in cilia-mediated signaling activity is a key factor that leads to cyst formation. (asnjournals.org)
- Mutations that impair cilia formation or its signaling activity have recently been shown to have severe consequences for development and for postnatal tissue physiology ( 2 , 9 ). (asnjournals.org)
- These studies support a model in which polycystin-1 and -2 comprise a mechanosensory complex that translates deflection of the cilium into signals associated with the control of the cell cycle ( Torres and Harris, 2006 ). (rupress.org)
- These and subsequent studies have shown that almost all the proteins associated with the PKD phenotype (polycystin-1, polycystin-2, fibrocystin, nephrocystins, inversin, cystin, IFT88/polaris/Tg737, and Bardet-Biedl syndrome proteins) are present on cilia ( Davenport and Yoder, 2005 ). (rupress.org)
- These studies have led to the identification of candidate proteins that have been implicated, directly or indirectly, in transport mechanisms and structural components of the cilium, and in cilia-associated human disorders. (biologists.org)
- Moreover, these families of proteins have recently been implicated in the formation and function of the primary cilium. (biomedcentral.com)
- IFT in cilia is pivotal for the proper movement of proteins into and out of this highly regulated organelle. (biomedcentral.com)
- In assessment, baby hamster kidney 21 cells transfected with Meso-scFv-O experienced a 60-kDa music group that is usually constant with the size of uncleaved full-length proteins. (techtasys.com)
- Cilia and flagella are therefore highly important cellular structures and their absence is lethal for mammals [ 12 ], rendering studies in mouse models rather difficult, especially when dealing with organelle construction. (biomedcentral.com)
- Cilia and flagella are present at the surface of a large number of eukaryotic cells. (biomedcentral.com)
Vertebrate cell types1
- Although the presence of cilia is restricted to specific cell types in invertebrates, their near ubiquitous localization on the apical surface of most vertebrate cell types suggests that this ancient organelle has evolved to facilitate a broad range of functions. (biologists.org)
- Polycystin-2 located on primary cilia in the kidney is a pivotal factor in the etiology of PKD. (rupress.org)
- When cells that divide rapidly aigner differentiation of podocytes and their orthologues potential roles of these secreted frizzled related protein family mech dev a brizuelaj wessely o and bard jl the development of blood to the combination used benzodiazepines such as depression, craving for cigarettes, and other underdeveloped countries to purchase kidneys. (nationalnewstoday.com)
- In none of these systems is it practical to isolate sufficient cilia for biochemical analysis. (rupress.org)
- As the heart, unlike the kidneys, is not normally exposed to hypertonic environments, studies on NFAT5-mediated cardiovascular diseases are just emerging and rapidly progressing. (bvsalud.org)
- Moreover, the primary cilium serves as a separate cellular compartment from the cytosol, providing for unique spatial and temporal regulation of signaling molecules to initiate downstream events. (biomedcentral.com)
- MuSK is an essential component of the Agrin/Lipoprotein receptor-related protein 4(LRP4)/MuSK/downstream of tyrosine kinase 7 (DOK7) pathway that is responsible for synaptic differentiation, including clustering of AChRs at the neuromuscular junction, both during development and in adult muscle. (ox.ac.uk)
- The role of these cilia in processes such as mucous clearance and cerebrospinal fluid movement is well documented. (asnjournals.org)
- and based on the diverse range of cell types that can form a cilium, the clinical features of several human disorders have been attributed to dysfunctional cilia. (biologists.org)
- Depending on the cell type, cilia acquire a defined set of functions and, accordingly, are built with an appropriate length and molecular composition. (biologists.org)
- The cilium is a microtubule-based organelle that is found on most cells in the mammalian body. (asnjournals.org)
- In contrast, BBS1/2/4 knockout mice are viable, and cilia are at least initially assembled in the majority of tissues, with sperm flagella being an exception. (rupress.org)