Published in Mol Ther on February 22, 2017
Intraflagellar transport. Nat Rev Mol Cell Biol (2002) 10.86
A core complex of BBS proteins cooperates with the GTPase Rab8 to promote ciliary membrane biogenesis. Cell (2007) 10.75
The ciliopathies: an emerging class of human genetic disorders. Annu Rev Genomics Hum Genet (2006) 9.56
Recombinant adeno-associated virus purification using novel methods improves infectious titer and yield. Gene Ther (1999) 7.96
Site-specific integration by adeno-associated virus. Proc Natl Acad Sci U S A (1990) 7.06
Targeted integration of adeno-associated virus (AAV) into human chromosome 19. EMBO J (1991) 6.13
Analysis of AAV serotypes 1-9 mediated gene expression and tropism in mice after systemic injection. Mol Ther (2008) 5.83
Integration of the adeno-associated virus genome into cellular DNA in latently infected human Detroit 6 cells. J Virol (1980) 5.09
Disruption of Bardet-Biedl syndrome ciliary proteins perturbs planar cell polarity in vertebrates. Nat Genet (2005) 4.76
The conserved Bardet-Biedl syndrome proteins assemble a coat that traffics membrane proteins to cilia. Cell (2010) 3.83
Disruption of intraflagellar transport in adult mice leads to obesity and slow-onset cystic kidney disease. Curr Biol (2007) 3.76
Loss of C. elegans BBS-7 and BBS-8 protein function results in cilia defects and compromised intraflagellar transport. Genes Dev (2004) 3.21
Bardet-Biedl syndrome proteins are required for the localization of G protein-coupled receptors to primary cilia. Proc Natl Acad Sci U S A (2008) 3.21
Contribution of olfactory neural stem cells to tissue maintenance and regeneration. Nat Neurosci (2007) 3.12
Bbs2-null mice have neurosensory deficits, a defect in social dominance, and retinopathy associated with mislocalization of rhodopsin. Proc Natl Acad Sci U S A (2004) 3.11
Loss of BBS proteins causes anosmia in humans and defects in olfactory cilia structure and function in the mouse. Nat Genet (2004) 2.84
Mutations in a member of the Ras superfamily of small GTP-binding proteins causes Bardet-Biedl syndrome. Nat Genet (2004) 2.64
The Chlamydomonas reinhardtii BBSome is an IFT cargo required for export of specific signaling proteins from flagella. J Cell Biol (2009) 2.55
Functional analyses of variants reveal a significant role for dominant negative and common alleles in oligogenic Bardet-Biedl syndrome. Proc Natl Acad Sci U S A (2010) 2.45
A knockin mouse model of the Bardet-Biedl syndrome 1 M390R mutation has cilia defects, ventriculomegaly, retinopathy, and obesity. Proc Natl Acad Sci U S A (2007) 2.36
Mechanism of transport of IFT particles in C. elegans cilia by the concerted action of kinesin-II and OSM-3 motors. J Cell Biol (2006) 2.32
Bardet-Biedl syndrome. Eur J Hum Genet (2012) 2.15
The intraflagellar transport protein IFT27 promotes BBSome exit from cilia through the GTPase ARL6/BBS3. Dev Cell (2014) 2.09
Odorant receptor gene choice is reset by nuclear transfer from mouse olfactory sensory neurons. Nature (2004) 2.09
Mkks-null mice have a phenotype resembling Bardet-Biedl syndrome. Hum Mol Genet (2005) 2.04
Neural regeneration and the peripheral olfactory system. Anat Rec (2002) 2.04
Sensory ciliogenesis in Caenorhabditis elegans: assignment of IFT components into distinct modules based on transport and phenotypic profiles. Mol Biol Cell (2007) 1.82
Adeno-associated virus serotype 9 vectors transduce murine alveolar and nasal epithelia and can be readministered. Proc Natl Acad Sci U S A (2006) 1.78
GFAP promoter elements required for region-specific and astrocyte-specific expression. Glia (2008) 1.65
Peripheral olfactory projections are differentially affected in mice deficient in a cyclic nucleotide-gated channel subunit. Neuron (2000) 1.62
Reconstitution of the rat olfactory epithelium after methyl bromide-induced lesion. J Comp Neurol (1995) 1.54
Immunity to adenovirus and adeno-associated viral vectors: implications for gene therapy. Gene Ther (2003) 1.54
Transneuronal regulation of tyrosine hydroxylase expression in olfactory bulb of mouse and rat. J Neurosci (1983) 1.54
The BBSome controls IFT assembly and turnaround in cilia. Nat Cell Biol (2012) 1.50
A novel protein LZTFL1 regulates ciliary trafficking of the BBSome and Smoothened. PLoS Genet (2011) 1.49
Dopamine receptor 1 localizes to neuronal cilia in a dynamic process that requires the Bardet-Biedl syndrome proteins. Cell Mol Life Sci (2010) 1.45
Intrinsic protein-protein interaction-mediated and chaperonin-assisted sequential assembly of stable bardet-biedl syndrome protein complex, the BBSome. J Biol Chem (2012) 1.42
Absence of adenylyl cyclase 3 perturbs peripheral olfactory projections in mice. J Neurosci (2007) 1.39
Retinal disease expression in Bardet-Biedl syndrome-1 (BBS1) is a spectrum from maculopathy to retina-wide degeneration. Invest Ophthalmol Vis Sci (2006) 1.39
Adult naris closure profoundly reduces tyrosine hydroxylase expression in mouse olfactory bulb. Brain Res (1993) 1.34
BBS proteins interact genetically with the IFT pathway to influence SHH-related phenotypes. Hum Mol Genet (2012) 1.33
The advent of AAV9 expands applications for brain and spinal cord gene delivery. Expert Opin Biol Ther (2012) 1.30
Qualitative and quantitative freeze-fracture studies on olfactory and nasal respiratory structures of frog, ox, rat, and dog. I. A general survey. Cell Tissue Res (1980) 1.30
Retinal dystrophy in Bardet-Biedl syndrome and related syndromic ciliopathies. Prog Retin Eye Res (2011) 1.28
Olfactory receptor proteins in axonal processes of chemosensory neurons. J Neurosci (2004) 1.26
An autoradiographic study of the mouse olfactory epithelium: evidence for long-lived receptors. Anat Rec (1984) 1.23
Gene therapy prevents photoreceptor death and preserves retinal function in a Bardet-Biedl syndrome mouse model. Proc Natl Acad Sci U S A (2011) 1.22
Neuropeptide Y family receptors traffic via the Bardet-Biedl syndrome pathway to signal in neuronal primary cilia. Cell Rep (2013) 1.19
Ultrastructural aspects of olfactory signaling. Chem Senses (1997) 1.15
Cell dynamics in the adult mouse olfactory epithelium: a quantitative autoradiographic study. J Neurosci (1991) 1.10
Effects of absorption enhancers on the transport of model compounds in Caco-2 cell monolayers: assessment by confocal laser scanning microscopy. J Pharm Sci (1997) 1.08
Abnormal development of NG2+PDGFR-α+ neural progenitor cells leads to neonatal hydrocephalus in a ciliopathy mouse model. Nat Med (2012) 1.08
Single-dose lentiviral gene transfer for lifetime airway gene expression. J Gene Med (2009) 1.07
Gene therapy rescues cilia defects and restores olfactory function in a mammalian ciliopathy model. Nat Med (2012) 1.05
Loss of Bardet-Biedl syndrome protein-8 (BBS8) perturbs olfactory function, protein localization, and axon targeting. Proc Natl Acad Sci U S A (2011) 1.03
Subretinal gene therapy of mice with Bardet-Biedl syndrome type 1. Invest Ophthalmol Vis Sci (2013) 1.01
Update on the genetics of bardet-biedl syndrome. Mol Syndromol (2013) 1.00
Qualitative and quantitative freeze-fracture studies on olfactory and nasal respiratory epithelial surfaces of frog, ox, rat, and dog. II. Cell apices, cilia, and microvilli. Cell Tissue Res (1980) 1.00
Retinal morphology in patients with BBS1 and BBS10 related Bardet-Biedl Syndrome evaluated by Fourier-domain optical coherence tomography. Vision Res (2007) 0.99
Direct evidence for BBSome-associated intraflagellar transport reveals distinct properties of native mammalian cilia. Nat Commun (2014) 0.98
Metabolism-dependent toxicity of methimazole in the olfactory nasal mucosa. Pharmacol Toxicol (1995) 0.97
Biodistribution of adeno-associated virus serotype 9 (AAV9) vector after intrathecal and intravenous delivery in mouse. Front Neuroanat (2014) 0.96
Methimazole toxicity in rodents: covalent binding in the olfactory mucosa and detection of glial fibrillary acidic protein in the olfactory bulb. Toxicol Appl Pharmacol (1999) 0.96
Continuous neural plasticity in the olfactory intrabulbar circuitry. J Neurosci (2010) 0.96
Transduction physiology of olfactory receptor cilia. Brain Res (1984) 0.96
Studies of olfactory system neural plasticity: the contribution of the unilateral naris occlusion technique. Neural Plast (2012) 0.93
Lysophosphatidylcholine as an adjuvant for lentiviral vector mediated gene transfer to airway epithelium: effect of acyl chain length. Respir Res (2010) 0.90
Expression of the tight junction protein ZO-1 in the olfactory system: presence of ZO-1 on olfactory sensory neurons and glial cells. J Comp Neurol (1994) 0.89
Promoter-specific lentivectors for long-term, cardiac-directed therapy of Fabry disease. J Cardiol (2010) 0.87
Architectures of multisubunit complexes revealed by a visible immunoprecipitation assay using fluorescent fusion proteins. J Cell Sci (2015) 0.85
BBS4 and BBS5 show functional redundancy in the BBSome to regulate the degradative sorting of ciliary sensory receptors. Sci Rep (2015) 0.84
Ectopic expression of human BBS4 can rescue Bardet-Biedl syndrome phenotypes in Bbs4 null mice. PLoS One (2013) 0.84
Activity-dependent regulation of dopamine content in the olfactory bulbs of naris-occluded rats. Neuroscience (1998) 0.84
Impact of lentiviral vector-mediated transduction on the tightness of a polarized model of airway epithelium and effect of cationic polymer polyethylenimine. J Biomed Biotechnol (2010) 0.83
Enhancement of adenovirus-mediated gene transfer to the airways by DEAE dextran and sodium caprate in vivo. Mol Ther (2003) 0.82
Accumulation of non-outer segment proteins in the outer segment underlies photoreceptor degeneration in Bardet-Biedl syndrome. Proc Natl Acad Sci U S A (2015) 0.82
Smelling the roses and seeing the light: gene therapy for ciliopathies. Trends Biotechnol (2013) 0.82
Olfaction evaluation and correlation with brain atrophy in Bardet-Biedl syndrome. Clin Genet (2014) 0.78
The glucose transporter GLUT1 and the tight junction protein occludin in nasal olfactory mucosa. Chem Senses (2002) 0.78
Comparative genomics and gene expression analysis identifies BBS9, a new Bardet-Biedl syndrome gene. Am J Hum Genet (2005) 2.25
Discovery and functional analysis of a retinitis pigmentosa gene, C2ORF71. Am J Hum Genet (2010) 1.03