
We study the primary cilium, a surface-exposed organelle required for vision, olfaction and developmental signaling. Our lab is interested in the mechanisms of trafficking in and out of cilia and how trafficking decisions impact signaling at the cilium. One research axis focuses on Bardet-Biedl Syndrome (BBS), a ciliopathy characterized by obesity, skeletal malformations and kidney cysts. The BBSome is an evolutionarily conserved complex of 8 BBS proteins that traffics signaling receptors out of cilia and we aim to decipher the molecular details of BBSome-mediated trafficking and the cellular, biochemical and structural level. A second research direction is aimed at cilia-derived extracellular vesicles (EVs). Ciliary signaling receptors that fail conventional exit are packaged into ciliary EVs. By dissecting the mechanisms of ciliary EV biogenesis, we will gain insight into the roles of these extracellular parcels in cell-cell communication.
Publications
BBSome-Mediated Clearance of Ubiquitinated IMPG2 Defines a Constitutive Ciliary Retrieval Pathway in Photoreceptors.
Intraflagellar transport trains can switch rails and move along multiple microtubules in intact primary cilia.
Long-term effectiveness and acceptability of switching from intravenous to subcutaneous infliximab in patients with inflammatory bowel disease treated with intensified doses: The REMSWITCH-LT study.
The ancestral ESCRT protein TOM1L2 selects ubiquitinated cargoes for retrieval from cilia.
Shedding of ciliary vesicles at a glance.
Time-resolved proteomics profiling of the ciliary Hedgehog response.
Ubiquitin chains earmark GPCRs for BBSome-mediated removal from cilia.
The Molecular Architecture of Native BBSome Obtained by an Integrated Structural Approach.
Establishing and regulating the composition of cilia for signal transduction.
Effects of α-tubulin acetylation on microtubule structure and stability.
Adalimumab for patients with Crohn's disease complicated by intra-abdominal abscess: a multicentre, prospective, observational cohort study.
The molecular machines that traffic signaling receptors into and out of cilia.
BBSome trains remove activated GPCRs from cilia by enabling passage through the transition zone.
A CRISPR-based screen for Hedgehog signaling provides insights into ciliary function and ciliopathies.
BORC Regulates the Axonal Transport of Synaptic Vesicle Precursors by Activating ARL-8.
Dramatic Increase in Incidence of Ulcerative Colitis and Crohn's Disease (1988-2011): A Population-Based Study of French Adolescents.
Microtubules acquire resistance from mechanical breakage through intralumenal acetylation.
Membrane traffic control by cytoskeletal and molecular machines.
Fifteen years of research on oral-facial-digital syndromes: from 1 to 16 causal genes.
Measurement of tubulin oligomers self-assembly by FRET.
Tubulin acetylation protects long-lived microtubules against mechanical ageing.
Endoscopic Detection of Small Bowel Dysplasia and Adenocarcinoma in Crohn's Disease: A Prospective Cohort-Study in High-Risk Patients.
Ileal or Anastomotic Location of Lesions Does Not Impact Rate of Postoperative Recurrence in Crohn's Disease Patients Classified i2 on the Rutgeerts Score.
Abstract B068: Structural basis for Notch1 engagement of Delta-like 4 and Jagged1.
Localization of GFP-Tagged Proteins at the Electron Microscope.
Loss of the BBSome perturbs endocytic trafficking and disrupts virulence of Trypanosoma brucei.
Proteomics of Primary Cilia by Proximity Labeling.
Cytoplasmic Dynein Antagonists with Improved Potency and Isoform Selectivity.
Microtubules self-repair in response to mechanical stress.
Structural biology. Structural basis for Notch1 engagement of Delta-like 4.
Analysis of soluble protein entry into primary cilia using semipermeabilized cells.
Structural basis for membrane targeting of the BBSome by ARL6.
The intraflagellar transport protein IFT27 promotes BBSome exit from cilia through the GTPase ARL6/BBS3.
How do cilia organize signalling cascades?
The oral-facial-digital syndrome gene C2CD3 encodes a positive regulator of centriole elongation.
Α-tubulin K40 acetylation is required for contact inhibition of proliferation and cell-substrate adhesion.
Movement of Signaling Receptors Inside Primary Cilia.
Effects of tubulin acetylation and tubulin acetyltransferase binding on microtubule structure.
Cilia grow by taking a bite out of the cell.
An in vitro assay for entry into cilia reveals unique properties of the soluble diffusion barrier.
Exome sequencing of Bardet-Biedl syndrome patient identifies a null mutation in the BBSome subunit BBIP1 (BBS18).
Structure of the α-tubulin acetyltransferase, αTAT1, and implications for tubulin-specific acetylation.
Give chance a chance.
Primary cilia: how to keep the riff-raff in the plasma membrane.
Primary cilia membrane assembly is initiated by Rab11 and transport protein particle II (TRAPPII) complex-dependent trafficking of Rabin8 to the centrosome.
The major alpha-tubulin K40 acetyltransferase alphaTAT1 promotes rapid ciliogenesis and efficient mechanosensation.
Maxence Nachury: a transporting view of the primary cilium. Interview by Ben Short.
A septin diffusion barrier at the base of the primary cilium maintains ciliary membrane protein distribution.
The perennial organelle: assembly and disassembly of the primary cilium.
Trafficking to the ciliary membrane: how to get across the periciliary diffusion barrier?
Constructing and deconstructing roles for the primary cilium in tissue architecture and cancer.
The BBSome.
A BBSome subunit links ciliogenesis, microtubule stability, and acetylation.
How to build a signalling organelle: A molecular pathway for biogenesis of the ciliary membrane.
The nucleolar phosphatase Cdc14B is dispensable for chromosome segregation and mitotic exit in human cells.
Tandem affinity purification of the BBSome, a critical regulator of Rab8 in ciliogenesis.
[Cronkhite-Canada syndrome].
The END network couples spindle pole assembly to inhibition of the anaphase-promoting complex/cyclosome in early mitosis.
Emi1 stably binds and inhibits the anaphase-promoting complex/cyclosome as a pseudosubstrate inhibitor.
Xenopus Cdc14 alpha/beta are localized to the nucleolus and centrosome and are required for embryonic cell division.
The direction of transport through the nuclear pore can be inverted.
Cloning and characterization of hSRP1 gamma, a tissue-specific nuclear transport factor.
555 Mission Bay Blvd South, Rm 352X
UCSF Box 3120
San Francisco, CA 94158
United States