ZFIN ID: ZDB-PUB-120323-3
Nephrocystins and MKS proteins interact with IFT particle and facilitate transport of selected ciliary cargos
Zhao, C., and Malicki, J.
Date: 2011
Source: The EMBO journal   30(13): 2532-2544 (Journal)
Registered Authors: Malicki, Jarema, Zhao, Chengtian
Keywords: hair cell, photoreceptor, planar cell polarity, sensory neuron, zebrafish
MeSH Terms:
  • Adaptor Proteins, Signal Transducing/metabolism*
  • Adaptor Proteins, Signal Transducing/physiology*
  • Animals
  • Animals, Genetically Modified
  • Biological Transport, Active/physiology
  • Cilia/genetics
  • Cilia/metabolism*
  • Cilia/physiology
  • Humans
  • Membrane Proteins/metabolism*
  • Membrane Proteins/physiology*
  • Multiprotein Complexes/metabolism
  • Multiprotein Complexes/physiology
  • Organogenesis/genetics
  • Organogenesis/physiology
  • Protein Binding
  • Protein Transport
  • Proteins/metabolism
  • Transcription Factors/metabolism
  • Two-Hybrid System Techniques
  • Zebrafish/embryology
  • Zebrafish/metabolism
  • Zebrafish Proteins/genetics
  • Zebrafish Proteins/metabolism
  • Zebrafish Proteins/physiology
PubMed: 21602787 Full text @ EMBO J.

Cilia are required for the development and function of many organs. Efficient transport of protein cargo along ciliary axoneme is necessary to sustain these processes. Despite its importance, the mode of interaction between the intraflagellar ciliary transport (IFT) mechanism and its cargo proteins remains poorly understood. Our studies demonstrate that IFT particle components, and a Meckel–Gruber syndrome 1 (MKS1)-related, B9 domain protein, B9d2, bind each other and contribute to the ciliary localization of Inversin (Nephrocystin 2). B9d2, Inversin, and Nephrocystin 5 support, in turn, the transport of a cargo protein, Opsin, but not another photoreceptor ciliary transmembrane protein, Peripherin. Interestingly, the components of this mechanism also contribute to the formation of planar cell polarity in mechanosensory epithelia. These studies reveal a molecular mechanism that mediates the transport of selected ciliary cargos and is of fundamental importance for the differentiation and survival of sensory cells.