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Introduction to Primary Cilia
Author(s) -
Satir Peter
Publication year - 2006
Publication title -
the faseb journal
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.709
H-Index - 277
eISSN - 1530-6860
pISSN - 0892-6638
DOI - 10.1096/fasebj.20.4.a436-e
Subject(s) - cilium , intraflagellar transport , axoneme , microbiology and biotechnology , motile cilium , dynein , biology , ciliogenesis , primary ciliary dyskinesia , polycystic kidney disease , bardet–biedl syndrome , retinitis pigmentosa , microtubule , centrosome , neuroscience , anatomy , medicine , cell , endocrinology , kidney , flagellum , lung , genetics , retina , bronchiectasis , cell cycle , phenotype , gene
Primary cilia are cellular antennae that are found on a variety of human cells, including kidney tubule and bile duct epithelia, chondrocytes and fibroblasts, neurons and glia. Primary cilia are built in the same manner as motile 9+2 cilia of all organisms, using specialized molecular motors for transport of protein building blocks. However, they are generally non‐motile, built around a 9+0 axoneme, missing the central microtubules and except for nodal cilia, missing dynein arms. The axoneme is surrounded by a ciliary membrane, containing specialized receptors and channels that provide a signaling mechanism for the cell. Primary cilia signaling is important for homeostatic maintenance of tissues, preventing uncontrolled cell division and dedifferentiation. Failure of signaling leads to diverse embryonic defects, including a reversal of left‐right symmetry of the body and chronic obstructive pulmonary disease, so‐called primary ciliary dyskinesia (PCD), defects in neurogenesis, if hedgehog signaling in neuronal primary cilia is affected, hydrocephalus, polycystic kidney disease (PKD), polycystic liver disease and a form of retinitis pigmentosa. The growing importance of normal morphogenesis and function of primary cilia in receptor and channel localization for prevention of human pathologies is becoming apparent.