title: Gorab Is Required for Dermal Condensate Cells to Respond to Hedgehog Signals during Hair Follicle Morphogenesis.
abstract: GORAB is a golgin that localizes predominantly at the Golgi apparatus and physically interacts with small guanosine triphosphatases. GORAB is ubiquitously expressed in mammalian tissues, including the skin. However, the biological function of this golgin in skin is unknown. Here, we report that disrupting the expression of the Gorab gene in mice results in hair follicle morphogenesis defects that were characterized by impaired follicular keratinocyte differentiation. This hair follicle phenotype was associated with markedly suppressed hedgehog (Hh) signaling pathway in dermal condensates in vivo. Gorab-deficient dermal mesenchymal cells also displayed a significantly reduced capability to respond to Hh pathway activation in vitro. Furthermore, we found that the formation of the primary cilium, a cellular organelle that is essential for the Hh pathway, was impaired in mutant dermal condensate cells, suggesting that Gorab may be required for the Hh pathway through facilitating the formation of primary cilia. Thus, data obtained from this study provided insight into the biological functions of Gorab during embryonic morphogenesis of the skin in which Hh signaling and primary cilia exert important functions.
authors.affiliation: Institute of Laboratory Animal Science, Chinese Academy of Medical Science, Comparative Medical Center, Peking Union Medical College, and Key Laboratory of Human Disease Comparative Medicine, Ministry of Health, Beijing, China.
authors.forename: Ying
authors.initials: Y
authors.lastname: Liu
authors.affiliation: Department of Pathology, Stony Brook University, Stony Brook, New York, USA; Graduate Program in Genetics, Stony Brook University, Stony Brook, New York, USA.
authors.forename: Elizabeth R
authors.initials: ER
authors.lastname: Snedecor
authors.affiliation: Department of Pathology, Stony Brook University, Stony Brook, New York, USA.
authors.forename: Yeon Ja
authors.initials: YJ
authors.lastname: Choi
authors.affiliation: Department of Pathology, Stony Brook University, Stony Brook, New York, USA.
authors.forename: Ning
authors.initials: N
authors.lastname: Yang
authors.affiliation: Institute of Laboratory Animal Science, Chinese Academy of Medical Science, Comparative Medical Center, Peking Union Medical College, and Key Laboratory of Human Disease Comparative Medicine, Ministry of Health, Beijing, China.
authors.forename: Xu
authors.initials: X
authors.lastname: Zhang
authors.affiliation: Institute of Laboratory Animal Science, Chinese Academy of Medical Science, Comparative Medical Center, Peking Union Medical College, and Key Laboratory of Human Disease Comparative Medicine, Ministry of Health, Beijing, China.
authors.forename: Yuhuan
authors.initials: Y
authors.lastname: Xu
authors.affiliation: Institute of Laboratory Animal Science, Chinese Academy of Medical Science, Comparative Medical Center, Peking Union Medical College, and Key Laboratory of Human Disease Comparative Medicine, Ministry of Health, Beijing, China.
authors.forename: Yunlin
authors.initials: Y
authors.lastname: Han
authors.affiliation: Department of Dermatology, Stony Brook University, Stony Brook, New York, USA.
authors.forename: Evan C
authors.initials: EC
authors.lastname: Jones
authors.affiliation: Department of Pathology, Stony Brook University, Stony Brook, New York, USA.
authors.forename: Kenneth R
authors.initials: KR
authors.lastname: Shroyer
authors.affiliation: Department of Dermatology, Stony Brook University, Stony Brook, New York, USA.
authors.forename: Richard A
authors.initials: RA
authors.lastname: Clark
authors.affiliation: Institute of Laboratory Animal Science, Chinese Academy of Medical Science, Comparative Medical Center, Peking Union Medical College, and Key Laboratory of Human Disease Comparative Medicine, Ministry of Health, Beijing, China.
authors.forename: Lianfeng
authors.initials: L
authors.lastname: Zhang
authors.affiliation: Institute of Laboratory Animal Science, Chinese Academy of Medical Science, Comparative Medical Center, Peking Union Medical College, and Key Laboratory of Human Disease Comparative Medicine, Ministry of Health, Beijing, China. Electronic address: qinchuan@pumc.edu.cn.
authors.forename: Chuan
authors.initials: C
authors.lastname: Qin
authors.affiliation: Institute of Laboratory Animal Science, Chinese Academy of Medical Science, Comparative Medical Center, Peking Union Medical College, and Key Laboratory of Human Disease Comparative Medicine, Ministry of Health, Beijing, China; Department of Pathology, Stony Brook University, Stony Brook, New York, USA; Department of Dermatology, Stony Brook University, Stony Brook, New York, USA. Electronic address: jiang.chen@stonybrook.edu.
authors.forename: Jiang
authors.initials: J
authors.lastname: Chen
mesh_terms: D000818:Animals; D002478:Cells, Cultured; D005347:Fibroblasts / Q000166:cytology* / Q000502:physiology; D005455:Fluorescent Antibody Technique; D018859:Hair Follicle / Q000196:embryology*; D053823:Hedgehog Proteins / Q000378:metabolism*; D015603:Keratinocytes / Q000166:cytology* / Q000502:physiology; D051379:Mice; D018345:Mice, Knockout; D008819:Mice, Nude; D008822:Mice, Transgenic; D023421:Models, Animal; D009024:Morphogenesis / Q000502:physiology*; D012313:RNA / Q000378:metabolism; D060888:Real-Time Polymerase Chain Reaction; D017468:Receptors, Fibroblast Growth Factor / Q000235:genetics / Q000378:metabolism*; D012680:Sensitivity and Specificity; D012795:Sialoglycoproteins / Q000235:genetics / Q000378:metabolism*; D015398:Signal Transduction
chemical_list: D053823:Hedgehog Proteins; D017468:Receptors, Fibroblast Growth Factor; D012795:Sialoglycoproteins; C086711:cysteine-rich fibroblast growth factor receptor; D012313:RNA