Simultaneous measurement of smoothened entry into and exit from the primary cilium. [PDF]
Ciliary accumulation of signaling proteins must result from a rate of ciliary entry that exceeds ciliary exit, but approaches for distinguishing ciliary entry vs. exit are lacking.
Jynho Kim +5 more
doaj +1 more source
Mutations in DYNC2LI1 disrupt cilia function and cause short rib polydactyly syndrome. [PDF]
The short rib polydactyly syndromes (SRPSs) are a heterogeneous group of autosomal recessive, perinatal lethal skeletal disorders characterized primarily by short, horizontal ribs, short limbs and polydactyly.
Daniel H. Cohn +12 more
core +2 more sources
Overexpression of Hedgehog family proteins contributes to the aetiology of many cancers. To be highly active, Hedgehog proteins must be palmitoylated at their N-terminus by the MBOAT family multispanning membrane enzyme Hedgehog acyltransferase (Hhat ...
Antonios D Konitsiotis +8 more
doaj +1 more source
The transcriptional activator Gli2 modulates T-cell receptor signalling through attenuation of AP-1 and NFκB activity [PDF]
Different tissues contain diverse and dynamic cellular niches, providing distinct signals to tissue-resident or migratory infiltrating immune cells.
Agathocleous +72 more
core +5 more sources
Mouse dispatched mutants fail to distribute hedgehog proteins and are defective in hedgehog signaling [PDF]
Hedgehog (Hh) signaling plays a major role in multiple aspects of embryonic development, which involves both short- and long-range signaling from localized Hh sources. One unusual aspect of Hh signaling is the autoproteolytic processing of Hh followed by lipid modification.
Takatoshi, Kawakami +5 more
openaire +2 more sources
Canonical Sonic Hedgehog Signaling in Early Lung Development
The canonical hedgehog (HH) signaling pathway is of major importance during embryonic development. HH is a key regulatory morphogen of numerous cellular processes, namely, cell growth and survival, differentiation, migration, and tissue polarity. Overall,
Hugo Fernandes-Silva +2 more
doaj +1 more source
The centrosomal deubiquitylase USP21 regulates Gli1 transcriptional activity and stability [PDF]
USP21 is a centrosome-associated deubiquitylase (DUB) that has been implicated in the formation of primary cilia - crucial organelles for the regulation of the Hedgehog (Hh) signaling pathway in vertebrates. Here, we identify KCTD6 - a cullin-3 E3-ligase
Bertsoulaki, Erithelgi +6 more
core +1 more source
Loss of Pin1 Suppresses Hedgehog-Driven Medulloblastoma Tumorigenesis
Medulloblastoma is the most common malignant brain tumor in children. Therapeutic approaches to medulloblastoma (combination of surgery, radiotherapy, and chemotherapy) have led to significant improvements, but these are achieved at a high cost to ...
Tao Xu +14 more
doaj +1 more source
A Hedgehog-Induced BTB Protein Modulates Hedgehog Signaling by Degrading Ci/Gli Transcription Factor [PDF]
The Ci/Gli family of transcription factors mediates Hedgehog (Hh) signaling in many key developmental processes. Here we identify a Hh-induced MATH and BTB domain containing protein (HIB) as a negative regulator of the Hh pathway. Overexpressing HIB down regulates Ci and blocks Hh signaling, whereas inactivating HIB results in Ci accumulation and ...
Zhang, Qing +5 more
openaire +2 more sources
A novel protein LZTFL1 regulates ciliary trafficking of the BBSome and Smoothened. [PDF]
Many signaling proteins including G protein-coupled receptors localize to primary cilia, regulating cellular processes including differentiation, proliferation, organogenesis, and tumorigenesis.
Seongjin Seo +6 more
doaj +1 more source

