You are viewing an incomplete version of our website. Please click to reload the website as full version.

Signalisation Hedgehog

Le signal Hedgehog (Hh) se conserve parfaitement à travers les cordés de différentes classes taxonomiques. Il est essentiel dans le développement de l’embryon. La voie Hedehog a été décrite et caractérisée pour la première fois chez la mouche du vinaigre, la Drosophila melanogaster. Chez les mammifères, le signal Hh régule le sort des cellules, la polarité des tissus, et la structure cellulaire durant l’embryogénèse précoce et la morphogénèse de certains organes et de certains tissus. Par conséquent, il est mis en latence dans la plupart des tissus adultes, mais il peut être réactivé suite à des blessures afin de stimuler la réparation et la régénération.

Différents ligands Hh, dont les homologues sonic hedgehog (SHH), indian hedgehog (IHH), et desert hedgehog (DHH) sont produits en tant que précurseurs qui soutiennent le clivage autocatalytique, l’attachement du cholestérol C-terminal, et la palmitylation N-terminale avant la sécrétion. La libération et l’accumulation extracellulaire des ligands matures sont régulées par les homologues de la protéine dispatchée (Dips) de la Drosophile. La liaison entre les ligands Hh sécrétés et l’homologue récepteur (PTCH1, PTCH2) déclenche la stimulation du réseau de signaux : l’effet inhibiteur de PTCH sur le récepteur transmembranaire SMO est supprimé, ce qui conduit à l’activation des facteurs de transcription de l’oncogène lié au gliome (GLI).

Une structure-clé spécialisée de ce processus est le cil primaire en microtubule. En l’absence du Hh non traité, les protéines GLI non activantes, ainsi que leur régulateur SUFU, sont concentrées à la pointe distale du cil primaire. À partir de la liaison du ligand Hh, PTCH se relocalise au niveau de la surface cellulaire, rendant ainsi possible la translocation du SMO vers le cil primaire et la régulation à la baisse du SUFU. Plusieurs protéines du cil primaire, essentielles du point de vue de la structure, ont également des effets régulateurs sur la cascade de signaux Hh.

Une régulation aberrante de Hh/GLI entraîne des problèmes graves pour les tissus et l’apparition de nombreux cancers agressifs. La cascade Hh/GLI a également été mise en relation avec la régulation des gènes déterminant le caractère souche des cellules et avec la survie des cellules-souches des cancers.

Téléchargement au format PDF

Hedgehog

SHH - Sonic Hedgehog

This gene encodes a protein that is instrumental in patterning the early embryo. It has been implicated as the key inductive signal in patterning of the ventral neural tube, the anterior-posterior limb axis, and the ventral somites. Of three human proteins showing sequence and functional similarity to the sonic hedgehog protein of Drosophila, this protein is the most similar. The protein is...   More...

IHH - Indian Hedgehog

This gene encodes a member of the hedgehog family of secreted signaling molecules. Hedgehog proteins are essential regulators of a variety of...

DHH - desert Hedgehog

This gene encodes a member of the hedgehog family. The hedgehog gene family encodes signaling molecules that play an important role in regulating...

Hh Processing

HHAT - Hedgehog Acyltransferase

Skinny hedgehog' (SKI1) encodes an enzyme that acts within the secretory pathway to catalyze amino-terminal palmitoylation of 'hedgehog' (see MIM 600725).[supplied by OMIM, Jul 2002].   More...
Antigène dans cette catégorie:
HHATL - Hedgehog Acyltransferase-Like: HHATL anticorps   HHATL Proteins
PRKACA (Protein Kinase A, alpha): PRKACA anticorps PRKACA ELISA Kits PRKACA Proteins
PRKACB (Protein Kinase, CAMP Dependent, Catalytic, beta): PRKACB anticorps PRKACB ELISA Kits PRKACB Proteins
PRKACG (Protein Kinase, CAMP-Dependent, Catalytic, gamma): PRKACG anticorps   PRKACG Proteins
PROKR1 - Prokineticin Receptor 1: PROKR1 anticorps PROKR1 ELISA Kits PROKR1 Proteins
PRKAR1A - Protein Kinase, CAMP-Dependent, Regulatory, Type I, alpha (Tissue Specific Extinguisher 1): PRKAR1A anticorps PRKAR1A ELISA Kits PRKAR1A Proteins
PRKAR1B (Protein Kinase, CAMP-Dependent, Regulatory, Type I, beta): PRKAR1B anticorps PRKAR1B ELISA Kits PRKAR1B Proteins
PROKR2 - Prokineticin Receptor 2: PROKR2 anticorps PROKR2 ELISA Kits PROKR2 Proteins
PRKAR2A - Protein Kinase, CAMP-Dependent, Regulatory, Type II, alpha: PRKAR2A anticorps PRKAR2A ELISA Kits PRKAR2A Proteins
PRKAR2B - Protein Kinase, CAMP-Dependent, Regulatory, Type II, beta: PRKAR2B anticorps PRKAR2B ELISA Kits PRKAR2B Proteins
CSNK1A1 (Casein Kinase 1, alpha 1): CSNK1A1 anticorps CSNK1A1 ELISA Kits CSNK1A1 Proteins
CSNK1D - Casein Kinase 1 delta: CSNK1D anticorps CSNK1D ELISA Kits CSNK1D Proteins
CSNK1E - CK1 epsilon: CSNK1E anticorps CSNK1E ELISA Kits CSNK1E Proteins
CSNK1G1 - Casein Kinase 1, gamma 1: CSNK1G1 anticorps CSNK1G1 ELISA Kits CSNK1G1 Proteins
CSNK1G2 - Casein Kinase 1 gamma 2: CSNK1G2 anticorps CSNK1G2 ELISA Kits CSNK1G2 Proteins
CSNK1G3 - Casein Kinase 1, gamma 3: CSNK1G3 anticorps CSNK1G3 ELISA Kits CSNK1G3 Proteins
GSK3b - GSK3 beta: GSK3b anticorps GSK3b ELISA Kits  
SKP1 (S-Phase Kinase-Associated Protein 1): SKP1 anticorps SKP1 ELISA Kits SKP1 Proteins
CUL1 - Cullin 1: CUL1 anticorps CUL1 ELISA Kits CUL1 Proteins
BTRC (beta-Transducin Repeat Containing): BTRC anticorps BTRC ELISA Kits BTRC Proteins

Hh Receptors

PTCH1 - Patched 1

This gene encodes a member of the patched gene family. The encoded protein is the receptor for sonic hedgehog, a secreted molecule implicated in the formation of embryonic structures and in tumorigenesis, as well as the desert hedgehog and indian hedgehog proteins. This gene functions as a tumor suppressor. Mutations of this gene have been associated with basal cell nevus syndrome, esophageal...   More...
Antigène dans cette catégorie:
PTCH2 - Patched 2: PTCH2 anticorps   PTCH2 Proteins
(SMO):      

Hh Effectors

GLI1 (Zinc Finger Protein GLI1):

This gene encodes a member of the Kruppel family of zinc finger proteins. The encoded transcription factor is activated by the sonic hedgehog signal transduction cascade and regulates stem cell proliferation. The activity and nuclear localization of this protein is negatively regulated by p53 in an inhibitory loop. Multiple transcript variants encoding different isoforms have been found for...   More...

GLI2 (GLI Family Zinc Finger 2):

This gene encodes a protein which belongs to the C2H2-type zinc finger protein subclass of the Gli family. Members of this subclass are...

GLI3 (GLI Family Zinc Finger 3):

This gene encodes a protein which belongs to the C2H2-type zinc finger proteins subclass of the Gli family. They are characterized as DNA-binding...

Primary Cilium

DZIP1 (DAZ Interacting Protein 1):

Acts as a permissive factor that is required for the proper regulation of Hedgehog (Hh) target genes in response to Hh signals. Acts downstream of the Smoothened protein to modulate Gli activity in the somites of the developing embryo.   More...

EVC (Ellis Van Creveld Syndrome):

This gene encodes a protein containing a leucine zipper and a transmembrane domain. This gene has been implicated in both Ellis-van Creveld...

KIF7 (Kinesin Family Member 7):

This gene encodes a cilia-associated protein belonging to the kinesin family. This protein plays a role in the sonic hedgehog (SHH) signaling...
Antigène dans cette catégorie:
EVC2 - Ellis Van Creveld Syndrome 2: EVC2 anticorps EVC2 ELISA Kits EVC2 Proteins
DYNC2H1 (Dynein, Cytoplasmic 2, Heavy Chain 1):   DYNC2H1 ELISA Kits  
DYNC2LI1 (Dynein, Cytoplasmic 2, Light Intermediate Chain 1): DYNC2LI1 anticorps DYNC2LI1 ELISA Kits DYNC2LI1 Proteins
KIF3A (Kinesin Family Member 3A): KIF3A anticorps KIF3A ELISA Kits KIF3A Proteins
KIF3B (Kinesin Family Member 3B): KIF3B anticorps KIF3B ELISA Kits KIF3B Proteins
KIF3C - Kinesin Family Member 3C: KIF3C anticorps KIF3C ELISA Kits KIF3C Proteins
KIFAP3 - Kinesin Associated Protein 3: KIFAP3 anticorps KIFAP3 ELISA Kits KIFAP3 Proteins
TRIM28 - Tripartite Motif Containing 28: TRIM28 anticorps TRIM28 ELISA Kits TRIM28 Proteins
IFT43 (Intraflagellar Transport 43 Homolog (Chlamydomonas)): IFT43 anticorps IFT43 ELISA Kits IFT43 Proteins
WDR35 (WD Repeat Domain 35): WDR35 anticorps WDR35 ELISA Kits  
TTC21B (Tetratricopeptide Repeat Domain 21B): TTC21B anticorps TTC21B ELISA Kits  
IFT140 - Intraflagellar Transport 140 Homolog (Chlamydomonas): IFT140 anticorps    
WDR19 - WD Repeat Domain 19: WDR19 anticorps WDR19 ELISA Kits WDR19 Proteins
IFT20 (Intraflagellar Transport 20 Homolog (Chlamydomonas)): IFT20 anticorps IFT20 ELISA Kits IFT20 Proteins
PDK4 (Pyruvate Dehydrogenase Kinase, Isozyme 4): PDK4 anticorps PDK4 ELISA Kits PDK4 Proteins
IFT27 (Intraflagellar Transport 27 Homolog (Chlamydomonas)): IFT27 anticorps IFT27 ELISA Kits IFT27 Proteins
IFT46 (Intraflagellar Transport 46 Homolog (Chlamydomonas)): IFT46 anticorps IFT46 ELISA Kits IFT46 Proteins
TRAF3IP1 (TNF Receptor-Associated Factor 3 Interacting Protein 1): TRAF3IP1 anticorps TRAF3IP1 ELISA Kits TRAF3IP1 Proteins
IFT57 (Intraflagellar Transport 57 Homolog (Chlamydomonas)): IFT57 anticorps IFT57 ELISA Kits IFT57 Proteins
TTC30B (Tetratricopeptide Repeat Domain 30B): TTC30B anticorps TTC30B ELISA Kits TTC30B Proteins
IFT74 (Intraflagellar Transport Protein 74 Homolog): IFT74 anticorps    
IFT80 (Intraflagellar Transport 80 Homolog (Chlamydomonas)): IFT80 anticorps IFT80 ELISA Kits IFT80 Proteins
IFT81 - Intraflagellar Transport 81 Homolog (Chlamydomonas): IFT81 anticorps IFT81 ELISA Kits IFT81 Proteins
IFT88 (Intraflagellar Transport 88 Homolog (Chlamydomonas)): IFT88 anticorps IFT88 ELISA Kits IFT88 Proteins
BBS1 (Bardet-Biedl Syndrome 1): BBS1 anticorps   BBS1 Proteins
BBS2 (Bardet-Biedl Syndrome 2): BBS2 anticorps   BBS2 Proteins
BBS4 (Bardet-Biedl Syndrome 4): BBS4 anticorps   BBS4 Proteins
BBS5 - Bardet-Biedl Syndrome 5: BBS5 anticorps   BBS5 Proteins
BBS7 (Bardet-Biedl Syndrome 7): BBS7 anticorps   BBS7 Proteins
TTC8 (Tetratricopeptide Repeat Domain 8): TTC8 anticorps TTC8 ELISA Kits TTC8 Proteins
BBS9 (Bardet-Biedl Syndrome 9): BBS9 anticorps   BBS9 Proteins

Hh Regulators

DISP1 (Dispatched Homolog 1 (Drosophila)):

The pattern of cellular proliferation and differentiation that leads to normal development of embryonic structures often depends upon the localized production of secreted protein signals. Cells surrounding the source of a particular signal respond in a graded manner according to the effective concentration of the signal, and this response produces the pattern of cell types constituting the...   More...

DISP2 (Dispatched Homolog 2 (Drosophila)):

The pattern of cellular proliferation and differentiation that leads to normal development of embryonic structures often depends upon the localized...

SUFUH (Suppressor of Fused Homolog (Drosophila)):

The Hedgehog signaling pathway plays an important role in early human development. The pathway is a signaling cascade that plays a role in pattern...
Antigène dans cette catégorie:
HHIP (Hedgehog Interacting Protein): HHIP anticorps HHIP ELISA Kits HHIP Proteins
KCTD11 - Potassium Channel Tetramerisation Domain Containing 11: KCTD11 anticorps KCTD11 ELISA Kits KCTD11 Proteins