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Hedgehog Signalweg

Der Hedgehog(Hh)-Signalweg tritt bei Chordaten unterschiedlicher taxonomischer Einordnung in hochkonservierter Form auf. Er ist wesentlicher Bestandteil der Embryonalentwicklung. Der Hedgehog-Signalweg wurde erstmals anhand der Fruchtfliege Drosophila melanogaster beschrieben und abgegrenzt. Bei Säugetieren reguliert der Hedgehog-Signalweg das Zellschicksal, die Gewebepolarität, die Musterbildung in der frühen Embryogenese sowie die Morphogenese bei bestimmten Organen und Geweben. In einem Großteil des adulten Gewebes ist er stillgelegt, kann aber infolge von Verletzungen zur Unterstützung der Reparatur und Neubildung reaktiviert werden.

Unterschiedliche Hedgehog-Liganden, darunter die homologen Proteine Sonic Hedgehog (Shh), Indian Hedgehog (Ihh) und Desert Hedgehog (Dhh), werden als Vorstufe gebildet, welche vor der Sekretion die autokatalytische Spaltung, eine C-terminale Cholesterinbindung und eine N-terminale Palmitoylierung durchlaufen. Die Freisetzung und extrazelluläre Akkumulation der gereiften Liganden wird von den Homologen des Drosophila Dispatched-Proteins reguliert. Die Bindung des sekretierten Hedgehog-Liganden an das homologe Rezeptorprotein (PTCH1, PTCH2) bewirkt die Stimulierung des Signalnetzwerks: Die unterdrückende Wirkung des PTHC auf den transmembranen Rezeptor SMO wird abgeschwächt, wodurch Gliom-assoziierte onkogene (GLI-)Transkriptionsfaktoren aktiviert werden.

Eine der wichtigsten spezialisierten Strukturen in diesem Prozess ist die auf Mikrotubuli basierende primäre Zilie. Bei Nichteinsetzen des Hedgehog-Signals konzentrieren sich unverarbeitete, nicht-aktivierende Proteine sowie ihr Regulator SUFU in der distalen Spitze der primären Zilie. Nach der Bindung des Hedgehog-Liganden verlagert sich PTCH an die Zelloberfläche und ermöglicht somit die Verlagerung von SMO zur primären Zilie sowie die Herunterregulierung von SUFU. Zahlreiche primäre Zilien von maßgeblicher Struktur haben ebenfalls eine regulatorische Wirkung auf den Hedgehog-Signalweg.

Eine anomale Hh/GLI-Regulierung führt zu schwerwiegenden Gewebestörungen und der Entwicklung einer Reihe aggressiver Krebsarten. Der Hh/GLI-Signalweg ist außerdem mit der Genregulation von Stammzellen sowie dem Überleben von Krebsstammzellen verknüpft.

Pathway Abbildung als PDF speichern.

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...
Antigene in dieser Kategorie:
HHATL - Hedgehog Acyltransferase-Like: HHATL Antikörper   HHATL Proteine
PRKACA (Protein Kinase A, alpha): PRKACA Antikörper PRKACA ELISA Kits PRKACA Proteine
PRKACB (Protein Kinase, CAMP Dependent, Catalytic, beta): PRKACB Antikörper PRKACB ELISA Kits PRKACB Proteine
PRKACG (Protein Kinase, CAMP-Dependent, Catalytic, gamma): PRKACG Antikörper   PRKACG Proteine
PROKR1 - Prokineticin Receptor 1: PROKR1 Antikörper PROKR1 ELISA Kits PROKR1 Proteine
PRKAR1A - Protein Kinase, CAMP-Dependent, Regulatory, Type I, alpha (Tissue Specific Extinguisher 1): PRKAR1A Antikörper PRKAR1A ELISA Kits PRKAR1A Proteine
PRKAR1B (Protein Kinase, CAMP-Dependent, Regulatory, Type I, beta): PRKAR1B Antikörper PRKAR1B ELISA Kits PRKAR1B Proteine
PROKR2 - Prokineticin Receptor 2: PROKR2 Antikörper PROKR2 ELISA Kits PROKR2 Proteine
PRKAR2A - Protein Kinase, CAMP-Dependent, Regulatory, Type II, alpha: PRKAR2A Antikörper PRKAR2A ELISA Kits PRKAR2A Proteine
PRKAR2B - Protein Kinase, CAMP-Dependent, Regulatory, Type II, beta: PRKAR2B Antikörper PRKAR2B ELISA Kits PRKAR2B Proteine
CSNK1A1 (Casein Kinase 1, alpha 1): CSNK1A1 Antikörper CSNK1A1 ELISA Kits CSNK1A1 Proteine
CSNK1D - Casein Kinase 1 delta: CSNK1D Antikörper CSNK1D ELISA Kits CSNK1D Proteine
CSNK1E - CK1 epsilon: CSNK1E Antikörper CSNK1E ELISA Kits CSNK1E Proteine
CSNK1G1 - Casein Kinase 1, gamma 1: CSNK1G1 Antikörper CSNK1G1 ELISA Kits CSNK1G1 Proteine
CSNK1G2 - Casein Kinase 1 gamma 2: CSNK1G2 Antikörper CSNK1G2 ELISA Kits CSNK1G2 Proteine
CSNK1G3 - Casein Kinase 1, gamma 3: CSNK1G3 Antikörper CSNK1G3 ELISA Kits CSNK1G3 Proteine
GSK3b - GSK3 beta: GSK3b Antikörper    
SKP1 (S-Phase Kinase-Associated Protein 1): SKP1 Antikörper SKP1 ELISA Kits SKP1 Proteine
CUL1 - Cullin 1: CUL1 Antikörper CUL1 ELISA Kits CUL1 Proteine
BTRC (beta-Transducin Repeat Containing): BTRC Antikörper BTRC ELISA Kits BTRC Proteine

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...
Antigene in dieser Kategorie:
PTCH2 - Patched 2: PTCH2 Antikörper   PTCH2 Proteine
(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...
Antigene in dieser Kategorie:
EVC2 - Ellis Van Creveld Syndrome 2: EVC2 Antikörper EVC2 ELISA Kits EVC2 Proteine
DYNC2H1 (Dynein, Cytoplasmic 2, Heavy Chain 1):   DYNC2H1 ELISA Kits  
DYNC2LI1 (Dynein, Cytoplasmic 2, Light Intermediate Chain 1): DYNC2LI1 Antikörper DYNC2LI1 ELISA Kits DYNC2LI1 Proteine
KIF3A (Kinesin Family Member 3A): KIF3A Antikörper KIF3A ELISA Kits KIF3A Proteine
KIF3B (Kinesin Family Member 3B): KIF3B Antikörper KIF3B ELISA Kits KIF3B Proteine
KIF3C - Kinesin Family Member 3C: KIF3C Antikörper KIF3C ELISA Kits KIF3C Proteine
KIFAP3 - Kinesin Associated Protein 3: KIFAP3 Antikörper KIFAP3 ELISA Kits KIFAP3 Proteine
TRIM28 - Tripartite Motif Containing 28: TRIM28 Antikörper TRIM28 ELISA Kits TRIM28 Proteine
IFT43 (Intraflagellar Transport 43 Homolog (Chlamydomonas)): IFT43 Antikörper IFT43 ELISA Kits IFT43 Proteine
WDR35 (WD Repeat Domain 35): WDR35 Antikörper WDR35 ELISA Kits  
TTC21B (Tetratricopeptide Repeat Domain 21B): TTC21B Antikörper TTC21B ELISA Kits  
IFT140 - Intraflagellar Transport 140 Homolog (Chlamydomonas): IFT140 Antikörper    
WDR19 - WD Repeat Domain 19: WDR19 Antikörper WDR19 ELISA Kits WDR19 Proteine
IFT20 (Intraflagellar Transport 20 Homolog (Chlamydomonas)): IFT20 Antikörper IFT20 ELISA Kits IFT20 Proteine
PDK4 (Pyruvate Dehydrogenase Kinase, Isozyme 4): PDK4 Antikörper PDK4 ELISA Kits PDK4 Proteine
IFT27 (Intraflagellar Transport 27 Homolog (Chlamydomonas)): IFT27 Antikörper IFT27 ELISA Kits IFT27 Proteine
IFT46 (Intraflagellar Transport 46 Homolog (Chlamydomonas)): IFT46 Antikörper IFT46 ELISA Kits IFT46 Proteine
TRAF3IP1 (TNF Receptor-Associated Factor 3 Interacting Protein 1): TRAF3IP1 Antikörper TRAF3IP1 ELISA Kits TRAF3IP1 Proteine
IFT57 (Intraflagellar Transport 57 Homolog (Chlamydomonas)): IFT57 Antikörper IFT57 ELISA Kits IFT57 Proteine
TTC30B (Tetratricopeptide Repeat Domain 30B): TTC30B Antikörper TTC30B ELISA Kits TTC30B Proteine
IFT74 (Intraflagellar Transport Protein 74 Homolog): IFT74 Antikörper    
IFT80 (Intraflagellar Transport 80 Homolog (Chlamydomonas)): IFT80 Antikörper IFT80 ELISA Kits IFT80 Proteine
IFT81 - Intraflagellar Transport 81 Homolog (Chlamydomonas): IFT81 Antikörper IFT81 ELISA Kits IFT81 Proteine
IFT88 (Intraflagellar Transport 88 Homolog (Chlamydomonas)): IFT88 Antikörper IFT88 ELISA Kits IFT88 Proteine
BBS1 (Bardet-Biedl Syndrome 1): BBS1 Antikörper   BBS1 Proteine
BBS2 (Bardet-Biedl Syndrome 2): BBS2 Antikörper   BBS2 Proteine
BBS4 (Bardet-Biedl Syndrome 4): BBS4 Antikörper   BBS4 Proteine
BBS5 - Bardet-Biedl Syndrome 5: BBS5 Antikörper   BBS5 Proteine
BBS7 (Bardet-Biedl Syndrome 7): BBS7 Antikörper   BBS7 Proteine
TTC8 (Tetratricopeptide Repeat Domain 8): TTC8 Antikörper TTC8 ELISA Kits TTC8 Proteine
BBS9 (Bardet-Biedl Syndrome 9): BBS9 Antikörper   BBS9 Proteine

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...
Antigene in dieser Kategorie:
HHIP (Hedgehog Interacting Protein): HHIP Antikörper HHIP ELISA Kits HHIP Proteine
KCTD11 - Potassium Channel Tetramerisation Domain Containing 11: KCTD11 Antikörper KCTD11 ELISA Kits KCTD11 Proteine