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Hedgehog Signaling

刺猬(Hh)因子信号在不同分类学分类的脊索动物中高度保留。对于胚胎的发育来说其至关重要。第一次成功描绘和表征刺猬因子途径是在黑腹果蝇中。在哺乳动物中,Hh因子信号在胚胎早期形成以及具体器官和组织形态形成的过程中调节细胞命运、组织极性和样式。随后就在大多数成熟组织中不工作了,但是在有损伤的情况下可以重新激活来促进修复和再生。

不同的Hh因子配位体,包括:同系物音刺猬(SHH)因子、印度刺猬(IHH)因子和沙漠刺猬因子(DHH),已经生产出来作为在分泌之前经过自催化切割、C端胆固醇附着、N端棕榈酰化的前体。通过果蝇脱出(Disp)蛋白的同系物调节成熟配位体的释放和细胞外积聚。分泌的Hh因子配位体与受体同系物(PTCH1、PTCH2)连接刺激了信号网络:跨膜受体SMO上PTCH的抑制效果减轻,从而激活胶质瘤相关致癌基因(GLI)转录因子。

这个过程重要的专门结构是基于微管的初级纤毛。在没有未处理的Hh因子信号的情况下,未激活GLI蛋白及其调节因子SUFU集中在初级纤毛的远端。连接Hh因子配位体后,PTCH迁移到细胞表面,使得SMO易位到初级纤毛以及向下调节SUFU成为可能。很多结构上重要的初级纤毛蛋白也对Hh因子信号级联有调节作用。

畸变的Hh/GLI调节造成重性组织紊乱以及各种进展期癌症的发展。Hh/GLI级联也和干性基因的调节和癌症干细胞的存活有关。

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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...   更多...

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].   更多...
此分类里的别的抗原:
HHATL - Hedgehog Acyltransferase-Like: HHATL 抗体   HHATL 蛋白
PRKACA (Protein Kinase A, alpha): PRKACA 抗体 PRKACA ELISA试剂盒 PRKACA 蛋白
PRKACB (Protein Kinase, CAMP Dependent, Catalytic, beta): PRKACB 抗体 PRKACB ELISA试剂盒 PRKACB 蛋白
PRKACG (Protein Kinase, CAMP-Dependent, Catalytic, gamma): PRKACG 抗体   PRKACG 蛋白
PROKR1 - Prokineticin Receptor 1: PROKR1 抗体 PROKR1 ELISA试剂盒 PROKR1 蛋白
PRKAR1A - Protein Kinase, CAMP-Dependent, Regulatory, Type I, alpha (Tissue Specific Extinguisher 1): PRKAR1A 抗体 PRKAR1A ELISA试剂盒 PRKAR1A 蛋白
PRKAR1B (Protein Kinase, CAMP-Dependent, Regulatory, Type I, beta): PRKAR1B 抗体 PRKAR1B ELISA试剂盒 PRKAR1B 蛋白
PROKR2 - Prokineticin Receptor 2: PROKR2 抗体 PROKR2 ELISA试剂盒 PROKR2 蛋白
PRKAR2A - Protein Kinase, CAMP-Dependent, Regulatory, Type II, alpha: PRKAR2A 抗体 PRKAR2A ELISA试剂盒 PRKAR2A 蛋白
PRKAR2B - Protein Kinase, CAMP-Dependent, Regulatory, Type II, beta: PRKAR2B 抗体 PRKAR2B ELISA试剂盒 PRKAR2B 蛋白
CSNK1A1 (Casein Kinase 1, alpha 1): CSNK1A1 抗体 CSNK1A1 ELISA试剂盒 CSNK1A1 蛋白
CSNK1D - Casein Kinase 1 delta: CSNK1D 抗体 CSNK1D ELISA试剂盒 CSNK1D 蛋白
CSNK1E - CK1 epsilon: CSNK1E 抗体 CSNK1E ELISA试剂盒 CSNK1E 蛋白
CSNK1G1 - Casein Kinase 1, gamma 1: CSNK1G1 抗体 CSNK1G1 ELISA试剂盒 CSNK1G1 蛋白
CSNK1G2 - Casein Kinase 1 gamma 2: CSNK1G2 抗体 CSNK1G2 ELISA试剂盒 CSNK1G2 蛋白
CSNK1G3 - Casein Kinase 1, gamma 3: CSNK1G3 抗体 CSNK1G3 ELISA试剂盒 CSNK1G3 蛋白
GSK3b - GSK3 beta: GSK3b 抗体    
SKP1 (S-Phase Kinase-Associated Protein 1): SKP1 抗体 SKP1 ELISA试剂盒 SKP1 蛋白
CUL1 - Cullin 1: CUL1 抗体 CUL1 ELISA试剂盒 CUL1 蛋白
BTRC (beta-Transducin Repeat Containing): BTRC 抗体 BTRC ELISA试剂盒 BTRC 蛋白

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...   更多...
此分类里的别的抗原:
PTCH2 - Patched 2: PTCH2 抗体   PTCH2 蛋白
(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...   更多...

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.   更多...

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

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...   更多...

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...
此分类里的别的抗原:
HHIP (Hedgehog Interacting Protein): HHIP 抗体 HHIP ELISA试剂盒 HHIP 蛋白
KCTD11 - Potassium Channel Tetramerisation Domain Containing 11: KCTD11 抗体 KCTD11 ELISA试剂盒 KCTD11 蛋白