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DHH

This gene encodes a member of the Hedgehog family. The hedgehog gene family encodes signaling molecules that play an important role in regulating morphogenesis. This protein is predicted to be made as a precursor that is autocatalytically cleaved; the N-terminal portion is soluble and contains the signalling activity while the C-terminal portion is involved in precursor processing. More importantly, the C-terminal product covalently attaches a cholesterol moiety to the N-terminal product, restricting the N-terminal product to the cell surface and preventing it from freely diffusing throughout the organism. Defects in this protein have been associated with partial gonadal dysgenesis (PGD) accompanied by minifascicular polyneuropathy. This protein may be involved in both male gonadal differentiation and perineurial development. [provided by RefSeq]
Full Name
desert hedgehog homolog (Drosophila)
Function
Intercellular signal essential for a variety of patterning events during development. May function as a spermatocyte survival factor in the testes. Essential for testes development.
Biological Process
Cell-cell signaling Source: InterPro
Cell fate specification Source: GO_Central
Leydig cell differentiation Source: Ensembl
Male sex determination Source: Ensembl
Myelination Source: Ensembl
Osteoblast differentiation Source: Ensembl
Protein autoprocessing Source: InterPro
Regulation of gene expression Source: GO_Central
Regulation of steroid biosynthetic process Source: Ensembl
Response to estradiol Source: Ensembl
Response to estrogen Source: Ensembl
Smoothened signaling pathway Source: GO_Central
Spermatid development Source: Ensembl
Cellular Location
Desert hedgehog protein N-product: Cell membrane. The N-terminal peptide remains associated with the cell surface.
Desert hedgehog protein C-product: Extracellular space. The C-terminal peptide diffuses from the cell.
Involvement in disease
Partial gonadal dysgenesis with minifascicular neuropathy 46,XY (PGD):
Characterized by the presence of a testis on one side and a streak or an absent gonad at the other, persistence of Muellerian duct structures, and a variable degree of genital ambiguity.
46,XY sex reversal 7 (SRXY7):
A disorder of sex development. Affected individuals have a 46,XY karyotype but present as phenotypically normal females. SRXY7 patients have no functional gonads.
PTM
The C-terminal domain displays an autoproteolysis activity and a cholesterol transferase activity. Both activities result in the cleavage of the full-length protein and covalent attachment of a cholesterol moiety to the C-terminal of the newly generated N-terminal fragment (N-product). This covalent modification appears to play an essential role in restricting the spatial distribution of the protein activity to the cell surface. The N-product is the active species in both local and long-range signaling, whereas the C-product has no signaling activity (By similarity).

Anti-DHH antibodies

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Target: DHH
Host: Rat
Antibody Isotype: IgG2
Specificity: Mouse
Clone: 6A12
Application*: WB
Target: DHH
Host: Mouse
Antibody Isotype: IgG2a, κ
Specificity: Human
Clone: 4F10
Application*: E, WB
Target: DHH
Host: Mouse
Antibody Isotype: IgG
Specificity: Human, Mouse, Rat
Clone: D-4
Application*: WB, IP, IF, E
Target: DHH
Host: Mouse
Antibody Isotype: IgG2a, κ
Specificity: Human
Clone: 1B12
Application*: E, WB
Target: DHH
Host: Mouse
Antibody Isotype: IgG2a, κ
Specificity: Human
Clone: 3E6
Application*: WB, E
More Infomation
For Research Use Only. Not For Clinical Use.
(P): Predicted
* Abbreviations
IFImmunofluorescence
IHImmunohistochemistry
IPImmunoprecipitation
WBWestern Blot
EELISA
MMicroarray
CIChromatin Immunoprecipitation
FFlow Cytometry
FNFunction Assay
IDImmunodiffusion
RRadioimmunoassay
TCTissue Culture
GSGel Supershift
NNeutralization
BBlocking
AActivation
IInhibition
DDepletion
ESELISpot
DBDot Blot
MCMass Cytometry/CyTOF
CTCytotoxicity
SStimulation
AGAgonist
APApoptosis
IMImmunomicroscopy
BABioassay
CSCostimulation
EMElectron Microscopy
IEImmunoelectrophoresis
PAPeptide Array
ICImmunocytochemistry
PEPeptide ELISA
MDMeDIP
SHIn situ hybridization
IAEnzyme Immunoassay
SEsandwich ELISA
PLProximity Ligation Assay
ECELISA(Cap)
EDELISA(Det)
BIBioimaging
IOImmunoassay
LFLateral Flow Immunoassay
LALuminex Assay
CImmunohistochemistry-Frozen Sections
PImmunohistologyp-Paraffin Sections
ISIntracellular Staining for Flow Cytometry
MSElectrophoretic Mobility Shift Assay
RIRNA Binding Protein Immunoprecipitation (RIP)
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