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Product Name DHH Chinese Name DHH蛋白抗体 Alias Desert hedgehog (Drosophila) homolog; Desert hedgehog; Desert hedgehog homolog (Drosophila); Desert hedgehog protein precursor. literatures Specific References (1) | SL5910R has been referenced in 1 publications.[IF=2.705] Wu J et al. Nicotine inhibits murine Leydig cell differentiation and maturation via regulating Hedgehog signal pathway. Biochem Biophys Res Commun. 2019 Feb 26;510(1):1-7. WB ; Mouse.Research Area immunology Signal transduction Stem cells Immunogen Species Rabbit Clonality Polyclonal React Species (predicted: Human, Mouse, Rat, Dog, Pig, Cow, Horse, ) Applications WB=1:500-2000 ELISA=1:5000-10000 IHC-P=1:100-500 IHC-F=1:100-500 IF=1:100-500 (Paraffin sections need antigen repair)
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.Theoretical molecular weight 44kDa Cellular localization The cell membrane Extracellular matrix Secretory protein Form Liquid Concentration 1mg/ml immunogen KLH conjugated synthetic peptide derived from human DHH: 101-200/396 Lsotype IgG Purification affinity purified by Protein A Buffer Solution 0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol. Storage Shipped at 4℃. Store at -20 °C for one year. Avoid repeated freeze/thaw cycles. Attention This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications. PubMed PubMed Product Detail 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.
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.
Subcellular Location:
Desert hedgehog protein N-product: Cell membrane; Lipid-anchor; Extracellular side (By similarity). Note=The N-terminal peptide remains associated with the cell surface (By similarity).
Desert hedgehog protein C-product: Secreted, extracellular space (By similarity). Note=The C-terminal peptide diffuses from the cell (By similarity).
Post-translational modifications:
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).
DISEASE:
Defects in DHH may be the cause of partial gonadal dysgenesis with minifascicular neuropathy 46,XY (PGD) [MIM:607080]. PGD is 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.
Defects in DHH may be the cause of complete pure gonadal dysgenesis 46,XY type (GDXYM) [MIM:233420]; also known as male-limited gonadal dysgenesis 46,XY. GDXYM is a type of hypogonadism in which no functional gonads are present to induce puberty in an externally female person whose karyotype is then found to be XY. The gonads are found to be non-functional streaks.
Similarity:
Belongs to the hedgehog family.
SWISS:
O43323
Gene ID:
50846
Database links:Entrez Gene: 50846 Human
Entrez Gene: 13363 Mouse
Omim: 605423 Human
SwissProt: O43323 Human
SwissProt: Q61488 Mouse
Unigene: 524382 Human
Unigene: 324688 Mouse
Unigene: 384073 Mouse
Unigene: 84274 Rat
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