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Key Specifications Table
| Species Reactivity | Key Applications | Host | Format | Antibody Type |
|---|---|---|---|---|
| R, H | WB, IHC | M | Purified | Monoclonal Antibody |
| Description | |
|---|---|
| Catalogue Number | 04-967 |
| Description | Anti-Desert hedgehog protein (DHH) Antibody, clone 19D7.2 |
| Alternate Names |
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| Background Information | Desert Hedgehog (DHH) is one of three signaling molecules belonging to the hedgehog family, a group of intracellular signaling molecules characterized for their regulatory ability in developmental tissue patterning. DHH is an essential component to the integrity of peripheral nerve function and structure. There is also strong support that it is critical to normal, testicular development, and the survival of spermatocytes. DHH cleaves into two chains; the DHH N-product which is localized at the cell surface, and the DHH C-product which diffuses from the cell. Defects in expression are though to cause complete pure gonadal dysgenesis 46,XY type (GDXYM), and partial gonadal dysgenesis with minifascicular neuropathy 46, XY (PGD). |
| Product Information | |
|---|---|
| Format | Purified |
| Control |
|
| Presentation | Purified mouse monoclonal IgMκ in buffer containing PBS with 0.05% sodium azide. |
| Quality Level | MQ100 |
| Applications | |
|---|---|
| Application | Detect Desert hedgehog protein (DHH) using this Anti-Desert hedgehog protein (DHH) Antibody, clone 19D7.2 validated for use in WB, IH. |
| Key Applications |
|
| Application Notes | Immunohistochemistry Analysis: 1:600 dilution from a previous lot detected Desert hedgehog protein in malignant teratoma tissue. |
| Biological Information | |
|---|---|
| Immunogen | KLH-conjugated linear peptide corresponding to human Desert hedgehog protein. |
| Epitope | Unknown |
| Clone | 19D7.2 |
| Concentration | Please refer to the Certificate of Analysis for the lot-specific concentration. |
| Host | Mouse |
| Isotype | IgMκ |
| Species Reactivity |
|
| Antibody Type | Monoclonal Antibody |
| Entrez Gene Number |
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| Entrez Gene Summary | 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]. |
| Gene Symbol |
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| Purification Method | Ion Exchange |
| UniProt Number |
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| UniProt Summary | 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). 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). INVOLVEMENT IN 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. SEQUENCE SIMILARITIES: Belongs to the hedgehog family. |
| Molecular Weight | ~ 44 kDa observed |
| Product Usage Statements | |
|---|---|
| Quality Assurance | Evaluated by Western Blot in rat spinal cord tissue lysate. Western Blot Analysis: 1 µg/mL of this antibody detected Desert hedgehog protein on 10 µg of rat spinal cord tissue lysate. |
| Usage Statement |
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| Storage and Shipping Information | |
|---|---|
| Storage Conditions | Stable for 1 year at 2-8°C from date of receipt. |
| Packaging Information | |
|---|---|
| Material Size | 100 µg |