货号：04-967

品牌：Merck Millipore

规格：100Ug

目录价：￥4438.00

市场价格：￥3772.30

会员价格：￥3550.40

金山科研平台，产品价格货期咨询微信：jinshanbio Description: Anti-Desert hedgehog protein (DHH) Antibody, clone 19D7.2 | 04-967 View All» Promotional Text: Special Shipping Offer on Antibodies100% Performance Guaranteed View All» Molecular Weight: ~ 44 kDa observed View All» Epitope: Unknown View All» Immunogen: KLH-conjugated linear peptide corresponding to human Desert hedgehog protein. View All» Clone: 19D7.2 View All» Isotype: IgMκ View All» 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). View All» Species Reactivity:

• Rat

• Human
  View All» Species Reactivity Note: Demonstrated to react with human and rat. View All» Application Notes: Immunohistochemistry Analysis: 1:600 dilution from a previous lot detected Desert hedgehog protein in malignant teratoma tissue. View All» Control: Rat spinal cord tissue lysate View All» 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. View All» Purification Method: Ion Exchange View All» Presentation: Purified mouse monoclonal IgMκ in buffer containing PBS with 0.05% sodium azide. View All» Storage Conditions: Stable for 1 year at 2-8°C from date of receipt. View All» UniProt Number: O43323 View All» Entrez Gene Number: NP_066382 View All» Gene Symbol:
  • DHH

  • HHG-3
    View All» Alternate Names:
    • desert hedgehog homolog (Drosophila)

    • desert hedgehog (Drosophila) homolog

    • desert hedgehog
      View All» Usage Statement: Unless otherwise stated in our catalog or other company documentation accompanying the product(s), our products are intended for research use only and are not to be used for any other purpose, which includes but is not limited to, unauthorized commercial uses, in vitro diagnostic uses, ex vivo or in vivo therapeutic uses or any type of consumption or application to humans or animals. View All» Key Applications:
      • Western Blotting

      • Immunohistochemistry
        View All» 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]. View All» 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. View All» Product Name: Anti-Desert hedgehog protein (DHH), clone 19D7.2 View All» Concentration: 1.0 mg/mL View All» Antibody Type: Monoclonal Antibody View All» Qty/Pk: 100 μg View All» Format: Purified View All» Host: Mouse View All»
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