DHH蛋白抗体

英文名称    DHH    

中文名称    DHH蛋白抗体    

别    名    Desert hedgehog (Drosophila) homolog; Desert hedgehog; Desert hedgehog homolog (Drosophila); Desert hedgehog protein precursor.    

说 明 书    0.2ml      

研究领域    免疫学  信号转导  干细胞      

抗体来源    Rabbit    

克隆类型    Polyclonal    

交叉反应    Human, Mouse, Rat, Dog, Pig, Cow, Horse,     

产品应用    WB=1:100-500 ELISA=1:500-1000 IP=1:20-100 IHC-P=1:100-500 IHC-F=1:100-500 IF=1:100-500 DHH蛋白抗体（石蜡切片需做抗原修复） 
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.    

分 子 量    44kDa    

性    状    Lyophilized or Liquid    

浓    度    1mg/1ml    

免 疫 原    KLH conjugated synthetic peptide derived from human DHH    

亚    型    IgG    

纯化方法    affinity purified by Protein A    

储 存 液    0.01M PBS, pH 7.4 with 10 mg/ml BSA and 0.1% Sodium azide    

保存条件    Store at -20 °C for one year. Avoid repeated freeze/thaw cycles. The lyophilized antibody is stable at room temperature for at least one month and for greater than a year when kept at -20°C. When reconstituted in sterile pH 7.4 0.01M PBS or diluent of antibody the antibody is stable for at least two weeks at 2-4 °C.    

DHH蛋白抗体产品介绍    background:

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.     

DHH蛋白抗体

hz-2751R Aurora A/B/C  有丝分裂激酶A/B/C抗体

hz-4118R LEO1  RNA聚合酶相关蛋白LEO1抗体

hz-1103R B7-H1/PD-L1/CD274  程序性死亡配体1抗体

hz-1868R B7-DC/CD273/PDL2  程序性死亡配体2抗体

hz-2471R ICOS-L/B7-H2/CD275  诱导协同刺激分子配体CD275抗体

hz-5410R phospho-LEO1(Ser551)  磷酸化RNA聚合酶相关蛋白LEO1抗体

hz-4661R ICOS-L/B7-H2/CD275  诱导协同刺激分子配体CD275抗体

hz-4164R CT054/C20orf54  核黄素转运蛋白2抗体

hz-4229R ZDHHC21  γ-氨基丁酸A受体相关膜蛋白3

hz-4119R ASGPR1/ASGR1  唾液酸糖蛋白受体1抗体

hz-4242R ITPR1  5-三磷酸肌醇受体1抗体

hz-4126R GPI  糖磷脂酰肌醇抗体

hz-0673R B7H4  B7-H4抗体

hz-4243R ITPR2  5-三磷酸肌醇受体2抗体

hz-5040R BAAT  长链脂肪酸酰基辅酶A水解酶抗体

hz-4121R Exendin 4  GLP1类似蛋白Exendin4抗体

hz-0164R BACE1/ASP2  β分泌酶抗体

hz-5215R phospho-BACE1(Ser498)  磷酸化β分泌酶抗体

hz-5216R Phospho-Bad(Ser118)  磷酸化相关死亡促进因子抗体

hz-5217R Phospho-Bad(Ser134)  磷酸化相关死亡促进因子抗体

hz-5219R phospho-BAD(Ser99)  磷酸化相关死亡促进因子抗体

hz-0892R Bad  相关死亡促进因子Bad抗体

hz-0893R phospho-BAD(Ser128)  磷酸化相关死亡促进因子抗体

hz-3056R Phospho-Bad(Ser136)  磷酸化相关死亡促进因子抗体

hz-3057R Phospho-Bad(Ser155)  磷酸化相关死亡促进因子抗体

hz-1304R Bad  相关死亡促进因子Bad抗体

hz-3040R Phospho-Bad(Ser112)  磷酸化相关死亡促进因子抗体

hz-3009R phospho-BAD(Ser75)  磷酸化相关死亡促进因子抗体

hz-5799R BAG3  Bcl2抑凋亡蛋白Bag3抗体

hz-5134R BAG2  Bcl-2结合抑凋亡蛋白2抗体

hz-3664R Bag1/RAP46  抑凋亡基因bag1抗体

hz-1284R Bak  Bcl-2同源拮抗剂抗体