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ATP7B

ATP7B(ATPase copper transporting beta),是一種跨膜ATP酶蛋白。它在細胞內(nèi)負責調(diào)節(jié)銅元素的轉(zhuǎn)運和代謝。ATP7B的結(jié)構(gòu)包括多個功能區(qū)域,其中包括多個轉(zhuǎn)運結(jié)構(gòu)域和催化ATP水解的結(jié)構(gòu)域。這些結(jié)構(gòu)域協(xié)同作用,使得ATP7B能夠在細胞膜上運輸銅離子。 ATP7B主要表達在肝臟細胞的高銅含量亞細胞器中,如高銅含量的高爾基體和胞質(zhì)溶酶體。它參與了細胞內(nèi)銅離子的轉(zhuǎn)運和分配,將銅從細胞質(zhì)輸送到高爾基體或從高爾基體釋放到胞質(zhì)溶酶體。 ATP7B的功能對于維持體內(nèi)銅平衡和避免銅積累至毒性水平非常重要。銅是生命過程中的關(guān)鍵元素,但在高濃度下會對細胞和組織產(chǎn)生毒性作用。因此,ATP7B的異常功能或突變可能導(dǎo)致Wilsons病,這是一種遺傳性銅代謝紊亂疾病。對ATP7B的研究對于理解銅代謝的調(diào)控機制以及Wilsons病的發(fā)病機理非常重要。同時,ATP7B也成為了治療Wilsons病的一個潛在靶點,研發(fā)針對ATP7B的治療方法有望改善這種疾病的治療效果。

熱銷產(chǎn)品

ATP7B Recombinant Monoclonal Antibody CSB-RA175460A0HU

驗證數(shù)據(jù)

IHC
CSB-RA175460A0HU IHC1

IHC image of CSB-RA175460A0HU diluted at 1:50 and staining in paraffin-embedded human brain tissue performed on a Leica BondTM system. After dewaxing and hydration, antigen retrieval was mediated by high pressure in a citrate buffer (pH 6.0). Section was blocked with 10% normal goat serum 30min at RT. Then primary antibody (1% BSA) was incubated at 4°C overnight. The primary is detected by a Goat anti-rabbit polymer IgG labeled by HRP and visualized using 0.19% DAB

IHC
CSB-RA175460A0HU IHC2

IHC image of CSB-RA175460A0HU diluted at 1:50 and staining in paraffin-embedded human ovarian cancer performed on a Leica BondTM system. After dewaxing and hydration, antigen retrieval was mediated by high pressure in a citrate buffer (pH 6.0). Section was blocked with 10% normal goat serum 30min at RT. Then primary antibody (1% BSA) was incubated at 4°C overnight. The primary is detected by a Goat anti-rabbit polymer IgG labeled by HRP and visualized using 0.19% DAB

IF
CSB-RA175460A0HU IF

Immunofluorescence staining of HepG2 with CSB-RA175460A0HU at 1:20, counter-stained with DAPI. The cells were fixed in 4% formaldehyde and blocked in 10% normal Goat Serum. The cells were then incubated with the antibody overnight at 4°C. The secondary antibody was Alexa Fluor 494-congugated AffiniPure Goat Anti-Rabbit IgG(H+L).

FC
CSB-RA175460A0HU FC

Overlay Peak curve showing HepG2 cells stained with CSB-RA175460A0HU (red line) at 1:50. The cells were fixed in 4% formaldehyde and permeated by 0.2% TritonX-100. Then 10% normal goat serum to block non-specific protein-protein interactions followed by the antibody (1μg/1*106cells) for 45min at 4℃. The secondary antibody used was FITC-conjugated Goat Anti-rabbit IgG(H+L) at 1:200 dilution for 35min at 4℃.Control antibody (green line) was rabbit IgG (1μg/1*106cells) used under the same conditions. Acquisition of >10,000 events was performed.

ATP7B Antibodies

ATP7B for Homo sapiens (Human)

ATP7B Proteins

ATP7B Proteins for Homo sapiens (Human)

ATP7B Proteins for Rattus norvegicus (Rat)

ATP7B Proteins for Mus musculus (Mouse)

ATP7B Proteins for Ovis aries (Sheep)