DNAJC5 Antibody
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中文名稱：DNAJC5兔多克隆抗體
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貨號(hào)：CSB-PA001802
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規(guī)格：￥1090
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圖片：
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Western Blot analysis of LOVO cells using CSP Polyclonal Antibody
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其他：
產(chǎn)品詳情
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Uniprot No.：
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基因名：
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別名：CLN 4 antibody; CLN4 antibody; CLN4B antibody; CSP antibody; cysteine string protein alpha antibody; Cysteine string protein antibody; DJC5 antibody; DnaJ (Hsp40) homolog subfamily C member 5 antibody; DnaJ homolog subfamily C member 5 antibody; DNAJC 5 antibody; Dnajc5 antibody; DNAJC5A antibody; DNJC5_HUMAN antibody; NCL antibody
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宿主：Rabbit
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反應(yīng)種屬：Human,Mouse,Rat
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免疫原：Synthesized peptide derived from the C-terminal region of Human CSP.
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免疫原種屬：Homo sapiens (Human)
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標(biāo)記方式：Non-conjugated
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抗體亞型：IgG
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純化方式：The antibody was affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogen.
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濃度：It differs from different batches. Please contact us to confirm it.
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保存緩沖液：Liquid in PBS containing 50% glycerol, 0.5% BSA and 0.02% sodium azide.
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產(chǎn)品提供形式：Liquid
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應(yīng)用范圍：WB, ELISA
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推薦稀釋比：
Application Recommended Dilution WB 1:500-1:2000 ELISA 1:10000 -
Protocols：
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儲(chǔ)存條件：Upon receipt, store at -20°C or -80°C. Avoid repeated freeze.
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貨期：Basically, we can dispatch the products out in 1-3 working days after receiving your orders. Delivery time maybe differs from different purchasing way or location, please kindly consult your local distributors for specific delivery time.
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用途：For Research Use Only. Not for use in diagnostic or therapeutic procedures.
產(chǎn)品評(píng)價(jià)
相關(guān)產(chǎn)品
靶點(diǎn)詳情
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功能：Acts as a general chaperone in regulated exocytosis. Acts as a co-chaperone for the SNARE protein SNAP-25. Involved in the calcium-mediated control of a late stage of exocytosis. May have an important role in presynaptic function. May be involved in calcium-dependent neurotransmitter release at nerve endings.
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基因功能參考文獻(xiàn)：
- Results indicate that by assisting local lysosome/proteasome processes, CSPalpha-mediated removal of toxic proteins via extracellular vesicles plays a central role in synaptic proteostasis. PMID: 28424476
- The importance of specific residues in the cysteine-string domain was investigated, revealing that a central core of palmitoylated cysteines is essential for aggregation of adult-onset neuronal ceroid lipofuscinosis CSPalpha L115R/L116 mutants. PMID: 28127059
- Phosphorylation of CSP triggers a major conformational switch that modulates its protein interactions. PMID: 27452402
- In fact, DnaJC5 overexpression induced tau release in cells, neurons, and brain tissue, but only when activity of the chaperone Hsc70 was intact and when tau was able to associate with this chaperone. PMID: 27261198
- This study demonstrated that Neuronal ceroid lipofuscinosis with DNAJC5/CSPalpha mutation has PPT1 pathology and exhibit aberrant protein palmitoylation. PMID: 26659577
- These results suggest that the degeneration seen in the patients with AD-ANCL reported here might be a consequence of both the early effects of CSPalpha mutations at the cellular soma. PMID: 26610600
- the presynaptic vesicle protein CSPalpha is a key player in synaptic degeneration and protection in Alzheimer's disease. PMID: 25631211
- Missense mutations in DNAJC5 does not play a major role in PD in the Chinese population. PMID: 24126164
- Results indicate of a p.L116del mutation in DNAJC5 from families with autosomal dominant Kufs disease. PMID: 22978711
- Palmitoylation-induced aggregation of mutant CSP-alpha proteins may underlie the development of adult-onset neuronal ceroid lipofuscinosis PMID: 22902780
- association of DNAJC5 mutations with autosomal dominant Kufs disease PMID: 22235333
- This is the first replication study of the identification of DNAJC5 as the disease-causing gene for autosomal dominant ANCL. The identification of the novel gene in ANCL will allow us to gain a better understanding of the pathological mechanism of ANCLs PMID: 22073189
- A neuroprotective role for CSPalpha in humans is confirmed. PMID: 21820099
- First evidence that CSP and HSP70, and their interactions with MARCKS, are involved in mucin secretion from airway epithelium. PMID: 18314541
- Csp not only regulates the exit of CFTR from the ER, but this action is accompanied by Hsc70/Hsp70 and CHIP-mediated CFTR degradation. PMID: 19098309
- Csp has a role in regulated CFTR trafficking at the plasma membrane. [CYSTEINE STRING PROTEIN] PMID: 12039948
- Cysteine string protein inhibits N-type calcium channels, but is blocked by mutant huntingtin PMID: 14570907
- CSP modulates G protein function by preferentially targeting the inactive GDP-bound form of G alpha(s) and promoting GDP/GTP exchange; the guanine nucleotide exchange activity of full-length CSP is regulated by Hsc70-SGT PMID: 15972823
- Cysteine string protein monitors late steps in cystic fibrosis transmembrane conductance regulator biogenesis PMID: 16469739
- palmitoylation of CSP is enhanced specifically by co-expression of the Golgi-localized palmitoyl transferases DHHC3, DHHC7, DHHC15, or DHHC17 PMID: 18596047
- Has a role in exocytosis of large dense core vesicles. PMID: 10194413
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相關(guān)疾病：Ceroid lipofuscinosis, neuronal, 4B (CLN4B)
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亞細(xì)胞定位：Cytoplasm, cytosol. Membrane; Lipid-anchor. Cytoplasmic vesicle, secretory vesicle, chromaffin granule membrane. Melanosome. Cell membrane.
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組織特異性：Expressed in pancreas, kidney, skeletal muscle, liver, lung, placenta, brain and heart.
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