Recombinant Human DnaJ homolog subfamily C member 5 (DNAJC5)

中文名稱：

人DNAJC5重組蛋白
貨號：

CSB-YP863975HU
說明書：

MSDS Datasheet
規格：
來源：

Yeast
其他：

中文名稱：

人DNAJC5重組蛋白
貨號：

CSB-EP863975HU
說明書：

MSDS Datasheet
規格：
來源：

E.coli
其他：

中文名稱：

人DNAJC5重組蛋白
貨號：

CSB-BP863975HU
說明書：

MSDS Datasheet
規格：
來源：

Baculovirus
其他：

中文名稱：

人DNAJC5重組蛋白
貨號：

CSB-MP863975HU
說明書：

MSDS Datasheet
規格：
來源：

Mammalian cell
其他：

產品詳情
產品評價
相關產品
靶點詳情

產品詳情

純度：

>85% (SDS-PAGE)
基因名：

DNAJC5
Uniprot No.：

Q9H3Z4
別名：

CLN 4; CLN4; CLN4B; CSP; cysteine string protein alpha; Cysteine string protein; DJC5; DnaJ (Hsp40) homolog subfamily C member 5; DnaJ homolog subfamily C member 5; DNAJC 5; Dnajc5; DNAJC5A; DNJC5_HUMAN; NCL
種屬：

Homo sapiens (Human)
蛋白長度：

full length protein
表達區域：

1-198
氨基酸序列

MADQRQRSLS TSGESLYHVL GLDKNATSDD IKKSYRKLAL KYHPDKNPDN PEAADKFKEI NNAHAILTDA TKRNIYDKYG SLGLYVAEQF GEENVNTYFV LSSWWAKALF VFCGLLTCCY CCCCLCCCFN CCCGKCKPKA PEGEETEFYV SPEDLEAQLQ SDEREATDTP IVIQPASATE TTQLTADSHP SYHTDGFN
蛋白標簽：

Tag?type?will?be?determined?during?the?manufacturing?process.

The tag type will be determined during production process. If you have specified tag type, please tell us and we will develop the specified tag preferentially.
產品提供形式：

Lyophilized powder Warning: in_array() expects parameter 2 to be array, null given in /www/web/cusabio_cn/public_html/caches/caches_template/default/content/show_product_protein.php on line 668
Note: We will preferentially ship the format that we have in stock, however, if you have any special requirement for the format, please remark your requirement when placing the order, we will prepare according to your demand.
復溶：

We recommend that this vial be briefly centrifuged prior to opening to bring the contents to the bottom. Please reconstitute protein in deionized sterile water to a concentration of 0.1-1.0 mg/mL.We recommend to add 5-50% of glycerol (final concentration) and aliquot for long-term storage at -20℃/-80℃. Our default final concentration of glycerol is 50%. Customers could use it as reference.
儲存條件：

Store at -20°C/-80°C upon receipt, aliquoting is necessary for mutiple use. Avoid repeated freeze-thaw cycles.
保質期：

The shelf life is related to many factors, storage state, buffer ingredients, storage temperature and the stability of the protein itself.
Generally, the shelf life of liquid form is 6 months at -20°C/-80°C. The shelf life of lyophilized form is 12 months at -20°C/-80°C.
貨期：

Delivery time may differ from different purchasing way or location, please kindly consult your local distributors for specific delivery time.
Note: All of our proteins are default shipped with normal blue ice packs, if you request to ship with dry ice, please communicate with us in advance and extra fees will be charged.
注意事項：

Repeated freezing and thawing is not recommended. Store working aliquots at 4°C for up to one week.
Datasheet ：

Please contact us to get it.

產品評價

靶點詳情

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.
基因功能參考文獻：
1. 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
2. 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
3. Phosphorylation of CSP triggers a major conformational switch that modulates its protein interactions. PMID: 27452402
4. 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
5. This study demonstrated that Neuronal ceroid lipofuscinosis with DNAJC5/CSPalpha mutation has PPT1 pathology and exhibit aberrant protein palmitoylation. PMID: 26659577
6. 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
7. the presynaptic vesicle protein CSPalpha is a key player in synaptic degeneration and protection in Alzheimer's disease. PMID: 25631211
8. Missense mutations in DNAJC5 does not play a major role in PD in the Chinese population. PMID: 24126164
9. Results indicate of a p.L116del mutation in DNAJC5 from families with autosomal dominant Kufs disease. PMID: 22978711
10. Palmitoylation-induced aggregation of mutant CSP-alpha proteins may underlie the development of adult-onset neuronal ceroid lipofuscinosis PMID: 22902780
11. association of DNAJC5 mutations with autosomal dominant Kufs disease PMID: 22235333
12. 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
13. A neuroprotective role for CSPalpha in humans is confirmed. PMID: 21820099
14. First evidence that CSP and HSP70, and their interactions with MARCKS, are involved in mucin secretion from airway epithelium. PMID: 18314541
15. 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
16. Csp has a role in regulated CFTR trafficking at the plasma membrane. [CYSTEINE STRING PROTEIN] PMID: 12039948
17. Cysteine string protein inhibits N-type calcium channels, but is blocked by mutant huntingtin PMID: 14570907
18. 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
19. Cysteine string protein monitors late steps in cystic fibrosis transmembrane conductance regulator biogenesis PMID: 16469739
20. palmitoylation of CSP is enhanced specifically by co-expression of the Golgi-localized palmitoyl transferases DHHC3, DHHC7, DHHC15, or DHHC17 PMID: 18596047
21. Has a role in exocytosis of large dense core vesicles. PMID: 10194413
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相關疾病：

Ceroid lipofuscinosis, neuronal, 4B (CLN4B)
亞細胞定位：

Cytoplasm, cytosol. Membrane; Lipid-anchor. Cytoplasmic vesicle, secretory vesicle, chromaffin granule membrane. Melanosome. Cell membrane.
組織特異性：

Expressed in pancreas, kidney, skeletal muscle, liver, lung, placenta, brain and heart.
數據庫鏈接：

HGNC: 16235

OMIM: 162350

KEGG: hsa:80331

STRING: 9606.ENSP00000354111

UniGene: Hs.164419