DNAJC5 Antibody

Code CSB-PA001802
Size US$100
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  • Western Blot analysis of LOVO cells using CSP Polyclonal Antibody
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Product Details

Uniprot No.
Target Names
DNAJC5
Alternative Names
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
Raised in
Rabbit
Species Reactivity
Human,Mouse,Rat
Immunogen
Synthesized peptide derived from the C-terminal region of Human CSP.
Immunogen Species
Homo sapiens (Human)
Conjugate
Non-conjugated
Isotype
IgG
Purification Method
The antibody was affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogen.
Concentration
It differs from different batches. Please contact us to confirm it.
Buffer
Liquid in PBS containing 50% glycerol, 0.5% BSA and 0.02% sodium azide.
Form
Liquid
Tested Applications
WB, ELISA
Recommended Dilution
Application Recommended Dilution
WB 1:500-1:2000
ELISA 1:10000
Troubleshooting and FAQs
Storage
Upon receipt, store at -20°C or -80°C. Avoid repeated freeze.
Lead Time
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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Target Background

Function
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.
Gene References into Functions
  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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Involvement in disease
Ceroid lipofuscinosis, neuronal, 4B (CLN4B)
Subcellular Location
Cytoplasm, cytosol. Membrane; Lipid-anchor. Cytoplasmic vesicle, secretory vesicle, chromaffin granule membrane. Melanosome. Cell membrane.
Tissue Specificity
Expressed in pancreas, kidney, skeletal muscle, liver, lung, placenta, brain and heart.
Database Links

HGNC: 16235

OMIM: 162350

KEGG: hsa:80331

STRING: 9606.ENSP00000354111

UniGene: Hs.164419

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