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anti-Human DNAJC5 Antikörper:
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Human Polyclonal DNAJC5 Primary Antibody für ICC, IP - ABIN1742330
García-Junco-Clemente, Cantero, Gómez-Sánchez, Linares-Clemente, Martínez-López, Luján, Fernández-Chacón: Cysteine string protein-alpha prevents activity-dependent degeneration in GABAergic synapses. in The Journal of neuroscience : the official journal of the Society for Neuroscience 2010
Show all 5 Pubmed References
This review summarizes studies that elucidate the neuroprotective role of Drosophila CSP, which is critical for maintaining neurotransmitter release and preventing neurodegeneration.
these data suggest that CSP promotes synaptic growth and evoked neurotransmitter release by mechanistically independent signaling pathways
study found dHIP14 to be an essential maternal effect gene required for photoreceptor synaptic transmission and for proper in vivo expression of the palmitoylated presynaptic proteins SNAP-25 and cysteine string protein
Our data indicate that HIP14 controls neurotransmitter release by regulating the trafficking of CSP to synapses.
csp interaction with Hsc 70 molecular chaperones is vital for regulated secretion in Xenopus oocytes
Study demonstrate that primary dermal fibroblasts from asymptomatic mutation carriers recapitulate features of adult-neuronal ceroid lipofuscinosis (AD-ANCL) in vitro including CSPalpha-p.L115R/CSPalpha-WT aggregates and the structural and functional lysosomal dysfunction found in the brains of AD-ANCL patients. Further findings support a gain-of-function mechanism for CSPalpha mutations leading to AD-ANCL.
This report describes the clinical history of autosomal dominant Kufs disease, the genetic mutation within the DNAJC5 gene, and the neuropathological findings demonstrating depletion of choline acetyltransferase in the brain.
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.
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.
Phosphorylation of CSP triggers a major conformational switch that modulates its protein interactions.
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.
This study demonstrated that Neuronal ceroid lipofuscinosis with DNAJC5/CSPalpha mutation has PPT1 pathology and exhibit aberrant protein palmitoylation.
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.
the presynaptic vesicle protein CSPalpha is a key player in synaptic degeneration and protection in Alzheimer's disease.
Missense mutations in DNAJC5 does not play a major role in PD in the Chinese population.
Results indicate of a p.L116del mutation in DNAJC5 from families with autosomal dominant Kufs disease.
Palmitoylation-induced aggregation of mutant CSP-alpha proteins may underlie the development of adult-onset neuronal ceroid lipofuscinosis
association of DNAJC5 mutations with autosomal dominant Kufs disease
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
A neuroprotective role for CSPalpha in humans is confirmed.
First evidence that CSP and HSP70, and their interactions with MARCKS, are involved in mucin secretion from airway epithelium.
Csp not only regulates the exit of CFTR from the ER, but this action is accompanied by Hsc70/Hsp70 and CHIP-mediated CFTR degradation.
Csp has a role in regulated CFTR trafficking at the plasma membrane. [CYSTEINE STRING PROTEIN]
Cysteine string protein inhibits N-type calcium channels, but is blocked by mutant huntingtin
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
These data show that neurodegenerative processes associated with lysosomal dysfunction may be presynaptically initiated by a concomitant reduction in alpha-synuclein and CSPalpha levels at nerve terminals.
CSPalpha has a role in regulating synaptic excitability and neurotransmission by limiting expression of BK channels.
The mRNA and protein expression of CSP1 was elevated in 3T3-L1 adipocytes in insulin resistant states caused by high levels of palmitate and chronic insulin exposure.
gamma-Synuclein binds synaptic vesicles but is unable to rescue the phenotype of CSPalpha-deficient mice.
Using hippocampal cultures, study shows that CSPalpha regulates the stability of client proteins and synaptic vesicle number. Analysis of CSPalpha-dynamin 1 interactions reveals unexpectedly that CSPalpha regulates the polymerization of dynamin 1.
Study shows that cysteine string protein-alpha is required to maintain the size of the releasable pool of synaptic vesicles in motorneurons, presumably by conveying stability to SNAP-25.
findings suggest that the neurodegeneration in CSPalpha KO mice is primarily produced by defective SNAP-25 function, which causes neurodegeneration by impairing SNARE-complex assembly
Data indicate that Cspalpha interacts with Syt9, and such a complex may be relevant in the calcium-mediated control of a late stage of exocytosis by triggering the specific recruitment of a folding catalyst at the fusion point.
Data show that deletion of CSPalpha produces an abnormal SNAP-25 conformer that inhibits SNARE-complex formation, and is subject to ubiquitylation and proteasomal degradation.
CSPalpha is not essential for the normal operation of Ca2+ channels or exocytosis but acts as a presynaptic chaperone that maintains continued synaptic function. Its deletion causes postnatal lethality of knockout mice.
Tonically active ribbon synapses in retina are particularly sensitive to the deletion of CSPalpha and that expression of at least one CSP isoform is essential to protect such tonically active synapses from neurodegeneration.
palmitoylation of CSP is enhanced specifically by co-expression of the Golgi-localized palmitoyl transferases DHHC3, DHHC7, DHHC15, or DHHC17
CSPalpha is indispensable for a normal Ca2+ sensitivity of exocytosis in vertebrate mature synapses
a role for Hsp40 crosstalk with CSPalpha in neuroprotection.
This gene is a member of the J protein family. J proteins function in many cellular processes by regulating the ATPase activity of 70 kDa heat shock proteins. The encoded protein plays a role in membrane trafficking and protein folding, and has been shown to have anti-neurodegenerative properties. The encoded protein is known to play a role in cystic fibrosis and Huntington's disease. A pseudogene of this gene is located on the short arm of chromosome 8.
, cystein string protein
, cysteine string protein
, cysteine string protein 3
, cysteine-string protein
, DnaJ (Hsp40) homolog, subfamily C, member 5
, cysteine string protein alpha
, dnaJ homolog subfamily C member 5