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Product Name CSP Chinese Name 半胱氨酸延伸蛋白α抗体 Alias 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. Research Area Cell biology Neurobiology Signal transduction Cell type markers Immunogen Species Rabbit Clonality Polyclonal React Species Human, Rat, (predicted: Mouse, Pig, Cow, Horse, Rabbit, Sheep, Guinea Pig, ) Applications WB=1:500-2000 ELISA=1:5000-10000 IHC-P=1:100-500 IHC-F=1:100-500 ICC=1:100-500 IF=1:100-500 (Paraffin sections need antigen repair)
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.Theoretical molecular weight 22kDa Cellular localization The cell membrane Form Liquid Concentration 1mg/ml immunogen KLH conjugated synthetic peptide derived from human CSP: 1-100/198 Lsotype IgG Purification affinity purified by Protein A Buffer Solution 0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol. Storage Shipped at 4℃. Store at -20 °C for one year. Avoid repeated freeze/thaw cycles. Attention This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications. PubMed PubMed Product Detail Cysteine string proteins (CSPs) are synaptic vesicle-associated, secretory vesicle proteins that are involved in Ca2+-regulated exocytosis of synaptic vesicles and modulation of presynaptic transmembrane calcium fluxes in neuroendocrine and endocrine cell types. CSP contains a J-domain that binds HSP 70/HSC 70 chaperone ATPases and a membrane-targeting, palmitoylated cysteine-rich string region. CSPs may act as molecular chaperones in synapses, and mediate conformational folding of components of the vesicular exocytotic machinery. CSP is involved in the fine tuning of neurotransmission through its interaction with receptor-coupled trimeric GTP binding proteins (G proteins) and N-type Ca2+ channels. Two variants of CSP have been described: CSP1; and the 31 amino acid, C-terminally truncated isoform, CSP2. Subcellular fractionation of insulinoma cells shows CSP1 in granular fractions, while the membrane and cytosol fractions contain predominantly CSP2. The fractions also contain additional proteins, presumably CSP dimers. Furthermore, in various mammalian cell lines (including rat brain) CSP1 expression predominates CSP2 expression.
Function:
May have an important role in presynaptic function. May be involved in calcium-dependent neurotransmitter release at nerve endings.
Subunit:
Homodimer (Probable). Interacts with the chaperone complex consisting of HSC70 and SGTA (By similarity).
Subcellular Location:
Membrane. Melanosome. Identified by mass spectrometry in melanosome fractions from stage I to stage IV.
Tissue Specificity:
Expressed in pancreas, kidney, skeletal muscle, liver, lung, placenta, brain and heart.
Post-translational modifications:
Fatty acylated. Heavily palmitoylated in the cysteine string motif.
DISEASE:
Neuronal ceroid lipofuscinosis 4B (CLN4B) [MIM:162350]: An adult-onset neuronal ceroid lipofuscinosis. Neuronal ceroid lipofuscinoses are progressive neurodegenerative, lysosomal storage diseases characterized by intracellular accumulation of autofluorescent liposomal material, and clinically by seizures, dementia, visual loss, and/or cerebral atrophy. CLN4B has no visual involvement and is characterized by seizures and other neurologic symptoms. Note=The disease is caused by mutations affecting the gene represented in this entry.
Similarity:
Contains 1 J domain.
SWISS:
Q9H3Z4
Gene ID:
80331
Database links:Entrez Gene: 80331 Human
Entrez Gene: 13002 Mouse
Omim: 611203 Human
SwissProt: Q9H3Z4 Human
SwissProt: P60904 Mouse
Unigene: 164419 Human
Unigene: 140761 Mouse
Unigene: 391807 Mouse
Unigene: 100120 Rat
Product Picture Tissue/cell: human placenta tissue; 4% Paraformaldehyde-fixed and paraffin-embedded;
Antigen retrieval: citrate buffer ( 0.01M, pH 6.0 ), Boiling bathing for 15min; Block endogenous peroxidase by 3% Hydrogen peroxide for 30min; Blocking buffer (normal goat serum,C-0005) at 37℃ for 20 min;
Incubation: Anti-CSP Polyclonal Antibody, Unconjugated(SL12944R) 1:200, overnight at 4°C, followed by conjugation to the secondary antibody(SP-0023) and DAB(C-0010) staining
Tissue/cell: Rat pancreas tissue; 4% Paraformaldehyde-fixed and paraffin-embedded;
Antigen retrieval: citrate buffer ( 0.01M, pH 6.0 ), Boiling bathing for 15min; Block endogenous peroxidase by 3% Hydrogen peroxide for 30min; Blocking buffer (normal goat serum,C-0005) at 37℃ for 20 min;
Incubation: Anti-CSP Polyclonal Antibody, Unconjugated(SL12944R) 1:200, overnight at 4°C, followed by conjugation to the secondary antibody(SP-0023) and DAB(C-0010) staining
Tissue/cell: rat brain tissue; 4% Paraformaldehyde-fixed and paraffin-embedded;
Antigen retrieval: citrate buffer ( 0.01M, pH 6.0 ), Boiling bathing for 15min; Block endogenous peroxidase by 3% Hydrogen peroxide for 30min; Blocking buffer (normal goat serum,C-0005) at 37℃ for 20 min;
Incubation: Anti-CSP Polyclonal Antibody, Unconjugated(SL12944R) 1:200, overnight at 4°C, followed by conjugation to the secondary antibody(SP-0023) and DAB(C-0010) staining
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