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    CSP Rabbit pAb (bs-12944R)  
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    產(chǎn)品編號 bs-12944R
    英文名稱(chēng) CSP Rabbit pAb
    中文名稱(chēng) 半胱氨酸延伸蛋白α抗體
    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.  
    研究領(lǐng)域 細胞生物  神經(jīng)生物學(xué)  信號轉導  細胞類(lèi)型標志物  
    抗體來(lái)源 Rabbit
    克隆類(lèi)型 Polyclonal
    克 隆 號
    交叉反應 Human,Rat (predicted: Mouse,Rabbit,Pig,Sheep,Cow,GuineaPig,Horse)
    產(chǎn)品應用 IHC-P=1:100-500,IHC-F=1:100-500,IF=1:100-500
    not yet tested in other applications.
    optimal dilutions/concentrations should be determined by the end user.
    理論分子量 22 kDa
    檢測分子量
    細胞定位 細胞膜 
    性    狀 Liquid
    濃    度 1mg/ml
    免 疫 原 KLH conjugated synthetic peptide derived from human CSP: 1-100/198 
    亞    型 IgG
    純化方法 affinity purified by Protein A
    緩 沖 液 0.01M TBS (pH7.4) with 1% BSA, 0.02% Proclin300 and 50% Glycerol.
    保存條件 Shipped at 4℃. Store at -20℃ for one year. Avoid repeated freeze/thaw cycles.
    注意事項 This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.
    PubMed PubMed
    產(chǎn)品介紹 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

    Entrez Gene: 79130 Rat

    Omim: 611203 Human

    SwissProt: Q9H3Z4 Human

    SwissProt: P60904 Mouse

    SwissProt: P60905 Rat

    Unigene: 164419 Human

    Unigene: 140761 Mouse

    Unigene: 391807 Mouse

    Unigene: 100120 Rat



    產(chǎn)品圖片
    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(bs-12944R) 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(bs-12944R) 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(bs-12944R) 1:200, overnight at 4°C, followed by conjugation to the secondary antibody(SP-0023) and DAB(C-0010) staining
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