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    KIF1B Rabbit pAb (bs-11033R)  
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    產品編號 bs-11033R
    英文名稱 KIF1B Rabbit pAb
    中文名稱 驅動蛋白家族成員1B抗體
    別    名 Charcot Marie Tooth neuropathy 2A; CMT 2; CMT 2A; CMT 2A1; CMT2 A; CMT2 A1; CMT2; CMT2A 1; CMT2A; CMT2A1; D4Mil1e; HMSN II; HMSNII; HMSNII, hereditary motor sensory neuropathy II; KIF 1B; KIF1 B; KIF1B p130; KIF1B p204; KIF1Bp130; KIF1Bp204; kinesin family member 1B; Kinesin like protein KIF1B; Klp; KIF1B_HUMAN.  
    研究領域 神經生物學  信號轉導  細胞粘附分子  細胞骨架  細胞外基質  
    抗體來源 Rabbit
    克隆類型 Polyclonal
    克 隆 號
    交叉反應 Rat
    產品應用 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 204 kDa
    檢測分子量
    細胞定位 細胞漿 
    性    狀 Liquid
    濃    度 1mg/ml
    免 疫 原 KLH conjugated synthetic peptide derived from human KIF1B: 13-120/1816 
    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
    產品介紹 KIF1B, or kinesin-like protein (Klp) functions as a motor for mitochondrial transport, and has a microtubule plus end-directed motility. The KIF1B beta isoform is abundant in brain, while the alpha isoform is abundant in skeletal muscle. Mutations in the KIF1B gene are the cause of Charcot-Marie-Tooth disease type 2A1, which is a primary peripheral axon neuropathy. The KIF1B beta isoform is down-regulated in sporadic amyotrophic lateral sclerosis (ALS).

    Function:
    Motor for anterograde transport of mitochondria. Has a microtubule plus end-directed motility. Isoform 2 is required for induction of neuronal apoptosis.

    Subunit:
    Interacts (via C-terminus end of the kinesin-motor domain) with CHP1; the interaction occurs in a calcium-dependent manner (By similarity). Interacts with KBP.

    Subcellular Location:
    Cytoplasmic vesicle (By similarity). Cytoplasm, cytoskeleton (By similarity). Mitochondrion. Note=Colocalizes with synaptophysin at synaptic cytoplasmic transport vesicles in the neurites of hippocampal neurons (By similarity).

    Tissue Specificity:
    Isoform 3 is abundant in the skeletal muscle. It is also expressed in fetal brain, lung and kidney, and adult heart, placenta, testis, ovary and small intestine. Isoform 2 is abundant in the brain and also expressed in fetal heart, lung, liver and kidney, and adult skeletal muscle, placenta, liver, kidney, heart, spleen, thymus, prostate, testis, ovary, small intestine, colon and pancreas.

    DISEASE:
    Defects in KIF1B are the cause of Charcot-Marie-Tooth disease type 2A1 (CMT2A1) [MIM:118210]. CMT2A1 is a form of Charcot-Marie-Tooth disease, the most common inherited disorder of the peripheral nervous system. Charcot-Marie-Tooth disease is classified in two main groups on the basis of electrophysiologic properties and histopathology: primary peripheral demyelinating neuropathy or CMT1, and primary peripheral axonal neuropathy or CMT2. Neuropathies of the CMT2 group are characterized by signs of axonal regeneration in the absence of obvious myelin alterations, normal or slightly reduced nerve conduction velocities, and progressive distal muscle weakness and atrophy.
    Defects in KIF1B are the cause of susceptibility to neuroblastoma type 1 (NBLST1) [MIM:256700]. A common neoplasm of early childhood arising from embryonic cells that form the primitive neural crest and give rise to the adrenal medulla and the sympathetic nervous system.
    Defects in KIF1B are a cause of susceptibility to pheochromocytoma (PCC) [MIM:171300]. A catecholamine-producing tumor of chromaffin tissue of the adrenal medulla or sympathetic paraganglia. The cardinal symptom, reflecting the increased secretion of epinephrine and norepinephrine, is hypertension, which may be persistent or intermittent.

    Similarity:
    Belongs to the kinesin-like protein family. Unc-104 subfamily.
    Contains 1 FHA domain.
    Contains 1 kinesin-motor domain.
    Contains 1 PH domain.

    SWISS:
    O60333

    Gene ID:
    23095

    Database links:

    Entrez Gene: 23095 Human

    Entrez Gene: 16561 Mouse

    Entrez Gene: 117548 Rat

    Omim: 605995 Human

    SwissProt: O60333 Human

    SwissProt: Q60575 Mouse

    SwissProt: O88658 Rat

    Unigene: 97858 Human

    Unigene: 402393 Mouse



    產品圖片
    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-KIF1B Polyclonal Antibody, Unconjugated(bs-11033R) 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; Blocking buffer (normal goat serum,C-0005) at 37℃ for 20 min; Incubation: Anti-KIF1B Polyclonal Antibody, FITC conjugated(bs-11033R-FITC) 1:200, at 1:200 dilution for 40 minutes at 37°C. DAPI(5ug/ml,blue,C-0033) was used to stain the cell nuclei
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