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Product Name KIF1B Chinese Name 驱动蛋白家族成员1B抗体 Alias 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. Research Area Neurobiology Signal transduction Cell adhesion molecule Cytoskeleton Extracellular matrix Immunogen Species Rabbit Clonality Polyclonal React Species Rat, (predicted: Human, Mouse, Chicken, Dog, Pig, Sheep, ) 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 204kDa Cellular localization cytoplasmic Form Liquid Concentration 1mg/ml immunogen KLH conjugated synthetic peptide derived from human KIF1B: 13-120/1816 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 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
Omim: 605995 Human
SwissProt: O60333 Human
SwissProt: Q60575 Mouse
Unigene: 97858 Human
Unigene: 402393 Mouse
Product Picture 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(SL11033R) 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(SL11033R-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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