驱动蛋白家族成员1叠抗体
规格:1尘驳/1尘濒
英文名: KIF1B
别名: 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; KIF1
分子量: 204kDa
储存液:0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glyce
克隆类型:笔辞濒测肠濒辞苍补濒
亚型:滨驳骋
纯化方法:affinity purified by Protein A
**原:KLH conjugated synthetic peptide derived from human KIF1B
交叉反应:Human, Mouse, Rat, Chicken, Dog, Pig, Sheep,
细胞定位:细胞浆
驱动蛋白家族成员1叠抗体产物介绍:background: 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驱动蛋白家族成员1叠抗体 in fetal brain, lung and kidney, and 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 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. 驱动蛋白家族成员1叠抗体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. 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 Important Note: This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.
驱动蛋白家族成员1叠抗体产物应用:WB=1:100-500 ELISA=1:500-1000 IHC-P=1:100-500 IHC-F=1:100-500 ICC=1:100-500 IF=1:100-500 (石蜡切片需做抗原修复) not yet tested in other applications. optimal dilutions/concentrations should be determined by the end user.
研究领域:神经生物学 信号转导 细胞粘附分子 细胞骨架 细胞外基质
储存条件: Store at -20 °C for one year. Avoid repeated freeze/thaw cycles.
来源: Rabbit
外观: Lyophilized or Liquid