FITC标记的动力蛋白激活蛋白1抗体
产品名称： FITC标记的动力蛋白激活蛋白1抗体
英文名称： Anti-DCTN1/DAP-150/FITC
产品编号： HZ-6929R-FITC
产品价格： null
产品产地： 中国/上海
品牌商标： HZbscience
更新时间： 2023-08-17T10:24:20
使用范围： IF=1:50-200
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Rabbit Anti-DCTN1/DAP-150/FITC Conjugated antibody
FITC标记的动力蛋白激活蛋白1抗体
| 英文名称 | Anti-DCTN1/DAP-150/FITC |
| 中文名称 | FITC标记的动力蛋白激活蛋白1抗体 |
| 别 名 | Alternative names150 kDa dynein associated polypeptide; 150 kDa dynein-associated polypeptide; DAP 150; DAP-150; DAP150; DCTN 1; DCTN1; DCTN1_HUMAN; DP 150; DP-150; DP150; Dynactin 1 (p150 Glued (Drosophila) homolog); Dynactin 1 (p150 glued homolog Drosophila); Dynactin 1; Dynactin subunit 1; Dynactin1; HMN7B; P135; p150 Glued (Drosophila) homolog; p150 glued; p150 glued homolog; p150(GLUED) DROSOPHILA HOMOLOG OF; p150-glued; p150glued. |
| 规格价格 | 100ul/2980元 购买 大包装/询价 |
| 说 明 书 | 100ul |
| 研究领域 | 肿瘤 细胞生物 神经生物学 信号转导 细胞周期蛋白 转录调节因子 |
| 抗体来源 | Rabbit |
| 克隆类型 | Polyclonal |
| 交叉反应 | Human, Mouse, Rat, Dog, Pig, Cow, Horse, Rabbit, |
| 产品应用 | IF=1:50-200 not yet tested in other applications. optimal dilutions/concentrations should be determined by the end user. |
| 分 子 量 | 142kDa |
| 性 状 | Lyophilized or Liquid |
| 浓 度 | 1mg/ml |
| 免 疫 原 | KLH conjugated synthetic peptide derived from human DCTN1/Dynactin 1 |
| 亚 型 | IgG |
| 纯化方法 | affinity purified by Protein A |
| 储 存 液 | 0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol. |
| 保存条件 | Store at -20 °C for one year. Avoid repeated freeze/thaw cycles. The lyophilized antibody is stable at room temperature for at least one month and for greater than a year when kept at -20°C. When reconstituted in sterile pH 7.4 0.01M PBS or diluent of antibody the antibody is stable for at least two weeks at 2-4 °C. |
| 产品介绍 | background: Required for the cytoplasmic dynein-driven retrograde movement of vesicles and organelles along microtubules. Dynein-dynactin interaction is a key component of the mechanism of axonal transport of vesicles and organelles. Tissue specificity; Brain. Involvement in disease; Defects in DCTN1 are the cause of distal hereditary motor neuronopathy type 7B (HMN7B); also known as progressive lower motor neuron disease (PLMND). HMN7B is a neuromuscular disorder. Distal hereditary motor neuronopathies constitute a heterogeneous group of neuromuscular disorders caused by selective degeneration of motor neurons in the anterior horn of the spinal cord, without sensory deficit in the posterior horn. The overall clinical picture consists of a classical distal muscular atrophy syndrome in the legs without clinical sensory loss. The disease starts with weakness and wasting of distal muscles of the anterior tibial and peroneal compartments of the legs. Later on, weakness and atrophy may expand to the proximal muscles of the lower limbs and/or to the distal upper limbs. Defects in DCTN1 are a cause of susceptibility to amyotrophic lateral sclerosis (ALS). ALS is a neurodegenerative disorder affecting upper and lower motor neurons, and resulting in fatal paralysis. Sensory abnormalities are absent. Death usually occurs within 2 to 5 years. The etiology is likely to be multifactorial, involving both genetic and environmental factors. Defects in DCTN1 are the cause of Perry syndrome (PERRYS); also called parkinsonism with alveolar hypoventilation and mental depression. Perry syndrome is a neuropsychiatric disorder characterized by mental depression not responsive to antidepressant drugs or electroconvulsive therapy, sleep disturbances, exhaustion and marked weight loss. Parkinsonism develops later and respiratory failure occurred terminally. Function: Required for the cytoplasmic dynein-driven retrograde movement of vesicles and organelles along microtubules. Dynein-dynactin interaction is a key component of the mechanism of axonal transport of vesicles and organelles. Subunit: Large macromolecular complex of at least 10 components; p150(glued) binds directly to microtubules and to cytoplasmic dynein. Interacts with the C-terminus of MAPRE1, MAPRE2 and MAPRE3. Interacts with FBXL5. Interacts with ECM29. Interacts (via C-terminus) with SNX6. Subcellular Location: Cytoplasm. Cytoplasm, cytoskeleton. Tissue Specificity: Brain. Post-translational modifications: Ubiquitinated by a SCF complex containing FBXL5, leading to its degradation by the proteasome. DISEASE: Defects in DCTN1 are the cause of distal hereditary motor neuronopathy type 7B (HMN7B) [MIM:607641]; also known as progressive lower motor neuron disease (PLMND). HMN7B is a neuromuscular disorder. Distal hereditary motor neuronopathies constitute a heterogeneous group of neuromuscular disorders caused by selective degeneration of motor neurons in the anterior horn of the spinal cord, without sensory deficit in the posterior horn. The overall clinical picture consists of a classical distal muscular atrophy syndrome in the legs without clinical sensory loss. The disease starts with weakness and wasting of distal muscles of the anterior tibial and peroneal compartments of the legs. Later on, weakness and atrophy may expand to the proximal muscles of the lower limbs and/or to the distal upper limbs. Similarity: Belongs to the dynactin 150 kDa subunit family. Contains 1 CAP-Gly domain. Database links: Entrez Gene: 1639 Human Entrez Gene: 13191 Mouse Entrez Gene: 29167 Rat Omim: 601143 Human SwissProt: Q14203 Human SwissProt: O08788 Mouse SwissProt: P28023 Rat Unigene: 516111 Human Unigene: 6919 Mouse Unigene: 11284 Rat Important Note: This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications |
细胞质动力蛋白驱动小泡和细胞器沿微管的逆行运动所必需的。动力蛋白-肌动蛋白相互作用是囊泡和细胞器轴突运输机制的重要组成部分。
组织特异性；脑。
DCTN1的缺陷是远端遗传性运动神经元病7B（HMN7B）的原因；也称为进行性下运动神经元病（PLMND）。HMN7B是一种神经肌肉疾病。远端遗传性运动神经病是由脊髓前角运动神经元选择性退化引起的神经肌肉紊乱的异质性组，在后角没有感觉缺陷。整体临床图片包括一个典型的远端肌肉萎缩综合征的腿没有临床感觉丧失。这种疾病始于腿部胫前和腓侧肌的远端肌肉无力和浪费。随后，虚弱和萎缩可扩展到下肢的近端肌肉和/或远端上肢。
DCTN1的缺陷是肌萎缩侧索硬化（ALS）易感性的原因之一。ALS是影响上、下运动神经元的神经退行性疾病，并导致致死性瘫痪。感觉异常是不存在的。死亡通常发生在2至5年内。病因可能是多因素的，涉及遗传和环境因素。
DCTN1的缺陷是PrRY综合征（Prrys）的原因，又称帕金森综合征伴肺泡通气不足和精神抑郁。帕里综合征是一种神经精神障碍，其特征是精神抑郁，对抗抑郁药物或电痉挛治疗、睡眠障碍、衰竭和明显的体重减轻没有反应。帕金森综合征发展较晚，呼吸衰竭终末发生。