胞质动力蛋白2重链1抗体
规格：1mg/1ml
英文名： DYNC2H1
别名： Cytoplasmic dynein 2 heavy chain 1; Cytoplasmic dynein 2 heavy chain; DYHC2_HUMAN; DYNC2H1; Dynein cytoplasmic heavy chain 2; Dynein heavy chain 11; Dynein heavy chain isotype 1B; hDHC11.
分子量： 492kDa
储存液：0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glyce
克隆类型：Polyclonal
亚型：IgG
纯化方法：affinity purified by Protein A
**原：KLH conjugated synthetic peptide derived from human DYNC2H1
交叉反应：Human, Mouse, Rat, Dog, Pig, Cow, Horse, Rabbit, Sheep,
细胞定位：细胞浆
胞质动力蛋白2重链1抗体产品介绍：background: This gene encodes a large cytoplasmic dynein protein that is involved in retrograde transport in the cilium and has a role in intraflagellar transport, a process required for ciliary/flagellar assembly. Mutations in this gene cause a heterogeneous spectrum of conditions related to altered primary cilium function and often involve polydactyly, abnormal skeletogenesis, and polycystic kidneys. Alternative splicing results in multiple transcript variants encoding distinct proteins. [provided by RefSeq, Jan 2010] Function: May function as a motor for intraflagellar retrograde transport. Functions in cilia biogenesis. May play a role in transport between endoplasmic reticulum 胞质动力蛋白2重链1抗体and Golgi or organization of the Golgi in cells. Subcellular Location: Cytoplasm； cytoskeleton； cilium axoneme. Cell membrane. Cytoplasm. Localizes to the apical cytoplasm (By similarity). According to PubMed:8666668, it localizes to Golgi apparatus, cytoplasmic vesicle and endoplasmic reticulum. DISEASE: Defects in DYNC2H1 are the cause of asphyxiating thoracic dystrophy type 3 (ATD3) [MIM:613091].胞质动力蛋白2重链1抗体 ATD3 is an autosomal recessive osteochondrodysplasia which often leads to death in infancy because of a severely constricted thoracic cage and respiratory insufficiency. Defects in DYNC2H1 are the cause of short rib-polydactyly syndrome type 3 (SRPS3) [MIM:263510]; also called Verma-Naumoff syndrome. A lethal skeletal dysplasia characterized by markedly short ribs, short limbs, polydactyly, narrow thorax, and multiple anomalies of major organs, including heart, intestines, genitalia, kidney, liver, and pancreas. Similarity: Belongs to the dynein heavy chain family. Gene ID: 79659 Database links: Entrez Gene: 79659 Human SwissProt: Q8NCM8 Human Unigene: 503721 Human Important Note: This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.
胞质动力蛋白2重链1抗体产品应用：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：细胞生物 发育生物学 信号转导 转运蛋白
储存条件： Store at -20 °C for one year. Avoid repeated freeze/thaw cycles.
来源： Rabbit
外观： Lyophilized or Liquid