辅酶生物合成单加氧酶COQ6抗体
规格：1mg/1ml
英文名： COQ6
别名： CGI-10; Coenzyme Q6 homolog (yeast); Coenzyme Q6 homolog, monooxygenase (S. cerevisiae); Coenzyme Q6 homolog, monooxygenase (yeast); coq6; COQ6_HUMAN; Ubiquinone biosynthesis monooxygenase COQ6.
分子量： 51kDa
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 COQ6
交叉反应：Human, Mouse, Rat, Rabbit,
细胞定位：
辅酶生物合成单加氧酶COQ6抗体产品介绍：background: The protein encoded by this gene belongs to the ubiH/COQ6 family. It is an evolutionarily conserved monooxygenase required for the biosynthesis of coenzyme Q10 (or ubiquinone), which is an essential component of the mitochondrial electron transport chain, and one of the most potent lipophilic antioxidants implicated in the protection of cell damage by reactive oxygen species. Knockdown of this gene in mouse and zebrafish results in decreased growth due to increased apoptosis. Mutations in辅酶生物合成单加氧酶COQ6抗体 this gene are associated with autosomal recessive coenzyme Q10 deficiency-6 (COQ10D6), which manifests as nephrotic syndrome with sensorineural deafness. Alternatively spliced transcript variants encoding different isoforms have been described for this gene. [provided by RefSeq, Jun 2012] Subcellular Location: Golgi apparatus. Cell projection. Note=Localizes to podocyte cell processes. Tissue Specificity: Widely epressed. DISEASE: Coenzyme Q10 deficiency, primary, 6 (COQ10D6) [MIM:614650]: An autosomal recessive disorder characterized by 辅酶生物合成单加氧酶COQ6抗体onset in infancy of severe progressive nephrotic syndrome resulting in end-stage renal failure and sensorineural deafness. Renal biopsy usually shows focal segmental glomerulosclerosis. Note=The disease is caused by mutations affecting the gene represented in this entry. Similarity: Belongs to the ubiH/COQ6 family. Gene ID: 51004 Database links: Entrez Gene: 51004 Human SwissProt: Q9Y2Z9 Human Unigene: 131555 Human Important Note: This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.
辅酶生物合成单加氧酶COQ6抗体产品应用：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：细胞生物 神经生物学 细胞凋亡
储存条件： Store at -20 °C for one year. Avoid repeated freeze/thaw cycles.
来源： Rabbit
外观： Lyophilized or Liquid