辅酶合成加氧酶COQ9抗体
规格：1mg/1ml
英文名： COQ9
别名： C16orf49; Chromosome 16 open reading frame 49; Coenzyme Q9 homolog (S. cerevisiae); Coenzyme Q9 homolog (yeast); HSPC326; COQ9_HUMAN; PSEC0129; Ubiquinone biosynthesis protein COQ9, mitochondrial.
分子量： 35kDa
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 COQ9
交叉反应：Human, Mouse, Rat, Pig, Cow, Horse, Sheep,
细胞定位：细胞膜
辅酶合成加氧酶COQ9抗体产品介绍：background: This locus represents a mitochondrial ubiquinone biosynthesis gene. The encoded protein is likely necessary for biosynthesis of coenzyme Q10, as mutations at this locus have been associated with autosomal-recessive neonatal-onset primary coenzyme Q10 deficiency.[provided by RefSeq, Sep 2010] Function: Coenzyme Q10 (CoQ10), or ubiquinone, is a mobile lipophilic electron carrier critical for electron transfer by the mitochondrial inner membrane respiratory chain. COQ9 is 1 of several enzymes involved in biosynthesis of CoQ10 and likely functions in modification of the benzoquinone ring. Subcellular Location: Mitochondrion DISEASE: Coenzyme辅酶合成加氧酶COQ9抗体 Q10 deficiency, primary, 5 (COQ10D5) [MIM:614654]: A form of coenzyme Q10 deficiency, an autosomal recessive disorder with variable manifestations consistent with 5 major phenotypes. The phenotypes include an encephalomyopathic form with seizures and ataxia; a multisystem infantile form with encephalopathy, cardiomyopathy and renal failure; a predominantly cerebellar form with ataxia and cerebellar atrophy辅酶合成加氧酶COQ9抗体; Leigh syndrome with growth retardation; and an isolated myopathic form. Note=The disease is caused by mutations affecting the gene represented in this entry. Similarity: Belongs to the COQ9 family. Gene ID: 57017 Database links: Entrez Gene: 57017 Human Entrez Gene: 67914 Mouse Omim: 612837 Human SwissProt: O75208 Human SwissProt: Q8K1Z0 Mouse Important Note: This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.
辅酶合成加氧酶COQ9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：肿瘤 细胞生物 信号转导 细胞类型标志物 新陈代谢
储存条件： Store at -20 °C for one year. Avoid repeated freeze/thaw cycles.
来源： Rabbit
外观： Lyophilized or Liquid