Anti-COQ9抗体This locus represents a mitochondrial ubiquinone biosynthesis gene. The encoded protein is likely necessary for biosynthesis of coenzyme Q10,

产品编号 yb-13989R
英文名称 Anti-COQ9抗体
中文名称 辅酶合成加氧酶COQ9抗体
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.
 Anti-COQ9抗体
说 明 书 0.1ml  0.2ml  
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  
抗体来源 Rabbit
克隆类型 Polyclonal
交叉反应 Human, Mouse, Rat, Pig, Cow, Horse, Sheep, 
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.
分 子 量 35kDa
细胞定位 细胞膜 
性    状 Lyophilized or Liquid
浓    度 1mg/1ml
免 疫 原 KLH conjugated synthetic peptide derived from human COQ9
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.
 
PubMed PubMed
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 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 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; Leigh syndrome with growth retardation; and an isolated myopathic form. Note=The disease is caused by mutations affecting the gene represented in this entry.