Anti-C9orf61抗体Friedreich's ataxia is an inherited disease that is characterized by a progressive degeneration of the spinal cord and nerve tissue. Caused by

产品编号 yb-15334R
英文名称 Anti-C9orf61抗体
中文名称 9号染色体开放阅读框61抗体
别    名 Chromosome 9 open reading frame 61; Friedreich ataxia region gene X123; MGC142243; MGC142245; OTTHUMP00000063356; Protein X123; RP11 548B3.1; Uncharacterized protein C9orf61; X123; F1892_HUMAN.
 Anti-C9orf61抗体   
说 明 书 0.2ml  
研究领域 细胞生物  免疫学  
抗体来源 Rabbit
克隆类型 Polyclonal
交叉反应 Human, 
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 47kDa
性    状 Lyophilized or Liquid
浓    度 1mg/1ml
免 疫 原 KLH conjugated synthetic peptide derived from human C9orf61
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:
Friedreich's ataxia is an inherited disease that is characterized by a progressive degeneration of the spinal cord and nerve tissue. Caused by a mutated gene region on chromosome 9 that results in mitochondrial malfunction, Friedreich's ataxia can lead to a variety of conditions including speech problems, vision impairment, muscle weakness, diabetes and scoliosis. X123, also known as C9orf61 (chromosome 9 open reading frame 61), is a 289 amino acid protein that is expressed at high levels in skeletal muscle and at lower levels in brain, heart and lung. The gene encoding X123 is located within the Friedreich's ataxia region on chromosome 9, suggesting a possible role for X123 in the pathogenesis of this disease.

Subcellular Location:
Membrane; Single-pass type I membrane protein (Potential). 
 
Tissue Specificity:
Prominently expressed in muscle.