产品货号 : mlR15239
英文名称 : C6orf206
中文名称 : 6号染色体开放阅读框206抗体
别 名 : C6orf206; Chromosome 6 open reading frame 206; CILD12; FLJ30845; Mitochondrial ribosomal protein S18A like 1; MRPS18AL1; Radial spoke head 9 homolog; Radial spoke head protein 9 homolog; RSPH 9; rsph9; RSPH9_HUMAN; Uncharacterized protein C6orf206.
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, Mouse, Rat, Dog, Pig, Cow, Sheep,
产品应用 : ELISA=1:500-1000 IHC-P=1:400-800 IHC-F=1:400-800 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 : 31kDa
细胞定位 : 细胞浆
性 状 : Lyophilized or Liquid
浓 度 : 1mg/ml
免 疫 原 : KLH conjugated synthetic peptide derived from human C6orf206:1-100/276
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 : Probable component of the axonemal radial spoke head. Radial spokes are regularly spaced along cilia, sperm and flagella axonemes. They consist of a thin stalk, which is attached to a subfiber of the outer doublet microtubule, and a bulbous head, which is attached to the stalk and appears to interact with the projections from the central pair of microtubules.
Subcellular Location:
Cytoplasm, cytoskeleton, cilium axoneme (Probable). Note=Radial spoke (Probable).
DISEASE:
Defects in RSPH9 are the cause of primary ciliary dyskinesia type 12 (CILD12) [MIM:612650]. CILD is an autosomal recessive disorder characterized by axonemal abnormalities of motile cilia. Respiratory infections leading to chronic inflammation and bronchiectasis are recurrent, due to defects in the respiratory cilia; reduced fertility is often observed in male patients due to abnormalities of sperm tails. Half of the patients exhibit situs inversus, due to dysfunction of monocilia at the embryonic node and randomization of left-right body asymmetry. Primary ciliary dyskinesia associated with situs inversus is referred to as Kartagener syndrome.
Similarity:
Belongs to the flagellar radial spoke RSP9 family.
SWISS:
Q9H1X1
Gene ID:
221421
Important Note:
This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.
产品图片