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Product Name C6orf206 Chinese Name 6号染色体开放阅读框206抗体 Alias 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. Research Area Cell biology immunology Immunogen Species Rabbit Clonality Polyclonal React Species Human, Mouse, (predicted: Rat, Cow, Sheep, ) Applications WB=1:500-2000 ELISA=1:5000-10000
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.Theoretical molecular weight 31kDa Cellular localization cytoplasmic Form Liquid Concentration 1mg/ml immunogen KLH conjugated synthetic peptide derived from human C6orf206: 201-276/276 Lsotype IgG Purification affinity purified by Protein A Buffer Solution 0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol. Storage Shipped at 4℃. Store at -20 °C for one year. Avoid repeated freeze/thaw cycles. Attention This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications. PubMed PubMed Product Detail 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
Database links:Entrez Gene: 221421 Human
Omim: 612648 Human
SwissProt: Q9H1X1 Human
Unigene: 534585 Human
Product Picture Sample:
Lane 1: Mouse NIH/3T3 cell lysates
Lane 2: Human SH-SY5Y cell lysates
Lane 3: Human MCF-7 cell lysates
Lane 4: Human THP-1 cell lysates
Primary: Anti-C6orf206 (SL24834R) at 1/1000 dilution
Secondary: IRDye800CW Goat Anti-Rabbit IgG at 1/20000 dilution
Predicted band size: 31 kDa
Observed band size: 33 kDa
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