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B9D1 Antibody

     
  • 1 - B9D1 Antibody ASC11440
    Western blot analysis of B9D1 in 293 cell lysate with B9D1 antibody at 1 µg/mL in (A) the absence and (B) the presence of blocking peptide.
  • 8 - B9D1 Antibody ASC11440
    Immunocytochemistry of B9D1 in 293 cells with B9D1 antibody at 5 µg/mL.
  • 3 - B9D1 Antibody ASC11440
    Immunofluorescence of B9D1 in 293 cells with B9D1 antibody at 20 µg/mL.
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Product Information
Application
  • Applications Legend:
  • E=ELISA
  • WB=Western Blotting
  • IHC=Immunohistochemistry
  • IHC-P=Immunohistochemistry (Paraffin)
  • IP=Immunoprecipitation
  • IF=Immunofluorescence
  • IC=Immunochemistry
  • ICC=Immunocytochemistry
  • FC=Flow Cytometry
  • DB=Dot Blot
WB, IF, ICC, E
Primary Accession Q9UPM9
Other Accession NP_056496, 343478275
Reactivity Human
Host Rabbit
Clonality Polyclonal
Isotype IgG
Calculated MW 22775 Da
Concentration (mg/ml) 1 mg/mL
Conjugate Unconjugated
Application Notes B9D1 antibody can be used for detection of B9D1 by Western blot at 1 µg/mL. Antibody can also be used for immunocytochemistry starting at 5 µg/mL. For immunofluorescence start at 20 µg/mL.
Additional Information
Gene ID 27077
Other Names B9 domain-containing protein 1, MKS1-related protein 1, B9D1, MKSR1
Target/Specificity B9D1; At least two isoforms of B9D1 are known to exist; this antibody will only recognize the longest isoform. B9D1 antibody is predicted to not cross-react with other DNAJC family members.
Reconstitution & Storage B9D1 antibody can be stored at 4℃ for three months and -20℃, stable for up to one year. As with all antibodies care should be taken to avoid repeated freeze thaw cycles. Antibodies should not be exposed to prolonged high temperatures.
PrecautionsB9D1 Antibody is for research use only and not for use in diagnostic or therapeutic procedures.

For Research Use Only. Not For Use In Diagnostic Procedures.

Protein Information
Name B9D1
Synonyms MKSR1
Function Component of the tectonic-like complex, a complex localized at the transition zone of primary cilia and acting as a barrier that prevents diffusion of transmembrane proteins between the cilia and plasma membranes. Required for ciliogenesis and sonic hedgehog/SHH signaling (By similarity).
Cellular Location Cytoplasm, cytoskeleton, cilium basal body. Cytoplasm, cytoskeleton, cilium axoneme. Note=Localizes at the transition zone, a region between the basal body and the ciliary axoneme.
Research Areas

BACKGROUND

B9D1 Antibody: Meckel syndrome (MKS) is an embryonic lethal, autosomal recessive disorder characterized by polycystic kidney disease, central nervous system defects, polydactyly and liver fibrosis. B9D1 is a B9 domain-containing protein, one of several that are involved in ciliogenesis. Alterations in expression of this gene have been found in a family with Meckel syndrome. B9D1, and its related protein B9D2, form a complex with MKS1, disruption of which causes MKS. B9D1 is thought to be required for normal hedgehog signaling, ciliogenesis, and ciliary protein localization.

REFERENCES

Williams CL, Winkelbauer ME, Schafer JC, et al. Functional redundancy of the B9 proteins and nephocystins in Caenorhabditis elegans ciliogenesis. Mol. Biol. Cell 2008; 19:2154-68.
Hopp K, Heyer CM, Hommerding CJ, et al. B9D1 is revealed as a novel Meckel syndrome (MKS) gene by targeted exon-enriched next-generation sequencing and deletion analysis. Hum. Mol. Genet. 2011; 20:2524-34.
Dowdle WE, Robinson JF, Kneist A, et al. Disruption of a ciliary B9 protein complex causes Meckel syndrome. Am. J. Hum. Genet. 2011; 89:94-110.

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